RARE DISEASE
Angiosarcoma
Angiosarcoma
Angiosarcoma
Drug discovery
3
drugs
With orphan designations
Overview
Angiosarcoma is a rare, aggressive endothelial malignancy comprising 1–2% of soft tissue sarcomas. It commonly arises in cutaneous sites (60% of cases), particularly the head/neck, with visceral or breast involvement linked to prior radiation, chronic lymphedema, or environmental carcinogens [1][2][16]. Diagnosis relies on histopathology and immunohistochemistry (CD31, ERG positivity), supported by imaging (CT/MRI) for staging. Treatment combines surgery, adjuvant radiotherapy (>70 Gy for unresectable cases), and taxane/anthracycline-based chemotherapy, with emerging roles for immunotherapy and targeted therapies in advanced disease [1][3][13].
Population
Median age at diagnosis: 71 years, with 54.5% female predominance overall [2][11].
Secondary angiosarcomas disproportionately affect women (breast/chest wall) and men (head/neck), often linked to prior radiotherapy [2][16].
Incidence rising to >1,000 annual US cases, driven by increased secondary cases [2][11].
Therapies
First-line: Wide-margin resection ± adjuvant radiotherapy (local control: 43% 5-year DFS with combined therapy) [1][18].
Systemic therapy: Weekly paclitaxel (median OS: 18.6 months) or liposomal doxorubicin; taxanes preferred for cutaneous subtypes [1][3][13].
Emerging options: Anti-PD-1 immunotherapy (e.g., pembrolizumab) and VEGF inhibitors (pazopanib) in trials, particularly for metastatic disease [3][8][15].
Categories: rare circulatory system diseases, rare developmental anomalies during embryogenesis, rare neoplastic diseases
Research Papers
1,299 drug discovery papers about Angiosarcoma, with 1 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
1,299 drug discovery papers about Angiosarcoma, with 1 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-09-06 | Primary splenic angiosarcoma presenting with multiple bone destruction as the initial manifestation: a case report and narrative review.
Primary splenic angiosarcoma (PSA) is an extremely rare and highly aggressive malignant vascular tumor. Its clinical presentation is often nonspecific, and the diagnosis is frequently delayed. Osseous metastasis as the initial clinical manifestation is particularly uncommon, posing significant diagnostic challenges. A critical yet underappreciated phenomenon is the discordance between histological differentiation and biological behavior in angiosarcoma: well-differentiated tumors can exhibit highly aggressive clinical courses. Furthermore, biopsy of vascular tumors carries a substantial risk of intractable hemorrhage, a complication that is often underestimated. We report the case of a 56-year-old female who presented with a 20-day history of lower back and right hip pain. Imaging revealed multiple osteolytic lesions involving the thoracolumbar vertebrae, pelvis, and sacrum, as well as multiple nodular lesions in the spleen. The initial clinical suspicion favored metastatic disease of unknown primary. Ultrasound-guided needle biopsy of the right iliac lesion yielded abundant hemorrhage but no definitive malignant tissue on initial pathology. Subsequent open biopsy of the L2 spinous process and right iliac bone resulted in life-threatening hemorrhage, necessitating emergency digital subtraction angiography (DSA) and super-selective embolization. Histopathological examination of the surgical specimens revealed a mixed-type vascular neoplasm with partially well-differentiated angiosarcomatous changes, with immunohistochemical positivity for CD31, CD34, ERG, and Fli-1. Remarkably, follow-up pelvic MRI performed only 21 days after the initial scan demonstrated interval enlargement of multiple bone lesions, indicating extraordinarily rapid disease progression despite the high-grade histological appearance. This case highlights several critical clinical considerations for angiosarcoma: (1) When both splenic and skeletal lesions coexist, suspected primary splenic angiosarcoma with bone metastases should be strongly considered; (2) The histological grade may be discordant with biological behavior-high-grade tumors can exhibit highly aggressive courses; (3) Imaging features can mimic benign vascular tumors such as hemangioma; (4) Biopsy of vascular tumors carries a substantial risk of intractable hemorrhage, and pre-procedural angiographic assessment should be considered. This case underscores the importance of integrating clinical, imaging, and pathological findings for accurate diagnosis and management of this rare malignancy.
2026-08-31 | Pathologic Complete Response Following Frailty-Adjusted Neoadjuvant Paclitaxel in an 88-Year-Old Patient With Radiation-Associated Breast Angiosarcoma.
Radiation-associated breast angiosarcoma is a rare but highly aggressive malignancy with limited evidence to guide optimal management in elderly patients. We present the case of an 88-year-old woman with a history of stage IIIA estrogen receptor-positive invasive ductal carcinoma of the right breast treated with breast-conserving surgery, chemotherapy, radiation therapy, and endocrine therapy in 2014. Approximately 11 years after completing radiation therapy, she developed progressive right breast firmness, violaceous skin discoloration, and persistent ecchymotic changes concerning for vascular malignancy. Imaging demonstrated diffuse skin thickening without a discrete mass, and punch biopsy of skin changes confirmed high-grade angiosarcoma with MYC amplification. Staging studies showed no distant metastatic disease. Given her advanced age, frailty, and comorbidities, she underwent neoadjuvant weekly paclitaxel with initial dose reduction followed by response- and tolerance-guided dose escalation. Despite treatment interruptions related to frailty and fall-related injuries, she completed 12 cycles and subsequently underwent right simple mastectomy. Final pathology demonstrated no residual angiosarcoma, consistent with a pathologic complete response. This case highlights the potential efficacy of frailty-adjusted neoadjuvant paclitaxel in elderly patients with radiation-associated breast angiosarcoma and supports individualized neoadjuvant strategies for patients who are not candidates for standard multi-agent chemotherapy.
2026-08-23 | Giant Breast Angiosarcoma in a 20-Year-Old Young Adult Female: A Case Report and Review of Literature.
Primary breast angiosarcoma (PAS) is an extremely rare vascular malignancy that typically presents as a breast mass or cutaneous changes. Given its highly aggressive nature, rapid progression, and early metastasis that result in a poor prognosis, coupled with a lack of standardized treatment guidelines, there is an urgent need to develop novel, effective therapeutic strategies. This report focused on a 20-year-old Uyghur woman presenting with a painless, rapidly enlarging mass in the left breast. Staging evaluation with breast MRI, whole-body contrast-enhanced CT, and bone scintigraphy revealed a large (10 × 7 cm) primary tumor and bilateral axillary lymphadenopathy suspicious for metastasis, with no evidence of distant disease (cT3N1M0, stage III). The high-grade angiosarcoma was confirmed through a core needle biopsy of the breast mass. With no remarkable medical or family history of breast cancer, the patient underwent complete surgical resection of the breast angiosarcoma, which provided a definitive pathological diagnosis. Given negative lymph node biopsy results, axillary lymph node dissection was not performed. Postoperatively, the patient received adjuvant chemotherapy with paclitaxel and bevacizumab. The regimen was well tolerated, and no disease recurrence was observed during follow-up. This case exemplified a practical management strategy for PAS, emphasizing the key role of clinical presentation and conventional imaging in guiding surgical decisions, particularly in resource-limited settings. As a rare, highly malignant tumor, PAS requires early diagnosis and multidisciplinary management, including thorough metastasis evaluation and personalized therapy, to optimize survival and quality of life.
2026-08-08 | Primary adrenal epithelioid angiosarcoma: An extraordinarily rare malignancy with a challenging diagnosis.
Adrenal epithelioid angiosarcoma is an exceedingly rare vascular endothelial neoplasm, accounting for a minute fraction of sarcomas with fewer than 60 cases reported in the medical literature. We report the case of a 70-year-old female with primary adrenal epithelioid angiosarcoma, presenting with a rapidly enlarging right adrenal mass and associated severe right upper quadrant pain. Biochemical testing revealed that the mass was nonfunctional, and contrast-enhanced computed tomography revealed a large, heterogeneously enhancing lesion with inferior vena cava compression. Given rapid interval growth and severe symptoms, she underwent laparoscopic adrenalectomy, and histopathology with immunohistochemistry confirmed a diagnosis of primary adrenal epithelioid angiosarcoma. Postoperative management included paclitaxel chemotherapy, and the patient has remained disease-free for 2 years post-operation. This case emphasizes that the clinical and imaging features of adrenal epithelioid angiosarcoma are nonspecific, and definitive diagnosis requires surgical pathology with vascular marker staining to avoid misclassification as metastatic carcinoma. Timely resection in combination with chemotherapy can yield favorable outcomes despite this tumor's aggressive behavior and overall poor prognosis.
2026-08-08 | Multimodality imaging features of primary mammary angiosarcoma: a case report with delayed osseous metastases.
Primary angiosarcoma of the breast is an uncommon and aggressive vascular malignancy that typically affects young women and poses diagnostic and therapeutic challenges. We report the case of a 26-year-old nulliparous woman who presented with progressive left breast swelling over approximately 18 months. Clinical examination revealed diffuse tender swelling without skin discoloration, nipple changes, or palpable lymphadenopathy. Ultrasonography demonstrated a complex cystic mass with thick septations and increased vascularity, while mammography showed global asymmetry of the left breast without suspicious calcifications. Breast MRI revealed a large 8.4 cm heterogeneously enhancing mass in the upper outer quadrant with possible focal skin involvement, but no chest wall invasion or nodal disease. Initial staging with 18F-FDG PET/CT showed no distant metastases. The patient underwent left nipple-sparing mastectomy, and histopathological examination confirmed intermediate-grade primary mammary angiosarcoma, FNCLCC grade 2. Subsequent re-excision achieved clear surgical margins. She received adjuvant ifosfamide-epirubicin chemotherapy and radiotherapy. Initial follow-up imaging showed no residual or recurrent disease. However, 14 months after completion of treatment, follow-up 18F-FDG PET/CT demonstrated new FDG-avid lytic lesions in the sacral and iliac bones, and Breast MRI revealed a small enhancing sternal lesion, consistent with delayed osseous metastatic disease. The patient was treated with palliative radiotherapy and systemic therapy. This case highlights the diagnostic complexity of primary breast angiosarcoma, its potential for delayed hematogenous osseous spread, and the value of multimodality imaging in diagnosis, staging, treatment planning, and surveillance.
antibodies
2026-08-01 | Immune checkpoint inhibitor rechallenge after pazopanib treatment in primary pulmonary angiosarcoma: A case report.
Primary pulmonary angiosarcoma is a rare malignancy with limited therapeutic options. We report a case of primary pulmonary angiosarcoma in which tumor regression was achieved with immune checkpoint inhibitor (ICI) rechallenge following pazopanib therapy. The tumor showed strong PD-L1 expression and infiltration of CD3+, CD4+, and CD8+ T cells without CD20+ B cells. After disease control with atezolizumab, pazopanib induced tumor shrinkage but was followed by disease progression. Pembrolizumab rechallenge resulted in tumor regression and sustained benefit. This case suggests that sequential VEGF-targeted therapy and PD-1 blockade may contribute to renewed sensitivity to ICI in tumors with a T-cell-inflamed microenvironment.
2026-05-10 | The case for tumour-agnostic reimbursement of dual immunotherapy.
Barjesteh van Waalwijk van Doorn-Khosrovani and colleagues raise important questions about this recommendation for tumour-agnostic reimbursement of nivolumab and ipilimumab in Australia. We respectfully offer a counterpoint and endorse the broad coverage. In 2025, Pharmaceutical Benefits Advisory Committee (PBAC) proposed that prescribing nivolumab and ipilimumab be guided by clinical judgement informed by the best available evidence for undefined "immunotherapy-sensitive" advanced or metastatic cancers, and, in January 2026 made a similar recommendation for pembrolizumab. Patients with rare and ultra-rare cancers, including but not limited to angiosarcoma, alveolar soft part sarcoma, gestational trophoblastic disease, small cell carcinoma of the ovary (hypercalcemic type), and adrenocortical carcinoma, face profound unmet need. Within many of these diseases, a subset of patients derives substantial benefit from immunotherapy. For example, the SWOG DART (Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors) trial, the largest prospective evaluation of nivolumab plus ipilimumab in rare/ultra-rare cancers, demonstrated clinically meaningful activity across multiple histologies, without biomarker selection. Notably, some patients with metastatic disease remain disease-free years after treatment, outcomes rarely achieved with chemotherapy or supportive care in refractory settings. Therefore, concerns that biomarker-agnostic approval signals an evidentiary retreat overlook both our incomplete understanding immune sensitivity and the global access limits to comprehensive genomic testing. Importantly, clinical judgement is not arbitrary; it reflects training, experience, ethical reasoning, and patient-centred decision making. The question is whether demanding perfect evidence should prevent access to potentially transformative treatment. Grounded in evidence and expertise, PBAC's decision prioritises meaningful access to effective therapy for patients with few alternatives.
2026-05-04 | Pembrolizumab as an Off-Label Treatment of Facial Angiosarcoma: A Case Report.
of Case: We report an atypical case of cutaneous angiosarcoma presenting as unilateral nasal swelling in an elderly male. The lesion was unresponsive to topical and antibiotic therapy. Diagnosis was confirmed after surgical excision, and disease control was ultimately achieved through radiation and off-label use of pembrolizumab. Patient Info: A 79-year-old male with a history of squamous cell carcinoma presented with a 1–2 month history of erythematous, scaly swelling of the left nasal tip and ala. Family history was notable for nonmelanoma skin cancer. Initial differential diagnoses included contact dermatitis, rosacea, and rhinophyma. A shave biopsy revealed actinic damage but no malignancy. Following persistent symptoms and referral to plastic surgery, debulking surgery was performed. Histopathology confirmed cutaneous angiosarcoma. The patient declined wide local excision and instead underwent a 4-week course of hypofractionated radiation. Subsequent imaging showed regional lymph node recurrence. He declined biopsy and surgery, and was started on off-label pembrolizumab immunotherapy for disease control. Following immunotherapy, the patient experienced sustained remission with no clinical or radiographic evidence of disease four years post-treatment. Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. This case illustrates the importance of considering angiosarcoma in atypical facial lesions and demonstrates disease control using a non-traditional, patient-centered treatment strategy. Immunotherapy with pembrolizumab may offer a promising option in select patients, particularly those with contraindications to extensive surgery.
2026-03-01 | 170P AI-assisted systematic review and pooled analysis of anthracycline- versus taxane-based chemotherapy in advanced or metastatic angiosarcoma
Ridaforolimus were evaluated.PRRX1 gain and loss-of-function studies were performed in vitro and in vivo.Results: Treated tumors showed enrichment of stemness-related pathways, including epithelial-mesenchymal transition, and overexpression of SOX2, KLF4, and PRRX1, with increased Ng2+ MSC and fibroblast/chondrocyte progenitor signatures.PRRX1 knockdown reduced proliferation and increased doxorubicin sensitivity, whereas PRRX1 overexpression induced resistance in vitro and expanded the tumor-initiating cell population with a 7-fold increase in stem cell frequency by in vivo limitingdilution analysis.Treated tumors also exhibited activation of the PI3K-AKT-mTOR pathway and upregulation of IGF1 and IGF2, with elevated mTOR activity confirmed by phospho-S6 IHC.Combined IGF blockade (Xentuzumab) and mTOR inhibition (Ridaforolimus) significantly suppressed growth of chemotherapy-experienced tumors.Conclusions: CDS is driven by an undruggable fusion oncogene and rapidly develops chemoresistance.We show that chemotherapy induces IGF-PI3K-mTOR activation and a PRRX1-associated stemness program.Targeting IGF ligands and mTOR suppresses growth of chemotherapy-treated tumors, and PRRX1 acts as a key regulator of stemness and resistance.We have identified a targetable vulnerability in CDS, and further investigation of the link between IGF/mTOR signaling and PRRX1-driven stemness is required.
2026-02-16 | Postradiation angiosarcoma of the breast: a 25-year single-institution analysis of clinicopathological characteristics and immunohistochemical biomarker study.
We examined the clinicopathologic features and immunohistochemical expression of commonly tested biomarkers relevant to targeted therapy in postradiation angiosarcoma (PRAS) of the breast, aiming to better understand tumor biology and provide baseline data on tumor biomarkers to inform future therapeutic strategies for PRAS. Clinicopathologic features, outcomes, and the immunohistochemical expression of tumor biomarkers, including human epidermal growth factor receptor 2 (HER2), programmed death-ligand 1 (PD-L1), and DNA mismatch repair proteins (MMR), were analyzed in breast PRAS specimens diagnosed from 2000 to 2024. A total of 24 breast PRAS specimens from 18 patients were included in the study. All PRAS patients were female, with a median age of 72 years at the time of diagnosis. High-grade tumors were observed in 47%, 43%, and 65% of specimens according to Fédération Nationale des Centers de Lutte Contre le Cancer (FNCLCC), Donnell-Rosen, and Kuba scoring methods, respectively. Surgical intervention, adjuvant chemotherapy, and radiotherapy were administered to 100%, 29%, and 23% of patients, respectively. Five patients (29%) died from the disease during a median follow-up period of 37 months. Exploratory analysis showed there was no significant association between histologic grade and overall survival, regardless of the grading system used. All PRASs were MMR-proficient with a low number of tumor-infiltrating lymphocytes (TILs) and exhibited an absence of HER2 protein expression. PD-L1 was positive in seven (32%) specimens and positively correlated with stromal TILs. Our findings suggest that breast PRAS are genomically stable and poorly immunogenic, and are likely ineligible for any HER2-targeted therapies based on current approval criteria. A subset of specimens demonstrated PD-L1 positivity, underscoring the need for further investigation of immunotherapy with anti-PD1/PD-L1 immune checkpoint inhibitors as a potential treatment option.
proteins
2026-01-29 | Hepatic Angiosarcoma Mimicking Sinusoidal Obstruction Syndrome: A Case Report.
Hepatic angiosarcoma (HAS) is an uncommon malignant tumor that is often misdiagnosed because of its nonspecific presentation and overlapping imaging findings with benign vascular tumors. Sinusoidal obstruction syndrome (SOS) is a disorder of hepatic microcirculation, usually associated with myeloablative chemotherapy and hematopoietic stem cell transplantation. The clinical and pathological overlap between SOS and hepatic angiosarcoma may complicate the diagnosis of hepatic angiosarcoma, as illustrated by this case. We report the case of a sixty-year-old man who presented to the emergency department with progressive jaundice, abdominal discomfort, and weight loss exceeding 5% of his baseline. Laboratory workup revealed a predominantly cholestatic pattern, and serologies for viral, autoimmune, and metabolic liver diseases were negative. Also, there were no known risk factors for liver disease or SOS. Repeated imaging studies, including computed tomography (CT) and magnetic resonance imaging (MRI), revealed a large vascular hepatic lesion, suggestive of a benign lesion, and Doppler revealed findings compatible with SOS. Liver biopsy and hemodynamic studies indicated hepatic outflow obstruction. After clinical deterioration and expert multidisciplinary review of imaging and histology, malignant endothelial proliferation consistent with hepatic angiosarcoma was identified. Hepatic angiosarcoma can closely mimic SOS morphologically and hemodynamically. The absence of identifiable risk factors and the progressive evolution of hepatic changes should raise suspicion of malignancy, prompting multidisciplinary reassessment and careful histologic review.
2026-01-15 | Angiosarcoma developing in an ovarian fibroma: A case report.
Angiosarcomas are malignant vascular tumors primarily located in the dermis or soft tissues predominantly in the head and neck region. Ovarian localization is extremely rare, with only a few published cases. A 63-year-old patient was admitted to the hospital due to severe pain in the hypogastric and abdominal regions. The condition was interpreted as an acute abdomen and referred to surgery, and an ovarian tumor that measured 23cm×11cm×5cm and weighed 7500g was resected. Macroscopically, the cut surface showed two well-defined areas: one consisting of a matte white tissue with a fascicular appearance and firm consistency and the other hemorrhagic with necrotic areas. Histological examination revealed two patterns: one characterized by a proliferation of spindle-shaped cells without atypia, and the other by neoplastic proliferation with marked atypia, forming vascular channels or fascicles. The vascular component was positive for CD31, CD34, and ERG. The findings were interpreted as an angiosarcoma associated with a fibroma, and the patient died in the immediate postoperative period. The low incidence and ominous prognosis of primary or metastatic ovarian angiosarcomas have been confirmed. Regarding the histogenesis, a strong likelihood exists of a relationship with the ovary's rich vascular network. In both well-differentiated and poorly differentiated cases, it is crucial to consider differential diagnoses and perform immunohistochemical techniques, which can assist in achieving an accurate diagnosis. The association between fibroma and angiosarcoma is poorly understood and the histogenesis remains unclear.
2026-01-07 | Adult urologic sarcomas: a single institution experience over 25 years.
Genitourinary (GU) sarcomas are rare soft tissue malignancies, comprising around 2% of all GU cancers. Due to their rarity, limited data exist on optimal management and long-term outcomes. This study presents a 25-year single-institution experience, evaluating clinical presentation, treatment strategies, and survival outcomes, aims to identify trends over time and potential predictors of prognosis. A retrospective review was conducted of patients aged ≥18 years diagnosed with GU sarcomas at the University of Iowa Hospitals and Clinics (1998-2023). Data on tumor subtype, staging, histopathology, treatment modalities, and survival outcomes were analyzed. Kaplan-Meier analysis estimated recurrence-free survival (RFS) and overall survival (OS). Among 33 cases, the most common presentations in order of frequency were liposarcoma (LPS) (n = 15), leiomyosarcoma (LMS) (n = 12), rhabdomyosarcoma (RMS) (n = 5), and angiosarcoma (n = 1). Paratesticular tumors (n = 23) were most frequent, followed by bladder (n = 5), prostate (n = 2), and kidney (n = 2). The median age was 51 for LMS, 60 for LPS, and 24 for RMS. LMS had higher stage (66.67%), grade (83.33%), recurrence (25.00%), and mortality (41.67%) rates compared to LPS (recurrence: 13.33%, mortality: 20.00%). At 36 months, RFS was 63% (95% CI: 39%-79%), and OS was 81% (95% CI: 57%-92%) for the entire cohort. Follow-up duration was 19.9 months for LMS and 33.8 months for LPS. Surgical resection remains the mainstay of treatment for GU sarcomas. Margin status, tumor grade, and size are key prognostic factors. LMS carries the highest recurrence risk, and RMS exhibits aggressive progression. Further investigation into targeted therapies is warranted to improve outcomes.
2025-10-20 | Characteristics, Outcomes, and Risk Factors in Primary and Secondary Angiosarcoma: A Retrospective Cohort Study.
Angiosarcomas (ASs) are a heterogeneous subtype of soft tissue sarcomas. They can be subdivided into primary and secondary AS, with secondary AS being predominant, particularly those following radiotherapy. The aim of this study was first to analyze our patient cohort on a descriptive level and then to identify possible risk factors with regard to one and 5-year survival using logistic regression. The study was designed as a retrospective, single-center cohort study. All patients with histologically confirmed AS over 18 years of age were included in the study. Binary logistic regression was used for univariate analysis screening of continuous or dichotomous variables, respectively. For multivariate analysis, binary multivariate logistic regression was performed to assess independent associations between chosen variables and AS. A total of 39 patients were included in this study. 14 (35.9%) had primary and 25 (64%) had secondary AS. Women were more frequently affected (76.9%) than men (23.1%). The 1-year survival rate was 87.2%, and the 5-year survival rate was 51.3%. In the logistic regression analyses, nicotine consumption and a history of carcinoma were identified as significant factors influencing the 1-year survival rate. For the 5-year survival rate, only breast cancer was found to be a significant influencing factor in the univariate analysis. Based on univariate logistic regression, all variables with a p value of < 0.1 were chosen to be included into multivariate analysis. The multivariate analysis showed diabetes mellitus (p=0.067) with an association to influence the 5-year survival rate. We were able to show that the proportion of secondary ASs is predominant. These occur after radiation treatment of the breast. Diabetes mellitus may be associated with reduced 5-year survival, although this finding did not reach statistical significance and requires further investigation. Due to its small sample size, this study should be regarded more as hypothesis-generating.
2025-07-28 | Clinicopathological features and prognostic insights of cutaneous versus non-cutaneous angiosarcoma: A retrospective single-center cohort study.
Angiosarcoma is a rare, aggressive malignancy of endothelial origin, accounting for 1-2 % of soft tissue sarcomas. It occurs in cutaneous (C-AS) and non-cutaneous (NC-AS) forms, with distinct clinical behaviors and prognoses. While C-AS primarily affects the head and neck, NC-AS involves visceral organs such as the heart, liver, and breast. Despite advances in treatment, survival remains poor, and recurrence is common. This retrospective, single-center cohort study analyzed 30 patients diagnosed with angiosarcoma at the University Hospital Zurich (2000-2023). The study compared clinicopathological characteristics, survival outcomes, and prognostic factors influencing survival and recurrence. Patients were stratified into C-AS (n = 10) and NC-AS (n = 20) groups. Overall survival (OS) and recurrence-free survival (RFS) were assessed using Kaplan-Meier and Cox regression analyses. Both subtypes shared a poor prognosis, with an overall 5-year survival rate of 12 %. NC-AS had a higher early mortality rate, with a significantly worse median OS (9 vs. 36 months, p = 0.04), while early recurrence after treatment was more frequent in C-AS. High-grade tumor differentiation and metastases at diagnosis were independent predictors of poorer OS (p = 0.01; p = 0.04). Multimodal treatment, including surgery and radiotherapy, was associated with improved survival (p < 0.05). No clinicopathological factor showed a statistically significant association with RFS. C-AS and NC-AS exhibit distinct prognostic profiles, with NC-AS associated with a higher metastatic burden and worse outcomes. Prognostic factors such as tumor grade and metastases at diagnosis influence survival. Multimodal treatment strategies appear beneficial, though recurrence remains a major challenge. Further prospective studies are needed to refine therapeutic approaches and explore emerging targeted therapies.
cell therapies
2026-09-06 | Liver transplantation for pediatric liver tumors: a case report of hepatic angiosarcoma with literature review.
Pediatric hepatic angiosarcoma (HAS) is poorly understood. The lack of effective therapies for unresectable pediatric liver tumors creates a clinical imperative, positioning liver transplantation (LT) as a pivotal modality for exploring the roadmap to prolonged survival. We report the first Chinese pediatric case of HAS treated with LT. A 2-year-old boy was admitted to hospital due to abdominal pain and distension. Imaging findings were suggestive of possible malignancy, but initial biopsy yielded a diagnosed infantile hepatic hemangioma (IHH). Following living-donor LT for unresectable disease, explant pathology revealed an angiosarcoma arising within a hemangioma. The patient ultimately died from metastasis 21 months after LT. Our review of pediatric LT for liver tumors over 15 years identified nine pediatric HAS cases, only one of whom was correctly diagnosed pre-LT, with survival ranging from 3 to 66 months. Regarding other tumors, six cases of IHH were reported prior to 2010, without survival data. Recent studies on hepatoblastoma (HB) report a 5-year overall survival (OS) of 80-90%, and the prognosis for hepatocellular carcinoma (HCC) has markedly improved, with a combined 5-year OS of 74%. In the largest reported study, the 5-year OS rates for hepatic epithelioid hemangioendothelioma (HEH) and hepatic undifferentiated embryonal sarcoma (HUES) were 60.6% and 90.0%, respectively. Evidence for biliary tract rhabdomyosarcoma (BT-RMS) and malignant rhabdoid tumor (MRT) of the liver are anecdotal, and no pediatric cases of primary hepatic kaposiform hemangioendothelioma (KHE) treated with LT have been reported. Pediatric HAS is rare and difficult to distinguish from IHH; contrast-enhanced computed tomography/magnetic resonance imaging (CT/MRI) aids differentiation. For suspected malignant transformation, contrast-enhanced ultrasound (CEUS) and ultrasound-guided multi-site biopsy are recommended. No standardized treatment exists for pediatric HAS. Given the poor long-term survival, LT or combined chemotherapy remains exploratory but is the only option for unresectable tumors. The high survival rates of LT for HB and HCC offer a promising blueprint. With advances in surgical techniques and increased living donor availability, pediatric LT deserves more attention.
2026-07-06 | Double free flap reconstruction using the piggyback technique for multifocal scalp radiation necrosis: a case report.
Scalp angiosarcoma is a rare, aggressive malignancy with poor prognosis, particularly when multifocal. We report a 62-year-old woman with multifocal scalp angiosarcoma, a 3.5× 3.5 cm ulcerated vertex lesion within an approximately 10× 10 cm clinically abnormal vertex field and three 1× 1 cm temporal satellite lesions (two in the right temporal region and one in the left temporal region). Initial wide excision with frozen biopsy-guided margins and reconstruction using a rotation flap and split-thickness skin grafts was followed by adjuvant radiotherapy. At 7 months after completion of radiotherapy, radiation-induced necrosis with bone exposure developed in the frontal and occipital regions, with multiple biopsies negative for recurrence. Double free flap reconstruction (radial forearm free flap and adipofascial anterolateral thigh flap using a piggyback anastomosis) was performed 11 months after radiotherapy using a single recipient vessel pair. Venous congestion on postoperative day 1 was successfully revised with vein graft interposition, resulting in complete flap survival. The patient was disease-free with stable flap coverage at the latest follow-up, more than 2.5 years after radiotherapy. This case demonstrates that double free flap reconstruction using a piggyback technique can be a safe and effective reconstructive option for extensive irradiated scalp defects in multifocal angiosarcoma.
2026-04-09 | From Palliation After Angiosarcoma Resection to Totally Autologous Aesthetic Breast Reconstruction Combining Kiss Latissimus Dorsi Flap and Contralateral Breast Sharing Internal Mammary Artery Perforator Flap: A Case Report.
Angiosarcoma of the breast is a rare but aggressive disease that often requires wide-margin resection resulting in massive thoracic defects. In this report we present the unique case of a patient who underwent primary chest wall reconstruction with palliative intent using Kiss Latissimus Dorsi (LD) Flap after resection of a huge angiosarcoma of the right breast, followed 3 years later by a secondary shift to totally autologous aesthetic breast reconstruction which was achieved by combining the first flap with breast-sharing internal mammary artery perforator (IMAP) flap and simultaneous contralateral breast reduction. The defect after extended mastectomy, measuring 24 × 18 cm, was resurfaced with a Kiss LD flap designed with two skin paddles of 24 × 13 and 14 × 5 cm, respectively. Three years after the initial reconstruction, the patient was tumor-free and required breast symmetrization. Therefore, a left reduction mammaplasty with simultaneous breast sharing Internal Mammary Artery Perforator (IMAP) flap was performed, augmenting the hypoplastic right breast with the entire lower pole of the contralateral side, transferring an 18 × 9 cm flap based on the fifth IMAP. Vascular safety of the procedure was guaranteed by intra-operative indocyanine green angiography evaluation. Recovery was uneventful and the 6 months post-operative follow-up confirmed flap integration, volume symmetry and high aesthetic satisfaction. This report underlines the versatility of this innovative combination of flaps in complex reconstructive scenarios where complex microsurgical transfers are contraindicated.
2025-11-22 | Primary cerebellar angiosarcoma: a rare case report and literature review.
Angiosarcoma is a highly aggressive malignant mesenchymal tumor, accounting for less than 1% of all soft tissue sarcomas. While most cases arise in the skin, soft tissues, liver, or heart, primary intracranial angiosarcoma (PIA) is an exceptionally rare and distinct clinicopathological entity. Due to nonspecific clinical presentations and imaging features, PIA poses significant diagnostic and therapeutic challenges. It is characterized by aggressive behavior, with high rates of recurrence and metastasis, resulting in poor overall prognosis. Here, we report a rare case of primary cerebellar angiosarcoma and review its epidemiology, pathogenesis, diagnostic considerations, and treatment strategies. A 53-year-old woman presented with progressive dizziness, occipital headache, nausea, and vomiting for five days. MRI revealed a hemorrhagic, highly vascular right cerebellar mass initially suspected to be a hemangioblastoma. Prior to surgery, her condition deteriorated, and emergency CT showed acute tumor hemorrhage, necessitating urgent resection. Intraoperatively, the tumor was highly vascular and poorly circumscribed, suggestive of a high-grade sarcoma, later confirmed as angiosarcoma. Despite gross total resection, the patient remained comatose due to brainstem compression. Follow-up CT on day 20 revealed rapid tumor recurrence. Given the poor prognosis, the family opted for palliative care, and the patient subsequently passed away. PIA is a rare and aggressive tumor with a poor prognosis, characterized by rapid progression and recurrence. Early detection and surgical resection are essential, but effective adjuvant therapies remain limited. A multidisciplinary approach is crucial to improve outcomes, and further research is needed to refine therapeutic strategies and enhance survival.
2025-02-06 | Usefulness of Dorsal Scapular Artery Perforator Flap for Donor Site Closure After Harvesting Latissimus Dorsi Flap/Thoracodorsal Artery Perforator Flap: A Report of Three Cases.
Reducing donor-site morbidity is crucial in free-flap reconstruction, with the primary goals of achieving dermis-to-dermis closure and avoiding skin grafts. Although latissimus dorsi (LD) myocutaneous and thoracodorsal artery perforator (TDAP) flaps usually allow primary closure, larger flap harvests may require skin grafts, potentially leading to undesirable outcomes. In such situations, local flap transfer can be considered for donor-site closure, yet there are few reports on this. This report aims to present cases where a dorsal scapular artery perforator (DSAP) rotational flap was used to close donor defects following large TDAP/LD flap harvests. The first case involved a 16-year-old girl who required a below-knee amputation due to an acute thrombotic event, leaving a 20 × 20 cm2-sized stump defect. Bilateral LD myocutaneous flaps were harvested, with the right donor site closed primarily, while a 20 × 9 cm2-sized defect remained on the left. A 22 × 9 cm2-sized DSAP flap, based on three perforators, was rotated to close the defect, showing excellent outcomes at 12 months. In the second and third cases, 75- and 78-year-old male patients with scalp angiosarcoma underwent wide excision and reconstruction with TDAP flaps, leaving 14 × 6 cm2 and 14 × 7 cm2-sized donor defects. DSAP flaps, 15 × 7 cm2 and 15 × 8 cm2 in size, were rotated based on nearby perforators to close these areas. Both patients showed no morbidities at postoperative 8 and 2 months, respectively. When primary closure of the donor site is challenging after harvesting large TDAP /LD flaps, a DSAP rotational flap can provide a valuable option for minimizing donor-site morbidity.
other
2026-07-31 | Management of Primary Hepatic Angiosarcoma: A Comprehensive Review.
Primary hepatic angiosarcoma (PHA) is the most common primary malignant mesenchymal tumour of the liver in adults, accounting for only 0.1-2% of all primary hepatic malignancies. It carries a median overall survival of 6-9 months, driven by aggressive biology, non-specific presentation, and almost universal late-stage diagnosis. No disease-specific tumour markers, pathognomonic imaging features, or PHA-exclusive prospective trials exist. A comprehensive narrative review was performed using PubMed, Embase, Cochrane Library, and Scopus. Search terms encompassed epidemiology, pathology, molecular biology, imaging, surgical and systemic management, and prognosis of PHA. Studies published up to June 2026 were included, with preference given to systematic reviews, multicenter cohorts, and prospective trials. ERG is the most sensitive and specific immunohistochemical marker for PHA (100% sensitivity vs. 79-87% for CD31/CD34). Surgical R0 resection - achievable in fewer than 25% of patients - is the only potentially curative modality, with median overall survival of 17.2 months post-resection versus 3.7 months without surgery. Liver transplantation is an absolute contraindication. For unresectable disease, cabozantinib plus nivolumab (Alliance A091902) produced an overall response rate of 59% in taxane-pretreated patients. Gemcitabine demonstrates second-line activity with an overall response rate of 38% and median overall survival of 9.9 months. Recurrent alterations in TP53, KDR, PIK3CA, PTPRB, and PLCG1 provide a rationale for routine molecular profiling, though no actionable target has been prospectively validated. Surgical resection where feasible, ICI-TKI combination therapy for unresectable disease, and routine molecular profiling represent the current cornerstones of PHA management. Progress requires international collaborative registries, multi-institutional trials, and specialist multidisciplinary care for all patients.
2026-01-23 | Phase II trial, multicenter, first-line paclitaxel-avelumab treatment of inoperable angiosarcoma (KCSG UN 18-15).
Angiosarcomas are rare tumors of vascular or lymphatic origin with clinically heterogeneous presentation and behavior, predominantly originating in the skin of the head and neck and the breast area. Given their location and/or rapid progression, surgical resection is frequently not feasible, even in those with localized diseases. Conventional cytotoxic chemotherapy has proven largely ineffective against angiosarcoma. In this prospective, phase II study, we evaluated the efficacy and toxicity of paclitaxel plus avelumab as first-line therapy for unresectable angiosarcoma. This study included patients with unresectable locally advanced or metastatic angiosarcomas, who did not receive systemic treatment. Paclitaxel (80 mg/m2) was intravenously infused on days 1, 8, and 15 of every 28-days cycle; avelumab (10 mg/kg) was intravenously infused every 2 weeks. Treatment continued until disease progression, unacceptable toxicity, death, or withdrawal of consent, whichever occurred first. The primary endpoint was the objective response rate (ORR); secondary endpoints included overall survival (OS), progression-free survival (PFS), and safety profiles. The final analysis included 32 patients (21 male and 11 female). The median age was 63.5 years (range 27-82 years). The ORR was 50.0% (n = 16), including one complete response. Median OS and PFS were 14.5 [95% confidence interval (CI) 9.4-24.6 months] and 6.0 (95% CI 5.4-9.5 months), respectively. Among patients who received at least one treatment dose (n = 33), adverse events (AEs) of any grade were reported in 90.9% (n = 30), and severe AEs in 12.1% (n = 4), including one death. The most common hematologic and non-hematologic AEs were neutropenia (n = 13, 39.4%) and pain (n = 12, 36.4%). Immune-related pneumonitis was reported in two patients. Avelumab plus weekly paclitaxel demonstrated preliminary evidence of efficacy and tolerability in inoperable angiosarcoma. Given the rarity of angiosarcoma, further multicenter, collaborative, and translational studies are warranted.
2025-11-10 | Splenic angiosarcoma with hepatic and cardiac metastases: a case report and literature review.
Splenic angiosarcoma (SA) is an rare malignant tumor in clinical practice, characterized by high malignancy, atypical early symptoms, and a dismal prognosis. In recent years, growing attention has been focused on its diagnosis and treatment. This report describes a patient who was admitted to the hospital due to left upper abdominal pain. Contrast-enhanced CT indicated unevenly enhanced tumors in the spleen, liver, and right atrium. The patient was treated with laparoscopic splenectomy + partial hepatectomy and was diagnosed based on postoperative pathology and immunohistochemistry (positive for CD31 and CD34). After the operation, anlotinib + toripalimab targeted immunotherapy was used, and the patient recovered well. This case suggests that SA is prone to metastasis in the early stage. Clinically, the diagnosis of SA requires a combination of pathological diagnosis and imaging diagnosis, and postoperative targeted immunotherapy may improve the prognosis.
2025-08-31 | Primary pulmonary artery sarcoma treated with simultaneous pulmonary valve replacement, artificial vessel replacement, and total right pneumonectomy: a case report.
Primary pulmonary artery sarcoma progresses extremely rapidly and has a poor prognosis. This condition is managed with surgical resection and multimodality therapy. However, standardized treatment is not available. A 54-year-old woman was brought to the emergency department because of chest pain and worsening dyspnea that had developed in the past month. Contrast-enhanced computed tomography scan revealed severe stenosis extending from the pulmonary artery trunk to the right pulmonary artery due to an embolic substance. Because primary pulmonary artery sarcoma was suspected, emergency surgery was performed to improve the patient's symptoms. In addition to maximal tumor resection, pulmonary artery valve replacement, artificial vessel replacement, and right total pneumonectomy were performed. Based on the assessment performed using the specimen collected perioperatively, a pathological diagnosis of angiosarcoma of the right pulmonary artery was made. The patient was discharged on postoperative day 17 with a good postoperative course. However, because of dyspnea, she was readmitted to the hospital on day 85. Tumor recurrence was noted, and chemotherapy was initiated. The patient developed cardiac failure and died on postoperative day 119. A pathological postmortem examination was performed. Metastatic lesions were found in the pericardial sac, left lung, right chest wall and pleura, and mediastinum. In the present case, postoperative recurrence was observed despite maximal resection of the surrounding tissues with tumor invasion and simultaneous reconstruction. Chemotherapy was initiated but was ineffective. Gene panel testing can help identify novel treatment options for patients with neoplastic diseases without standardized treatment. In addition, preparations should be made before surgery.
2025-03-09 | Angiosarcoma: Role of Immunotherapy.
Recent clinical trials have investigated immune checkpoint inhibitors (ICIs) alone, in combination with tyrosine kinase inhibitors (TKIs), or with chemotherapy in angiosarcoma, yielding mixed results across subtypes. Single-agent ICI therapy, such as cemiplimab (ORR 27.8%), has shown responses primarily in UV- and radiation-associated angiosarcomas, likely due to their higher tumor mutation burden (TMB), while dual ICI therapy (SWOG S1609, ORR 25%) suggests potential benefit but remains limited in cutaneous disease. ICI-TKI combinations, such as cabozantinib plus nivolumab (Alliance A091902, second-line, ORR 59%), demonstrated significant efficacy in both cutaneous and non-cutaneous angiosarcomas, suggesting anti-angiogenic therapy may enhance ICI responses in tumors historically resistant to checkpoint blockade. ICI-chemotherapy combinations have been less consistent. The Alliance A091902 first-line trial (paclitaxel ± nivolumab) found no overall PFS benefit, though scalp/face angiosarcoma patients appeared to fare better, raising the question of whether ICI alone might be equally effective in this subset. The South Korean paclitaxel + avelumab trial (ORR 50%) showed promising response rates, but the lack of detailed subgroup analysis limits interpretation. Other chemotherapy-ICI combinations, such as doxorubicin plus pembrolizumab, have shown isolated responses but require further study in larger cohorts. Moving forward, better biomarkers are critical for identifying which patients benefit most from ICIs, and while TKI-ICI combinations appear to hold the most promise, chemotherapy-ICI strategies need further refinement to optimize sequencing and patient selection in angiosarcoma treatment.
small molecules
2026-09-06 | Primary splenic angiosarcoma presenting with multiple bone destruction as the initial manifestation: a case report and narrative review.
Primary splenic angiosarcoma (PSA) is an extremely rare and highly aggressive malignant vascular tumor. Its clinical presentation is often nonspecific, and the diagnosis is frequently delayed. Osseous metastasis as the initial clinical manifestation is particularly uncommon, posing significant diagnostic challenges. A critical yet underappreciated phenomenon is the discordance between histological differentiation and biological behavior in angiosarcoma: well-differentiated tumors can exhibit highly aggressive clinical courses. Furthermore, biopsy of vascular tumors carries a substantial risk of intractable hemorrhage, a complication that is often underestimated. We report the case of a 56-year-old female who presented with a 20-day history of lower back and right hip pain. Imaging revealed multiple osteolytic lesions involving the thoracolumbar vertebrae, pelvis, and sacrum, as well as multiple nodular lesions in the spleen. The initial clinical suspicion favored metastatic disease of unknown primary. Ultrasound-guided needle biopsy of the right iliac lesion yielded abundant hemorrhage but no definitive malignant tissue on initial pathology. Subsequent open biopsy of the L2 spinous process and right iliac bone resulted in life-threatening hemorrhage, necessitating emergency digital subtraction angiography (DSA) and super-selective embolization. Histopathological examination of the surgical specimens revealed a mixed-type vascular neoplasm with partially well-differentiated angiosarcomatous changes, with immunohistochemical positivity for CD31, CD34, ERG, and Fli-1. Remarkably, follow-up pelvic MRI performed only 21 days after the initial scan demonstrated interval enlargement of multiple bone lesions, indicating extraordinarily rapid disease progression despite the high-grade histological appearance. This case highlights several critical clinical considerations for angiosarcoma: (1) When both splenic and skeletal lesions coexist, suspected primary splenic angiosarcoma with bone metastases should be strongly considered; (2) The histological grade may be discordant with biological behavior-high-grade tumors can exhibit highly aggressive courses; (3) Imaging features can mimic benign vascular tumors such as hemangioma; (4) Biopsy of vascular tumors carries a substantial risk of intractable hemorrhage, and pre-procedural angiographic assessment should be considered. This case underscores the importance of integrating clinical, imaging, and pathological findings for accurate diagnosis and management of this rare malignancy.
2026-08-31 | Pathologic Complete Response Following Frailty-Adjusted Neoadjuvant Paclitaxel in an 88-Year-Old Patient With Radiation-Associated Breast Angiosarcoma.
Radiation-associated breast angiosarcoma is a rare but highly aggressive malignancy with limited evidence to guide optimal management in elderly patients. We present the case of an 88-year-old woman with a history of stage IIIA estrogen receptor-positive invasive ductal carcinoma of the right breast treated with breast-conserving surgery, chemotherapy, radiation therapy, and endocrine therapy in 2014. Approximately 11 years after completing radiation therapy, she developed progressive right breast firmness, violaceous skin discoloration, and persistent ecchymotic changes concerning for vascular malignancy. Imaging demonstrated diffuse skin thickening without a discrete mass, and punch biopsy of skin changes confirmed high-grade angiosarcoma with MYC amplification. Staging studies showed no distant metastatic disease. Given her advanced age, frailty, and comorbidities, she underwent neoadjuvant weekly paclitaxel with initial dose reduction followed by response- and tolerance-guided dose escalation. Despite treatment interruptions related to frailty and fall-related injuries, she completed 12 cycles and subsequently underwent right simple mastectomy. Final pathology demonstrated no residual angiosarcoma, consistent with a pathologic complete response. This case highlights the potential efficacy of frailty-adjusted neoadjuvant paclitaxel in elderly patients with radiation-associated breast angiosarcoma and supports individualized neoadjuvant strategies for patients who are not candidates for standard multi-agent chemotherapy.
2026-08-23 | Giant Breast Angiosarcoma in a 20-Year-Old Young Adult Female: A Case Report and Review of Literature.
Primary breast angiosarcoma (PAS) is an extremely rare vascular malignancy that typically presents as a breast mass or cutaneous changes. Given its highly aggressive nature, rapid progression, and early metastasis that result in a poor prognosis, coupled with a lack of standardized treatment guidelines, there is an urgent need to develop novel, effective therapeutic strategies. This report focused on a 20-year-old Uyghur woman presenting with a painless, rapidly enlarging mass in the left breast. Staging evaluation with breast MRI, whole-body contrast-enhanced CT, and bone scintigraphy revealed a large (10 × 7 cm) primary tumor and bilateral axillary lymphadenopathy suspicious for metastasis, with no evidence of distant disease (cT3N1M0, stage III). The high-grade angiosarcoma was confirmed through a core needle biopsy of the breast mass. With no remarkable medical or family history of breast cancer, the patient underwent complete surgical resection of the breast angiosarcoma, which provided a definitive pathological diagnosis. Given negative lymph node biopsy results, axillary lymph node dissection was not performed. Postoperatively, the patient received adjuvant chemotherapy with paclitaxel and bevacizumab. The regimen was well tolerated, and no disease recurrence was observed during follow-up. This case exemplified a practical management strategy for PAS, emphasizing the key role of clinical presentation and conventional imaging in guiding surgical decisions, particularly in resource-limited settings. As a rare, highly malignant tumor, PAS requires early diagnosis and multidisciplinary management, including thorough metastasis evaluation and personalized therapy, to optimize survival and quality of life.
2026-08-08 | Primary adrenal epithelioid angiosarcoma: An extraordinarily rare malignancy with a challenging diagnosis.
Adrenal epithelioid angiosarcoma is an exceedingly rare vascular endothelial neoplasm, accounting for a minute fraction of sarcomas with fewer than 60 cases reported in the medical literature. We report the case of a 70-year-old female with primary adrenal epithelioid angiosarcoma, presenting with a rapidly enlarging right adrenal mass and associated severe right upper quadrant pain. Biochemical testing revealed that the mass was nonfunctional, and contrast-enhanced computed tomography revealed a large, heterogeneously enhancing lesion with inferior vena cava compression. Given rapid interval growth and severe symptoms, she underwent laparoscopic adrenalectomy, and histopathology with immunohistochemistry confirmed a diagnosis of primary adrenal epithelioid angiosarcoma. Postoperative management included paclitaxel chemotherapy, and the patient has remained disease-free for 2 years post-operation. This case emphasizes that the clinical and imaging features of adrenal epithelioid angiosarcoma are nonspecific, and definitive diagnosis requires surgical pathology with vascular marker staining to avoid misclassification as metastatic carcinoma. Timely resection in combination with chemotherapy can yield favorable outcomes despite this tumor's aggressive behavior and overall poor prognosis.
2026-08-08 | Multimodality imaging features of primary mammary angiosarcoma: a case report with delayed osseous metastases.
Primary angiosarcoma of the breast is an uncommon and aggressive vascular malignancy that typically affects young women and poses diagnostic and therapeutic challenges. We report the case of a 26-year-old nulliparous woman who presented with progressive left breast swelling over approximately 18 months. Clinical examination revealed diffuse tender swelling without skin discoloration, nipple changes, or palpable lymphadenopathy. Ultrasonography demonstrated a complex cystic mass with thick septations and increased vascularity, while mammography showed global asymmetry of the left breast without suspicious calcifications. Breast MRI revealed a large 8.4 cm heterogeneously enhancing mass in the upper outer quadrant with possible focal skin involvement, but no chest wall invasion or nodal disease. Initial staging with 18F-FDG PET/CT showed no distant metastases. The patient underwent left nipple-sparing mastectomy, and histopathological examination confirmed intermediate-grade primary mammary angiosarcoma, FNCLCC grade 2. Subsequent re-excision achieved clear surgical margins. She received adjuvant ifosfamide-epirubicin chemotherapy and radiotherapy. Initial follow-up imaging showed no residual or recurrent disease. However, 14 months after completion of treatment, follow-up 18F-FDG PET/CT demonstrated new FDG-avid lytic lesions in the sacral and iliac bones, and Breast MRI revealed a small enhancing sternal lesion, consistent with delayed osseous metastatic disease. The patient was treated with palliative radiotherapy and systemic therapy. This case highlights the diagnostic complexity of primary breast angiosarcoma, its potential for delayed hematogenous osseous spread, and the value of multimodality imaging in diagnosis, staging, treatment planning, and surveillance.
antibodies
2026-08-01 | Immune checkpoint inhibitor rechallenge after pazopanib treatment in primary pulmonary angiosarcoma: A case report.
Primary pulmonary angiosarcoma is a rare malignancy with limited therapeutic options. We report a case of primary pulmonary angiosarcoma in which tumor regression was achieved with immune checkpoint inhibitor (ICI) rechallenge following pazopanib therapy. The tumor showed strong PD-L1 expression and infiltration of CD3+, CD4+, and CD8+ T cells without CD20+ B cells. After disease control with atezolizumab, pazopanib induced tumor shrinkage but was followed by disease progression. Pembrolizumab rechallenge resulted in tumor regression and sustained benefit. This case suggests that sequential VEGF-targeted therapy and PD-1 blockade may contribute to renewed sensitivity to ICI in tumors with a T-cell-inflamed microenvironment.
2026-05-10 | The case for tumour-agnostic reimbursement of dual immunotherapy.
Barjesteh van Waalwijk van Doorn-Khosrovani and colleagues raise important questions about this recommendation for tumour-agnostic reimbursement of nivolumab and ipilimumab in Australia. We respectfully offer a counterpoint and endorse the broad coverage. In 2025, Pharmaceutical Benefits Advisory Committee (PBAC) proposed that prescribing nivolumab and ipilimumab be guided by clinical judgement informed by the best available evidence for undefined "immunotherapy-sensitive" advanced or metastatic cancers, and, in January 2026 made a similar recommendation for pembrolizumab. Patients with rare and ultra-rare cancers, including but not limited to angiosarcoma, alveolar soft part sarcoma, gestational trophoblastic disease, small cell carcinoma of the ovary (hypercalcemic type), and adrenocortical carcinoma, face profound unmet need. Within many of these diseases, a subset of patients derives substantial benefit from immunotherapy. For example, the SWOG DART (Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors) trial, the largest prospective evaluation of nivolumab plus ipilimumab in rare/ultra-rare cancers, demonstrated clinically meaningful activity across multiple histologies, without biomarker selection. Notably, some patients with metastatic disease remain disease-free years after treatment, outcomes rarely achieved with chemotherapy or supportive care in refractory settings. Therefore, concerns that biomarker-agnostic approval signals an evidentiary retreat overlook both our incomplete understanding immune sensitivity and the global access limits to comprehensive genomic testing. Importantly, clinical judgement is not arbitrary; it reflects training, experience, ethical reasoning, and patient-centred decision making. The question is whether demanding perfect evidence should prevent access to potentially transformative treatment. Grounded in evidence and expertise, PBAC's decision prioritises meaningful access to effective therapy for patients with few alternatives.
2026-05-04 | Pembrolizumab as an Off-Label Treatment of Facial Angiosarcoma: A Case Report.
of Case: We report an atypical case of cutaneous angiosarcoma presenting as unilateral nasal swelling in an elderly male. The lesion was unresponsive to topical and antibiotic therapy. Diagnosis was confirmed after surgical excision, and disease control was ultimately achieved through radiation and off-label use of pembrolizumab. Patient Info: A 79-year-old male with a history of squamous cell carcinoma presented with a 1–2 month history of erythematous, scaly swelling of the left nasal tip and ala. Family history was notable for nonmelanoma skin cancer. Initial differential diagnoses included contact dermatitis, rosacea, and rhinophyma. A shave biopsy revealed actinic damage but no malignancy. Following persistent symptoms and referral to plastic surgery, debulking surgery was performed. Histopathology confirmed cutaneous angiosarcoma. The patient declined wide local excision and instead underwent a 4-week course of hypofractionated radiation. Subsequent imaging showed regional lymph node recurrence. He declined biopsy and surgery, and was started on off-label pembrolizumab immunotherapy for disease control. Following immunotherapy, the patient experienced sustained remission with no clinical or radiographic evidence of disease four years post-treatment. Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. This case illustrates the importance of considering angiosarcoma in atypical facial lesions and demonstrates disease control using a non-traditional, patient-centered treatment strategy. Immunotherapy with pembrolizumab may offer a promising option in select patients, particularly those with contraindications to extensive surgery.
2026-03-01 | 170P AI-assisted systematic review and pooled analysis of anthracycline- versus taxane-based chemotherapy in advanced or metastatic angiosarcoma
Ridaforolimus were evaluated.PRRX1 gain and loss-of-function studies were performed in vitro and in vivo.Results: Treated tumors showed enrichment of stemness-related pathways, including epithelial-mesenchymal transition, and overexpression of SOX2, KLF4, and PRRX1, with increased Ng2+ MSC and fibroblast/chondrocyte progenitor signatures.PRRX1 knockdown reduced proliferation and increased doxorubicin sensitivity, whereas PRRX1 overexpression induced resistance in vitro and expanded the tumor-initiating cell population with a 7-fold increase in stem cell frequency by in vivo limitingdilution analysis.Treated tumors also exhibited activation of the PI3K-AKT-mTOR pathway and upregulation of IGF1 and IGF2, with elevated mTOR activity confirmed by phospho-S6 IHC.Combined IGF blockade (Xentuzumab) and mTOR inhibition (Ridaforolimus) significantly suppressed growth of chemotherapy-experienced tumors.Conclusions: CDS is driven by an undruggable fusion oncogene and rapidly develops chemoresistance.We show that chemotherapy induces IGF-PI3K-mTOR activation and a PRRX1-associated stemness program.Targeting IGF ligands and mTOR suppresses growth of chemotherapy-treated tumors, and PRRX1 acts as a key regulator of stemness and resistance.We have identified a targetable vulnerability in CDS, and further investigation of the link between IGF/mTOR signaling and PRRX1-driven stemness is required.
2026-02-16 | Postradiation angiosarcoma of the breast: a 25-year single-institution analysis of clinicopathological characteristics and immunohistochemical biomarker study.
We examined the clinicopathologic features and immunohistochemical expression of commonly tested biomarkers relevant to targeted therapy in postradiation angiosarcoma (PRAS) of the breast, aiming to better understand tumor biology and provide baseline data on tumor biomarkers to inform future therapeutic strategies for PRAS. Clinicopathologic features, outcomes, and the immunohistochemical expression of tumor biomarkers, including human epidermal growth factor receptor 2 (HER2), programmed death-ligand 1 (PD-L1), and DNA mismatch repair proteins (MMR), were analyzed in breast PRAS specimens diagnosed from 2000 to 2024. A total of 24 breast PRAS specimens from 18 patients were included in the study. All PRAS patients were female, with a median age of 72 years at the time of diagnosis. High-grade tumors were observed in 47%, 43%, and 65% of specimens according to Fédération Nationale des Centers de Lutte Contre le Cancer (FNCLCC), Donnell-Rosen, and Kuba scoring methods, respectively. Surgical intervention, adjuvant chemotherapy, and radiotherapy were administered to 100%, 29%, and 23% of patients, respectively. Five patients (29%) died from the disease during a median follow-up period of 37 months. Exploratory analysis showed there was no significant association between histologic grade and overall survival, regardless of the grading system used. All PRASs were MMR-proficient with a low number of tumor-infiltrating lymphocytes (TILs) and exhibited an absence of HER2 protein expression. PD-L1 was positive in seven (32%) specimens and positively correlated with stromal TILs. Our findings suggest that breast PRAS are genomically stable and poorly immunogenic, and are likely ineligible for any HER2-targeted therapies based on current approval criteria. A subset of specimens demonstrated PD-L1 positivity, underscoring the need for further investigation of immunotherapy with anti-PD1/PD-L1 immune checkpoint inhibitors as a potential treatment option.
proteins
2026-01-29 | Hepatic Angiosarcoma Mimicking Sinusoidal Obstruction Syndrome: A Case Report.
Hepatic angiosarcoma (HAS) is an uncommon malignant tumor that is often misdiagnosed because of its nonspecific presentation and overlapping imaging findings with benign vascular tumors. Sinusoidal obstruction syndrome (SOS) is a disorder of hepatic microcirculation, usually associated with myeloablative chemotherapy and hematopoietic stem cell transplantation. The clinical and pathological overlap between SOS and hepatic angiosarcoma may complicate the diagnosis of hepatic angiosarcoma, as illustrated by this case. We report the case of a sixty-year-old man who presented to the emergency department with progressive jaundice, abdominal discomfort, and weight loss exceeding 5% of his baseline. Laboratory workup revealed a predominantly cholestatic pattern, and serologies for viral, autoimmune, and metabolic liver diseases were negative. Also, there were no known risk factors for liver disease or SOS. Repeated imaging studies, including computed tomography (CT) and magnetic resonance imaging (MRI), revealed a large vascular hepatic lesion, suggestive of a benign lesion, and Doppler revealed findings compatible with SOS. Liver biopsy and hemodynamic studies indicated hepatic outflow obstruction. After clinical deterioration and expert multidisciplinary review of imaging and histology, malignant endothelial proliferation consistent with hepatic angiosarcoma was identified. Hepatic angiosarcoma can closely mimic SOS morphologically and hemodynamically. The absence of identifiable risk factors and the progressive evolution of hepatic changes should raise suspicion of malignancy, prompting multidisciplinary reassessment and careful histologic review.
2026-01-15 | Angiosarcoma developing in an ovarian fibroma: A case report.
Angiosarcomas are malignant vascular tumors primarily located in the dermis or soft tissues predominantly in the head and neck region. Ovarian localization is extremely rare, with only a few published cases. A 63-year-old patient was admitted to the hospital due to severe pain in the hypogastric and abdominal regions. The condition was interpreted as an acute abdomen and referred to surgery, and an ovarian tumor that measured 23cm×11cm×5cm and weighed 7500g was resected. Macroscopically, the cut surface showed two well-defined areas: one consisting of a matte white tissue with a fascicular appearance and firm consistency and the other hemorrhagic with necrotic areas. Histological examination revealed two patterns: one characterized by a proliferation of spindle-shaped cells without atypia, and the other by neoplastic proliferation with marked atypia, forming vascular channels or fascicles. The vascular component was positive for CD31, CD34, and ERG. The findings were interpreted as an angiosarcoma associated with a fibroma, and the patient died in the immediate postoperative period. The low incidence and ominous prognosis of primary or metastatic ovarian angiosarcomas have been confirmed. Regarding the histogenesis, a strong likelihood exists of a relationship with the ovary's rich vascular network. In both well-differentiated and poorly differentiated cases, it is crucial to consider differential diagnoses and perform immunohistochemical techniques, which can assist in achieving an accurate diagnosis. The association between fibroma and angiosarcoma is poorly understood and the histogenesis remains unclear.
2026-01-07 | Adult urologic sarcomas: a single institution experience over 25 years.
Genitourinary (GU) sarcomas are rare soft tissue malignancies, comprising around 2% of all GU cancers. Due to their rarity, limited data exist on optimal management and long-term outcomes. This study presents a 25-year single-institution experience, evaluating clinical presentation, treatment strategies, and survival outcomes, aims to identify trends over time and potential predictors of prognosis. A retrospective review was conducted of patients aged ≥18 years diagnosed with GU sarcomas at the University of Iowa Hospitals and Clinics (1998-2023). Data on tumor subtype, staging, histopathology, treatment modalities, and survival outcomes were analyzed. Kaplan-Meier analysis estimated recurrence-free survival (RFS) and overall survival (OS). Among 33 cases, the most common presentations in order of frequency were liposarcoma (LPS) (n = 15), leiomyosarcoma (LMS) (n = 12), rhabdomyosarcoma (RMS) (n = 5), and angiosarcoma (n = 1). Paratesticular tumors (n = 23) were most frequent, followed by bladder (n = 5), prostate (n = 2), and kidney (n = 2). The median age was 51 for LMS, 60 for LPS, and 24 for RMS. LMS had higher stage (66.67%), grade (83.33%), recurrence (25.00%), and mortality (41.67%) rates compared to LPS (recurrence: 13.33%, mortality: 20.00%). At 36 months, RFS was 63% (95% CI: 39%-79%), and OS was 81% (95% CI: 57%-92%) for the entire cohort. Follow-up duration was 19.9 months for LMS and 33.8 months for LPS. Surgical resection remains the mainstay of treatment for GU sarcomas. Margin status, tumor grade, and size are key prognostic factors. LMS carries the highest recurrence risk, and RMS exhibits aggressive progression. Further investigation into targeted therapies is warranted to improve outcomes.
2025-10-20 | Characteristics, Outcomes, and Risk Factors in Primary and Secondary Angiosarcoma: A Retrospective Cohort Study.
Angiosarcomas (ASs) are a heterogeneous subtype of soft tissue sarcomas. They can be subdivided into primary and secondary AS, with secondary AS being predominant, particularly those following radiotherapy. The aim of this study was first to analyze our patient cohort on a descriptive level and then to identify possible risk factors with regard to one and 5-year survival using logistic regression. The study was designed as a retrospective, single-center cohort study. All patients with histologically confirmed AS over 18 years of age were included in the study. Binary logistic regression was used for univariate analysis screening of continuous or dichotomous variables, respectively. For multivariate analysis, binary multivariate logistic regression was performed to assess independent associations between chosen variables and AS. A total of 39 patients were included in this study. 14 (35.9%) had primary and 25 (64%) had secondary AS. Women were more frequently affected (76.9%) than men (23.1%). The 1-year survival rate was 87.2%, and the 5-year survival rate was 51.3%. In the logistic regression analyses, nicotine consumption and a history of carcinoma were identified as significant factors influencing the 1-year survival rate. For the 5-year survival rate, only breast cancer was found to be a significant influencing factor in the univariate analysis. Based on univariate logistic regression, all variables with a p value of < 0.1 were chosen to be included into multivariate analysis. The multivariate analysis showed diabetes mellitus (p=0.067) with an association to influence the 5-year survival rate. We were able to show that the proportion of secondary ASs is predominant. These occur after radiation treatment of the breast. Diabetes mellitus may be associated with reduced 5-year survival, although this finding did not reach statistical significance and requires further investigation. Due to its small sample size, this study should be regarded more as hypothesis-generating.
2025-07-28 | Clinicopathological features and prognostic insights of cutaneous versus non-cutaneous angiosarcoma: A retrospective single-center cohort study.
Angiosarcoma is a rare, aggressive malignancy of endothelial origin, accounting for 1-2 % of soft tissue sarcomas. It occurs in cutaneous (C-AS) and non-cutaneous (NC-AS) forms, with distinct clinical behaviors and prognoses. While C-AS primarily affects the head and neck, NC-AS involves visceral organs such as the heart, liver, and breast. Despite advances in treatment, survival remains poor, and recurrence is common. This retrospective, single-center cohort study analyzed 30 patients diagnosed with angiosarcoma at the University Hospital Zurich (2000-2023). The study compared clinicopathological characteristics, survival outcomes, and prognostic factors influencing survival and recurrence. Patients were stratified into C-AS (n = 10) and NC-AS (n = 20) groups. Overall survival (OS) and recurrence-free survival (RFS) were assessed using Kaplan-Meier and Cox regression analyses. Both subtypes shared a poor prognosis, with an overall 5-year survival rate of 12 %. NC-AS had a higher early mortality rate, with a significantly worse median OS (9 vs. 36 months, p = 0.04), while early recurrence after treatment was more frequent in C-AS. High-grade tumor differentiation and metastases at diagnosis were independent predictors of poorer OS (p = 0.01; p = 0.04). Multimodal treatment, including surgery and radiotherapy, was associated with improved survival (p < 0.05). No clinicopathological factor showed a statistically significant association with RFS. C-AS and NC-AS exhibit distinct prognostic profiles, with NC-AS associated with a higher metastatic burden and worse outcomes. Prognostic factors such as tumor grade and metastases at diagnosis influence survival. Multimodal treatment strategies appear beneficial, though recurrence remains a major challenge. Further prospective studies are needed to refine therapeutic approaches and explore emerging targeted therapies.
cell therapies
2026-09-06 | Liver transplantation for pediatric liver tumors: a case report of hepatic angiosarcoma with literature review.
Pediatric hepatic angiosarcoma (HAS) is poorly understood. The lack of effective therapies for unresectable pediatric liver tumors creates a clinical imperative, positioning liver transplantation (LT) as a pivotal modality for exploring the roadmap to prolonged survival. We report the first Chinese pediatric case of HAS treated with LT. A 2-year-old boy was admitted to hospital due to abdominal pain and distension. Imaging findings were suggestive of possible malignancy, but initial biopsy yielded a diagnosed infantile hepatic hemangioma (IHH). Following living-donor LT for unresectable disease, explant pathology revealed an angiosarcoma arising within a hemangioma. The patient ultimately died from metastasis 21 months after LT. Our review of pediatric LT for liver tumors over 15 years identified nine pediatric HAS cases, only one of whom was correctly diagnosed pre-LT, with survival ranging from 3 to 66 months. Regarding other tumors, six cases of IHH were reported prior to 2010, without survival data. Recent studies on hepatoblastoma (HB) report a 5-year overall survival (OS) of 80-90%, and the prognosis for hepatocellular carcinoma (HCC) has markedly improved, with a combined 5-year OS of 74%. In the largest reported study, the 5-year OS rates for hepatic epithelioid hemangioendothelioma (HEH) and hepatic undifferentiated embryonal sarcoma (HUES) were 60.6% and 90.0%, respectively. Evidence for biliary tract rhabdomyosarcoma (BT-RMS) and malignant rhabdoid tumor (MRT) of the liver are anecdotal, and no pediatric cases of primary hepatic kaposiform hemangioendothelioma (KHE) treated with LT have been reported. Pediatric HAS is rare and difficult to distinguish from IHH; contrast-enhanced computed tomography/magnetic resonance imaging (CT/MRI) aids differentiation. For suspected malignant transformation, contrast-enhanced ultrasound (CEUS) and ultrasound-guided multi-site biopsy are recommended. No standardized treatment exists for pediatric HAS. Given the poor long-term survival, LT or combined chemotherapy remains exploratory but is the only option for unresectable tumors. The high survival rates of LT for HB and HCC offer a promising blueprint. With advances in surgical techniques and increased living donor availability, pediatric LT deserves more attention.
2026-07-06 | Double free flap reconstruction using the piggyback technique for multifocal scalp radiation necrosis: a case report.
Scalp angiosarcoma is a rare, aggressive malignancy with poor prognosis, particularly when multifocal. We report a 62-year-old woman with multifocal scalp angiosarcoma, a 3.5× 3.5 cm ulcerated vertex lesion within an approximately 10× 10 cm clinically abnormal vertex field and three 1× 1 cm temporal satellite lesions (two in the right temporal region and one in the left temporal region). Initial wide excision with frozen biopsy-guided margins and reconstruction using a rotation flap and split-thickness skin grafts was followed by adjuvant radiotherapy. At 7 months after completion of radiotherapy, radiation-induced necrosis with bone exposure developed in the frontal and occipital regions, with multiple biopsies negative for recurrence. Double free flap reconstruction (radial forearm free flap and adipofascial anterolateral thigh flap using a piggyback anastomosis) was performed 11 months after radiotherapy using a single recipient vessel pair. Venous congestion on postoperative day 1 was successfully revised with vein graft interposition, resulting in complete flap survival. The patient was disease-free with stable flap coverage at the latest follow-up, more than 2.5 years after radiotherapy. This case demonstrates that double free flap reconstruction using a piggyback technique can be a safe and effective reconstructive option for extensive irradiated scalp defects in multifocal angiosarcoma.
2026-04-09 | From Palliation After Angiosarcoma Resection to Totally Autologous Aesthetic Breast Reconstruction Combining Kiss Latissimus Dorsi Flap and Contralateral Breast Sharing Internal Mammary Artery Perforator Flap: A Case Report.
Angiosarcoma of the breast is a rare but aggressive disease that often requires wide-margin resection resulting in massive thoracic defects. In this report we present the unique case of a patient who underwent primary chest wall reconstruction with palliative intent using Kiss Latissimus Dorsi (LD) Flap after resection of a huge angiosarcoma of the right breast, followed 3 years later by a secondary shift to totally autologous aesthetic breast reconstruction which was achieved by combining the first flap with breast-sharing internal mammary artery perforator (IMAP) flap and simultaneous contralateral breast reduction. The defect after extended mastectomy, measuring 24 × 18 cm, was resurfaced with a Kiss LD flap designed with two skin paddles of 24 × 13 and 14 × 5 cm, respectively. Three years after the initial reconstruction, the patient was tumor-free and required breast symmetrization. Therefore, a left reduction mammaplasty with simultaneous breast sharing Internal Mammary Artery Perforator (IMAP) flap was performed, augmenting the hypoplastic right breast with the entire lower pole of the contralateral side, transferring an 18 × 9 cm flap based on the fifth IMAP. Vascular safety of the procedure was guaranteed by intra-operative indocyanine green angiography evaluation. Recovery was uneventful and the 6 months post-operative follow-up confirmed flap integration, volume symmetry and high aesthetic satisfaction. This report underlines the versatility of this innovative combination of flaps in complex reconstructive scenarios where complex microsurgical transfers are contraindicated.
2025-11-22 | Primary cerebellar angiosarcoma: a rare case report and literature review.
Angiosarcoma is a highly aggressive malignant mesenchymal tumor, accounting for less than 1% of all soft tissue sarcomas. While most cases arise in the skin, soft tissues, liver, or heart, primary intracranial angiosarcoma (PIA) is an exceptionally rare and distinct clinicopathological entity. Due to nonspecific clinical presentations and imaging features, PIA poses significant diagnostic and therapeutic challenges. It is characterized by aggressive behavior, with high rates of recurrence and metastasis, resulting in poor overall prognosis. Here, we report a rare case of primary cerebellar angiosarcoma and review its epidemiology, pathogenesis, diagnostic considerations, and treatment strategies. A 53-year-old woman presented with progressive dizziness, occipital headache, nausea, and vomiting for five days. MRI revealed a hemorrhagic, highly vascular right cerebellar mass initially suspected to be a hemangioblastoma. Prior to surgery, her condition deteriorated, and emergency CT showed acute tumor hemorrhage, necessitating urgent resection. Intraoperatively, the tumor was highly vascular and poorly circumscribed, suggestive of a high-grade sarcoma, later confirmed as angiosarcoma. Despite gross total resection, the patient remained comatose due to brainstem compression. Follow-up CT on day 20 revealed rapid tumor recurrence. Given the poor prognosis, the family opted for palliative care, and the patient subsequently passed away. PIA is a rare and aggressive tumor with a poor prognosis, characterized by rapid progression and recurrence. Early detection and surgical resection are essential, but effective adjuvant therapies remain limited. A multidisciplinary approach is crucial to improve outcomes, and further research is needed to refine therapeutic strategies and enhance survival.
2025-02-06 | Usefulness of Dorsal Scapular Artery Perforator Flap for Donor Site Closure After Harvesting Latissimus Dorsi Flap/Thoracodorsal Artery Perforator Flap: A Report of Three Cases.
Reducing donor-site morbidity is crucial in free-flap reconstruction, with the primary goals of achieving dermis-to-dermis closure and avoiding skin grafts. Although latissimus dorsi (LD) myocutaneous and thoracodorsal artery perforator (TDAP) flaps usually allow primary closure, larger flap harvests may require skin grafts, potentially leading to undesirable outcomes. In such situations, local flap transfer can be considered for donor-site closure, yet there are few reports on this. This report aims to present cases where a dorsal scapular artery perforator (DSAP) rotational flap was used to close donor defects following large TDAP/LD flap harvests. The first case involved a 16-year-old girl who required a below-knee amputation due to an acute thrombotic event, leaving a 20 × 20 cm2-sized stump defect. Bilateral LD myocutaneous flaps were harvested, with the right donor site closed primarily, while a 20 × 9 cm2-sized defect remained on the left. A 22 × 9 cm2-sized DSAP flap, based on three perforators, was rotated to close the defect, showing excellent outcomes at 12 months. In the second and third cases, 75- and 78-year-old male patients with scalp angiosarcoma underwent wide excision and reconstruction with TDAP flaps, leaving 14 × 6 cm2 and 14 × 7 cm2-sized donor defects. DSAP flaps, 15 × 7 cm2 and 15 × 8 cm2 in size, were rotated based on nearby perforators to close these areas. Both patients showed no morbidities at postoperative 8 and 2 months, respectively. When primary closure of the donor site is challenging after harvesting large TDAP /LD flaps, a DSAP rotational flap can provide a valuable option for minimizing donor-site morbidity.
other
2026-07-31 | Management of Primary Hepatic Angiosarcoma: A Comprehensive Review.
Primary hepatic angiosarcoma (PHA) is the most common primary malignant mesenchymal tumour of the liver in adults, accounting for only 0.1-2% of all primary hepatic malignancies. It carries a median overall survival of 6-9 months, driven by aggressive biology, non-specific presentation, and almost universal late-stage diagnosis. No disease-specific tumour markers, pathognomonic imaging features, or PHA-exclusive prospective trials exist. A comprehensive narrative review was performed using PubMed, Embase, Cochrane Library, and Scopus. Search terms encompassed epidemiology, pathology, molecular biology, imaging, surgical and systemic management, and prognosis of PHA. Studies published up to June 2026 were included, with preference given to systematic reviews, multicenter cohorts, and prospective trials. ERG is the most sensitive and specific immunohistochemical marker for PHA (100% sensitivity vs. 79-87% for CD31/CD34). Surgical R0 resection - achievable in fewer than 25% of patients - is the only potentially curative modality, with median overall survival of 17.2 months post-resection versus 3.7 months without surgery. Liver transplantation is an absolute contraindication. For unresectable disease, cabozantinib plus nivolumab (Alliance A091902) produced an overall response rate of 59% in taxane-pretreated patients. Gemcitabine demonstrates second-line activity with an overall response rate of 38% and median overall survival of 9.9 months. Recurrent alterations in TP53, KDR, PIK3CA, PTPRB, and PLCG1 provide a rationale for routine molecular profiling, though no actionable target has been prospectively validated. Surgical resection where feasible, ICI-TKI combination therapy for unresectable disease, and routine molecular profiling represent the current cornerstones of PHA management. Progress requires international collaborative registries, multi-institutional trials, and specialist multidisciplinary care for all patients.
2026-01-23 | Phase II trial, multicenter, first-line paclitaxel-avelumab treatment of inoperable angiosarcoma (KCSG UN 18-15).
Angiosarcomas are rare tumors of vascular or lymphatic origin with clinically heterogeneous presentation and behavior, predominantly originating in the skin of the head and neck and the breast area. Given their location and/or rapid progression, surgical resection is frequently not feasible, even in those with localized diseases. Conventional cytotoxic chemotherapy has proven largely ineffective against angiosarcoma. In this prospective, phase II study, we evaluated the efficacy and toxicity of paclitaxel plus avelumab as first-line therapy for unresectable angiosarcoma. This study included patients with unresectable locally advanced or metastatic angiosarcomas, who did not receive systemic treatment. Paclitaxel (80 mg/m2) was intravenously infused on days 1, 8, and 15 of every 28-days cycle; avelumab (10 mg/kg) was intravenously infused every 2 weeks. Treatment continued until disease progression, unacceptable toxicity, death, or withdrawal of consent, whichever occurred first. The primary endpoint was the objective response rate (ORR); secondary endpoints included overall survival (OS), progression-free survival (PFS), and safety profiles. The final analysis included 32 patients (21 male and 11 female). The median age was 63.5 years (range 27-82 years). The ORR was 50.0% (n = 16), including one complete response. Median OS and PFS were 14.5 [95% confidence interval (CI) 9.4-24.6 months] and 6.0 (95% CI 5.4-9.5 months), respectively. Among patients who received at least one treatment dose (n = 33), adverse events (AEs) of any grade were reported in 90.9% (n = 30), and severe AEs in 12.1% (n = 4), including one death. The most common hematologic and non-hematologic AEs were neutropenia (n = 13, 39.4%) and pain (n = 12, 36.4%). Immune-related pneumonitis was reported in two patients. Avelumab plus weekly paclitaxel demonstrated preliminary evidence of efficacy and tolerability in inoperable angiosarcoma. Given the rarity of angiosarcoma, further multicenter, collaborative, and translational studies are warranted.
2025-11-10 | Splenic angiosarcoma with hepatic and cardiac metastases: a case report and literature review.
Splenic angiosarcoma (SA) is an rare malignant tumor in clinical practice, characterized by high malignancy, atypical early symptoms, and a dismal prognosis. In recent years, growing attention has been focused on its diagnosis and treatment. This report describes a patient who was admitted to the hospital due to left upper abdominal pain. Contrast-enhanced CT indicated unevenly enhanced tumors in the spleen, liver, and right atrium. The patient was treated with laparoscopic splenectomy + partial hepatectomy and was diagnosed based on postoperative pathology and immunohistochemistry (positive for CD31 and CD34). After the operation, anlotinib + toripalimab targeted immunotherapy was used, and the patient recovered well. This case suggests that SA is prone to metastasis in the early stage. Clinically, the diagnosis of SA requires a combination of pathological diagnosis and imaging diagnosis, and postoperative targeted immunotherapy may improve the prognosis.
2025-08-31 | Primary pulmonary artery sarcoma treated with simultaneous pulmonary valve replacement, artificial vessel replacement, and total right pneumonectomy: a case report.
Primary pulmonary artery sarcoma progresses extremely rapidly and has a poor prognosis. This condition is managed with surgical resection and multimodality therapy. However, standardized treatment is not available. A 54-year-old woman was brought to the emergency department because of chest pain and worsening dyspnea that had developed in the past month. Contrast-enhanced computed tomography scan revealed severe stenosis extending from the pulmonary artery trunk to the right pulmonary artery due to an embolic substance. Because primary pulmonary artery sarcoma was suspected, emergency surgery was performed to improve the patient's symptoms. In addition to maximal tumor resection, pulmonary artery valve replacement, artificial vessel replacement, and right total pneumonectomy were performed. Based on the assessment performed using the specimen collected perioperatively, a pathological diagnosis of angiosarcoma of the right pulmonary artery was made. The patient was discharged on postoperative day 17 with a good postoperative course. However, because of dyspnea, she was readmitted to the hospital on day 85. Tumor recurrence was noted, and chemotherapy was initiated. The patient developed cardiac failure and died on postoperative day 119. A pathological postmortem examination was performed. Metastatic lesions were found in the pericardial sac, left lung, right chest wall and pleura, and mediastinum. In the present case, postoperative recurrence was observed despite maximal resection of the surrounding tissues with tumor invasion and simultaneous reconstruction. Chemotherapy was initiated but was ineffective. Gene panel testing can help identify novel treatment options for patients with neoplastic diseases without standardized treatment. In addition, preparations should be made before surgery.
2025-03-09 | Angiosarcoma: Role of Immunotherapy.
Recent clinical trials have investigated immune checkpoint inhibitors (ICIs) alone, in combination with tyrosine kinase inhibitors (TKIs), or with chemotherapy in angiosarcoma, yielding mixed results across subtypes. Single-agent ICI therapy, such as cemiplimab (ORR 27.8%), has shown responses primarily in UV- and radiation-associated angiosarcomas, likely due to their higher tumor mutation burden (TMB), while dual ICI therapy (SWOG S1609, ORR 25%) suggests potential benefit but remains limited in cutaneous disease. ICI-TKI combinations, such as cabozantinib plus nivolumab (Alliance A091902, second-line, ORR 59%), demonstrated significant efficacy in both cutaneous and non-cutaneous angiosarcomas, suggesting anti-angiogenic therapy may enhance ICI responses in tumors historically resistant to checkpoint blockade. ICI-chemotherapy combinations have been less consistent. The Alliance A091902 first-line trial (paclitaxel ± nivolumab) found no overall PFS benefit, though scalp/face angiosarcoma patients appeared to fare better, raising the question of whether ICI alone might be equally effective in this subset. The South Korean paclitaxel + avelumab trial (ORR 50%) showed promising response rates, but the lack of detailed subgroup analysis limits interpretation. Other chemotherapy-ICI combinations, such as doxorubicin plus pembrolizumab, have shown isolated responses but require further study in larger cohorts. Moving forward, better biomarkers are critical for identifying which patients benefit most from ICIs, and while TKI-ICI combinations appear to hold the most promise, chemotherapy-ICI strategies need further refinement to optimize sequencing and patient selection in angiosarcoma treatment.
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Drug Discovery Landscape
3 orphan drug designations for Angiosarcoma.
3 orphan drug designations for Angiosarcoma.
Drug | Therapy type | Orphan designation | Clinical trial status | Sponsor |
|---|---|---|---|---|
Oraxol | small molecules | FDA | Health Hope Pharma | |
PEG-b-PLA micelles containing (1) paclitaxel, (2) sirolimus (rapamycin) and (3) tanespimycin (17-AAG)(also known as: triolimus) | small molecules | FDA | — | Co-D Therapeutics, Inc. |
Human anti-integrin receptor av monoclonal antibody | antibodies | FDA | — | Janssen Research & Development, LLC |
“Terminated” – programs with discontinued clinical trials, or with no updates reported for 5 years after completion or 12 years after the last trial started.
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