RARE DISEASE
Primary benign peritoneal tumor
Primary benign peritoneal tumor
Primary benign peritoneal tumor
Drug discovery
0
drugs
With orphan designations
Overview
Primary benign peritoneal tumors are rare neoplasms originating from the mesothelial or submesothelial layers of the peritoneum. These include lesions such as multicystic mesothelioma and leiomyomatosis peritonealis disseminata (LPD). Though histologically benign, they may exhibit locally aggressive behavior or recurrence. Multicystic mesothelioma typically presents as multilocular cystic masses in the pelvis, while LPD manifests as multiple smooth muscle nodules. Diagnosis relies on imaging (CT/MRI) and histopathology, with management focused on cytoreduction and surveillance due to indolent biology [1][9][16].
Categories: rare abdominal surgical diseases, rare neoplastic diseases
Research Papers
13 drug discovery papers about Primary benign peritoneal tumor, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
13 drug discovery papers about Primary benign peritoneal tumor, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2025-03-01 | Definition of in vivo architectural patterns of peritoneal carcinomatosis to support surgeons’ resection strategies using probe-based confocal laser endomicroscopy (pCLE) approach
Aims Widespread peritoneal carcinomatosis is a condition found in many advanced stage tumors, such as ovarian, stomach and colorectal cancer, that can significantly increase the complexity of surgical procedures, as observed in women with advanced-stage epithelial ovarian cancer (EOC). Probe-based confocal laser endomicroscopy (pCLE) is a novel real-time imaging technique potentially helping the definition of architectural patterns of peritoneal carcinomatosis. This technique may find a role in supporting surgeons' resection strategies both in open and laparoscopic approach. Methods In this initial study we investigated pCLE potentiality enrolling women with FIGO Stage III/IV EOC referred to our institution for primary debulking surgery or interval debulking surgery. In vivo pCLE images and subsequent biopsies were acquired after i.v. injection of fluorescein, from various anatomical sites, including the parietal and visceral peritoneum, ovary, and omentum. Each pCLE sequence was compared with the histological analysis of the corresponding specimen to identify in vivo pCLE imaging features associated with benign and malignant tissue. Results Images acquired from normal tissues revealed a regular distribution of stromal fibers and consistent cellular architecture, with regular vessels and blood flow. No fluorescein extravasation was observed in healthy tissue. Conversely, leakage was a distinguishing feature of tumor-associated vessels, as all malignant nodules were easily recognized by the extravasation of the contrast agent, highlighting the abnormal functionality and permeability of tumor vessels. The leakage often surrounded dark clusters of neoplastic cells with irregular shapes and distributions. A substantial agreement between pCLE and histology emerged (Cohen’s K coefficient 0.66), while only a fair concordance between surgeon’s intraoperative assessment and histology was found (Cohen’s K coefficient 0.30). Conclusions This is the first study using pCLE technology for in vivo evaluation of the architectural patterns of both peritoneal carcinomatosis and normal peritoneum. A good concordance between pCLE and histology was observed. The results of this study suggest that pCLE may serve as a promising intraoperative technique to assist surgeons, both in open and laparoscopic approach, in order to accurately detect peritoneal metastasis in advanced cancers, thereby enhancing surgical radicality while avoiding unnecessary resection. Publication History Article published online: 27 March 2025 © 2025. European Society of Gastrointestinal Endoscopy. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
2018-11-20 | Cytoreductive Surgery and Intraperitoneal Hyperthermic Chemotherapy (HIPEC) by Minimally Invasive Approach, an Initial Experience.
The cytoreductive surgery (CRS) associated with hyperthermic intraperitoneal chemotherapy (HIPEC) has become the standard treatment in patients with carcinomatosis peritoneal from different origins. The use of a minimally invasive approach for this high complex procedure might be an alternative that provides them less morbidity and faster recovery with similar oncologic outcomes. We describe the initial experience of CRS and HIPEC done via the laparoscopic route in patients with minimal peritoneal metastases in our Unit from March 2016 to January 2018. A total of eight patients were operated by this minimally invasive approach. The different diagnoses were low-grade pseudomyxoma peritonei (2), benign multicystic mesothelioma (2), primary epithelial ovarian carcinomatosis (2) and locally advanced colon carcinoma T4 (2). The median age was 54 (20-62) years, the median PCI was 3 (2-4), the median operative time was 287 min (240-360), complete cytoreduction CC0 was achieved in all the patients, and no major morbidity was observed. The median length of stay was 4.75 days (4-5). After a median follow-up of 9.5 months, no relapse has been observed. The results suggest that this minimally invasive approach for CRS and HIPEC is feasible and safe in a highly selected group of patients with peritoneal surface malignancies.
2015-12-30 | Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma.
Benign multicystic mesothelioma is a rare tumour that originates from the abdominal peritoneum with a predisposition to the pelvic peritoneum. It typically affects women of reproductive age. There have been less than 200 cases of this rare neoplasia reported to date. We present the case of a 35-year-old woman who was referred to our centre because of the detection of a peritoneal carcinomatosis during a gynaecological exam. A diagnostic laparoscopy was performed. The findings included multiple cysts appearing as 'a bunch of grapes' occupying the omentum. Biopsies were taken during the surgery and the results showed benign multicystic peritoneal mesothelioma. Benign multicystic mesothelioma can simulate other conditions, such as malignant ovarian tumours or cystic lymphangioma. It is often diagnosed accidentally during surgery performed for another reason. The diagnosis is interoperative, observing multicystic structures grouped as a 'bunch of grapes' containing clear fluid with thin walls made of connective tissue. Immunohistochemistry confirmed mesothelial origin. Surgery is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Hyperthermic intraperitoneal chemotherapy can be considered as a primary treatment in patients with recurrences or even as a part of primary treatment associated with surgery. Survival at 5 years is 100% and invasive or malignant progression is extraordinary. The treatment approach should be multidisciplinary, and the patient should be referred to a referral centre.
2008-09-10 | Phase 2 study of intraperitoneal topotecan as consolidation chemotherapy in ovarian and primary peritoneal carcinoma.
Intravenous topotecan is approved for the treatment of ovarian cancer (OC). In intraperitoneal (i.p.) topotecan studies, 20 mg/m(2) dosing was tolerable. This study evaluated the feasibility, safety, and preliminary efficacy of i.p. topotecan as consolidation chemotherapy in patients with OC or primary peritoneal cancers (PPCs). Patients with stage III/IV ovarian or PPC in clinical complete response after surgical cytoreduction and intravenous carboplatin/paclitaxel chemotherapy who had benign findings or minimal persistent disease (< or = 1 cm diameter) at second-look surgery were eligible. Intraperitoneal topotecan 20 mg/m(2) was infused once every 21 days for 4 to 6 cycles. Kaplan-Meier estimates were used to calculate progression-free survival (PFS) and overall survival (OS). Twenty patients were enrolled (18 [90%] patients had OC). Sixteen patients received 4 cycles, 3 patients received 6 cycles, and 1 patient withdrew after 1 cycle. The mean delivered dose was 18 mg/m(2). Grade 3/4 toxicities included neutropenia and thrombocytopenia (45% for both). Grade 1/2 abdominal distension and nausea were reported in 60% and 40% of patients, respectively. Median PFS was 24 months from second-look surgery (95% confidence intervals [CI]: +/-10 months). Sixteen patients were alive and median OS was not reached at the time of data analysis. OS estimated at either 30 months from second-look surgery, or 3 years from initial diagnosis, was 84% (95% CI, 68%-100%). Consolidation i.p. topotecan is a feasible option for women withadvanced ovarian and primary peritoneal cancers. Further investigation of i.p. topotecan is warranted in this patient population.
2006-03-02 | Immunocytochemical detection of XIAP in body cavity effusions and washes.
Body cavity effusions may be the first manifestation of malignancy or of recurrence or relapse. We surveyed effusions and washes for expression of X-linked inhibitor of apoptosis (XIAP), a potent constituent of the inhibitor of apoptosis (IAP) family of proteins. IAPs prevent apoptosis by blocking the activation of caspases, thereby preventing caspase-mediated cell degradation. Elevated expression of XIAP could be an underpinning of relapse and/or resistance to apoptosis-inducing cancer therapy. We performed an immunocytochemical survey of XIAP expression in cell blocks from benign and malignant body cavity effusions and washes. In all, 116 alcohol-fixed, formalin postfixed paraffin-embedded cell block specimens from 82 pleural effusions, 22 ascites, 11 pelvic/peritoneal washes and one pericardial effusion were evaluated immunocytochemically with monoclonal anti-XIAP (#610763, BD Biosciences, San Jose, USA) 1:250, 4 degrees C x 72 h, and developed using EnVision-Plus reagents (Dako) and diaminobenzidine as chromagen. Particulate cytoplasmic staining was considered positive. The prevalence of staining for specific malignancies varied with the tissue of origin as follows: ovarian (13/13, 100%); lung (9/11, 82%), breast (6/13, 46%); gastric (4/7, 57%), colon (0/4, 0%), pancreas (2/3, 67%), gallbladder (1/1, 100%), fallopian tube (1/3, 33%), endometrial (6/7, 86%), mesothelioma (4/5, 80%), carcinoma of unknown primary (5/5, 100%) and hematopoietic malignancies (3/9, 33%). Overall, 54 out of 81 (67%) malignant effusions displayed XIAP positivity. Benign effusions (n = 35) were virtually XIAP-negative except for two cases (6%) in which histiocytes showed moderate staining. Weak nonspecific staining was sometimes noted in inflammatory cells or histiocytes. XIAP immunostaining, when strong, allows for ready distinction of malignant from benign and reactive cell populations. Strong XIAP staining was most prevalent in ovarian carcinomas and less prevalent in mammary carcinomas. The degree of XIAP staining of tumor cells may be a means of identifying the most therapy-resistant cases (ie, those with strong XIAP expression), and allow additional triaging to XIAP-blocking drugs presently being developed and clinically tested.
small molecules
2025-03-01 | Definition of in vivo architectural patterns of peritoneal carcinomatosis to support surgeons’ resection strategies using probe-based confocal laser endomicroscopy (pCLE) approach
Aims Widespread peritoneal carcinomatosis is a condition found in many advanced stage tumors, such as ovarian, stomach and colorectal cancer, that can significantly increase the complexity of surgical procedures, as observed in women with advanced-stage epithelial ovarian cancer (EOC). Probe-based confocal laser endomicroscopy (pCLE) is a novel real-time imaging technique potentially helping the definition of architectural patterns of peritoneal carcinomatosis. This technique may find a role in supporting surgeons' resection strategies both in open and laparoscopic approach. Methods In this initial study we investigated pCLE potentiality enrolling women with FIGO Stage III/IV EOC referred to our institution for primary debulking surgery or interval debulking surgery. In vivo pCLE images and subsequent biopsies were acquired after i.v. injection of fluorescein, from various anatomical sites, including the parietal and visceral peritoneum, ovary, and omentum. Each pCLE sequence was compared with the histological analysis of the corresponding specimen to identify in vivo pCLE imaging features associated with benign and malignant tissue. Results Images acquired from normal tissues revealed a regular distribution of stromal fibers and consistent cellular architecture, with regular vessels and blood flow. No fluorescein extravasation was observed in healthy tissue. Conversely, leakage was a distinguishing feature of tumor-associated vessels, as all malignant nodules were easily recognized by the extravasation of the contrast agent, highlighting the abnormal functionality and permeability of tumor vessels. The leakage often surrounded dark clusters of neoplastic cells with irregular shapes and distributions. A substantial agreement between pCLE and histology emerged (Cohen’s K coefficient 0.66), while only a fair concordance between surgeon’s intraoperative assessment and histology was found (Cohen’s K coefficient 0.30). Conclusions This is the first study using pCLE technology for in vivo evaluation of the architectural patterns of both peritoneal carcinomatosis and normal peritoneum. A good concordance between pCLE and histology was observed. The results of this study suggest that pCLE may serve as a promising intraoperative technique to assist surgeons, both in open and laparoscopic approach, in order to accurately detect peritoneal metastasis in advanced cancers, thereby enhancing surgical radicality while avoiding unnecessary resection. Publication History Article published online: 27 March 2025 © 2025. European Society of Gastrointestinal Endoscopy. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
2018-11-20 | Cytoreductive Surgery and Intraperitoneal Hyperthermic Chemotherapy (HIPEC) by Minimally Invasive Approach, an Initial Experience.
The cytoreductive surgery (CRS) associated with hyperthermic intraperitoneal chemotherapy (HIPEC) has become the standard treatment in patients with carcinomatosis peritoneal from different origins. The use of a minimally invasive approach for this high complex procedure might be an alternative that provides them less morbidity and faster recovery with similar oncologic outcomes. We describe the initial experience of CRS and HIPEC done via the laparoscopic route in patients with minimal peritoneal metastases in our Unit from March 2016 to January 2018. A total of eight patients were operated by this minimally invasive approach. The different diagnoses were low-grade pseudomyxoma peritonei (2), benign multicystic mesothelioma (2), primary epithelial ovarian carcinomatosis (2) and locally advanced colon carcinoma T4 (2). The median age was 54 (20-62) years, the median PCI was 3 (2-4), the median operative time was 287 min (240-360), complete cytoreduction CC0 was achieved in all the patients, and no major morbidity was observed. The median length of stay was 4.75 days (4-5). After a median follow-up of 9.5 months, no relapse has been observed. The results suggest that this minimally invasive approach for CRS and HIPEC is feasible and safe in a highly selected group of patients with peritoneal surface malignancies.
2015-12-30 | Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma.
Benign multicystic mesothelioma is a rare tumour that originates from the abdominal peritoneum with a predisposition to the pelvic peritoneum. It typically affects women of reproductive age. There have been less than 200 cases of this rare neoplasia reported to date. We present the case of a 35-year-old woman who was referred to our centre because of the detection of a peritoneal carcinomatosis during a gynaecological exam. A diagnostic laparoscopy was performed. The findings included multiple cysts appearing as 'a bunch of grapes' occupying the omentum. Biopsies were taken during the surgery and the results showed benign multicystic peritoneal mesothelioma. Benign multicystic mesothelioma can simulate other conditions, such as malignant ovarian tumours or cystic lymphangioma. It is often diagnosed accidentally during surgery performed for another reason. The diagnosis is interoperative, observing multicystic structures grouped as a 'bunch of grapes' containing clear fluid with thin walls made of connective tissue. Immunohistochemistry confirmed mesothelial origin. Surgery is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Hyperthermic intraperitoneal chemotherapy can be considered as a primary treatment in patients with recurrences or even as a part of primary treatment associated with surgery. Survival at 5 years is 100% and invasive or malignant progression is extraordinary. The treatment approach should be multidisciplinary, and the patient should be referred to a referral centre.
2008-09-10 | Phase 2 study of intraperitoneal topotecan as consolidation chemotherapy in ovarian and primary peritoneal carcinoma.
Intravenous topotecan is approved for the treatment of ovarian cancer (OC). In intraperitoneal (i.p.) topotecan studies, 20 mg/m(2) dosing was tolerable. This study evaluated the feasibility, safety, and preliminary efficacy of i.p. topotecan as consolidation chemotherapy in patients with OC or primary peritoneal cancers (PPCs). Patients with stage III/IV ovarian or PPC in clinical complete response after surgical cytoreduction and intravenous carboplatin/paclitaxel chemotherapy who had benign findings or minimal persistent disease (< or = 1 cm diameter) at second-look surgery were eligible. Intraperitoneal topotecan 20 mg/m(2) was infused once every 21 days for 4 to 6 cycles. Kaplan-Meier estimates were used to calculate progression-free survival (PFS) and overall survival (OS). Twenty patients were enrolled (18 [90%] patients had OC). Sixteen patients received 4 cycles, 3 patients received 6 cycles, and 1 patient withdrew after 1 cycle. The mean delivered dose was 18 mg/m(2). Grade 3/4 toxicities included neutropenia and thrombocytopenia (45% for both). Grade 1/2 abdominal distension and nausea were reported in 60% and 40% of patients, respectively. Median PFS was 24 months from second-look surgery (95% confidence intervals [CI]: +/-10 months). Sixteen patients were alive and median OS was not reached at the time of data analysis. OS estimated at either 30 months from second-look surgery, or 3 years from initial diagnosis, was 84% (95% CI, 68%-100%). Consolidation i.p. topotecan is a feasible option for women withadvanced ovarian and primary peritoneal cancers. Further investigation of i.p. topotecan is warranted in this patient population.
2006-03-02 | Immunocytochemical detection of XIAP in body cavity effusions and washes.
Body cavity effusions may be the first manifestation of malignancy or of recurrence or relapse. We surveyed effusions and washes for expression of X-linked inhibitor of apoptosis (XIAP), a potent constituent of the inhibitor of apoptosis (IAP) family of proteins. IAPs prevent apoptosis by blocking the activation of caspases, thereby preventing caspase-mediated cell degradation. Elevated expression of XIAP could be an underpinning of relapse and/or resistance to apoptosis-inducing cancer therapy. We performed an immunocytochemical survey of XIAP expression in cell blocks from benign and malignant body cavity effusions and washes. In all, 116 alcohol-fixed, formalin postfixed paraffin-embedded cell block specimens from 82 pleural effusions, 22 ascites, 11 pelvic/peritoneal washes and one pericardial effusion were evaluated immunocytochemically with monoclonal anti-XIAP (#610763, BD Biosciences, San Jose, USA) 1:250, 4 degrees C x 72 h, and developed using EnVision-Plus reagents (Dako) and diaminobenzidine as chromagen. Particulate cytoplasmic staining was considered positive. The prevalence of staining for specific malignancies varied with the tissue of origin as follows: ovarian (13/13, 100%); lung (9/11, 82%), breast (6/13, 46%); gastric (4/7, 57%), colon (0/4, 0%), pancreas (2/3, 67%), gallbladder (1/1, 100%), fallopian tube (1/3, 33%), endometrial (6/7, 86%), mesothelioma (4/5, 80%), carcinoma of unknown primary (5/5, 100%) and hematopoietic malignancies (3/9, 33%). Overall, 54 out of 81 (67%) malignant effusions displayed XIAP positivity. Benign effusions (n = 35) were virtually XIAP-negative except for two cases (6%) in which histiocytes showed moderate staining. Weak nonspecific staining was sometimes noted in inflammatory cells or histiocytes. XIAP immunostaining, when strong, allows for ready distinction of malignant from benign and reactive cell populations. Strong XIAP staining was most prevalent in ovarian carcinomas and less prevalent in mammary carcinomas. The degree of XIAP staining of tumor cells may be a means of identifying the most therapy-resistant cases (ie, those with strong XIAP expression), and allow additional triaging to XIAP-blocking drugs presently being developed and clinically tested.
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