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
1,290 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:
1,290 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:
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.
2026-08-04 | Selective Transarterial Embolization for Refractory Bleeding in Advanced Scalp Angiosarcoma: Two Cases.
Cutaneous angiosarcoma of the scalp is a highly aggressive vascular malignancy that predominantly affects elderly individuals and may cause severe tumor bleeding during disease progression. Hemorrhage from ulcerated tumors is often difficult to control, particularly in patients previously treated with chemoradiotherapy. We report two cases of advanced scalp angiosarcoma in which refractory tumor bleeding was successfully controlled by selective transarterial embolization. The first patient, a 75-year-old man with recurrent angiosarcoma of the frontal scalp region after multiple systemic therapies and radiotherapy, developed repeated arterial bleeding from ulcerated lesions requiring transfusion support. Angiography identified tumor-feeding branches of the superficial temporal artery and angular artery, and embolization achieved immediate hemostasis. A second embolization procedure was required for recurrent bleeding. The second patient, a 75-year-old woman, also developed persistent bleeding from a progressive scalp tumor despite chemoradiotherapy. Selective arterial embolization resulted in rapid and sustained bleeding control without major complications. These cases suggest that selective transarterial embolization may provide rapid control of refractory arterial tumor bleeding and reduce local treatment burden in selected patients with advanced scalp angiosarcoma.
2026-08-02 | When a haematoma is not a haematoma.
Cutaneous angiosarcoma (cAS) is a rare but highly aggressive vascular malignancy, often associated with chronic lymphoedema or previous radiotherapy. Its nonspecific clinical presentation can mimic benign conditions, leading to diagnostic delays. We report the case of a 70-year-old woman with chronic lymphoedema of the right arm following mastectomy and lymphadenectomy for breast cancer. She presented with a slowly enlarging haematoma-like lesion. Magnetic resonance imaging (MRI) performed externally suggested a benign haematoma. Due to progressive changes, the patient was referred to our department. High-resolution sonography revealed irregular dermal and subcutaneous vasculature with marked hyperperfusion. Deep skin biopsy demonstrated a vascular neoplasm composed of pleomorphic endothelial cells expressing ERG, CD31, D2-40 and c-Myc, with a high Ki-67 proliferation index, consistent with the diagnosis of cAS (Stewart-Treves syndrome). Computed tomography staging excluded metastatic disease, and systemic paclitaxel therapy was initiated. This case underlines the often inconspicuous clinical presentation of cAS and the limited diagnostic value of MRI for superficial lesions. High-resolution sonography may provide an important, noninvasive diagnostic clue by revealing marked hypervascularity, thereby prompting timely biopsy and improving early detection of this aggressive tumour.
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.
2026-08-08 | 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.
2026-08-04 | Selective Transarterial Embolization for Refractory Bleeding in Advanced Scalp Angiosarcoma: Two Cases.
Cutaneous angiosarcoma of the scalp is a highly aggressive vascular malignancy that predominantly affects elderly individuals and may cause severe tumor bleeding during disease progression. Hemorrhage from ulcerated tumors is often difficult to control, particularly in patients previously treated with chemoradiotherapy. We report two cases of advanced scalp angiosarcoma in which refractory tumor bleeding was successfully controlled by selective transarterial embolization. The first patient, a 75-year-old man with recurrent angiosarcoma of the frontal scalp region after multiple systemic therapies and radiotherapy, developed repeated arterial bleeding from ulcerated lesions requiring transfusion support. Angiography identified tumor-feeding branches of the superficial temporal artery and angular artery, and embolization achieved immediate hemostasis. A second embolization procedure was required for recurrent bleeding. The second patient, a 75-year-old woman, also developed persistent bleeding from a progressive scalp tumor despite chemoradiotherapy. Selective arterial embolization resulted in rapid and sustained bleeding control without major complications. These cases suggest that selective transarterial embolization may provide rapid control of refractory arterial tumor bleeding and reduce local treatment burden in selected patients with advanced scalp angiosarcoma.
2026-08-02 | When a haematoma is not a haematoma.
Cutaneous angiosarcoma (cAS) is a rare but highly aggressive vascular malignancy, often associated with chronic lymphoedema or previous radiotherapy. Its nonspecific clinical presentation can mimic benign conditions, leading to diagnostic delays. We report the case of a 70-year-old woman with chronic lymphoedema of the right arm following mastectomy and lymphadenectomy for breast cancer. She presented with a slowly enlarging haematoma-like lesion. Magnetic resonance imaging (MRI) performed externally suggested a benign haematoma. Due to progressive changes, the patient was referred to our department. High-resolution sonography revealed irregular dermal and subcutaneous vasculature with marked hyperperfusion. Deep skin biopsy demonstrated a vascular neoplasm composed of pleomorphic endothelial cells expressing ERG, CD31, D2-40 and c-Myc, with a high Ki-67 proliferation index, consistent with the diagnosis of cAS (Stewart-Treves syndrome). Computed tomography staging excluded metastatic disease, and systemic paclitaxel therapy was initiated. This case underlines the often inconspicuous clinical presentation of cAS and the limited diagnostic value of MRI for superficial lesions. High-resolution sonography may provide an important, noninvasive diagnostic clue by revealing marked hypervascularity, thereby prompting timely biopsy and improving early detection of this aggressive tumour.
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.
Access all drug discovery papers and probability of success in trials forecasts:
Access all drug discovery papers and probability of success in trials forecasts:
Drug Discovery Landscape
3 orphan drug designations for Angiosarcoma.
3 orphan drug designations for Angiosarcoma.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Oraxol | small molecules | FDA | 2018-04-16 | — | 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 | 2015-04-02 | — | Co-D Therapeutics, Inc. |
Human anti-integrin receptor av monoclonal antibody | antibodies | FDA | 2005-05-05 | — | Janssen Research & Development, LLC |
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