Related Experiment Video
Updated: Jul 25, 2025
Implantation and Monitoring by PET/CT of an Orthotopic Model of Human Pleural Mesothelioma in Athymic Mice
Published on: December 21, 2019
Rare Variants of Malignant Peritoneal Mesothelioma: a Literature Review
Ambarish Chatterjee1, Aditi Bhatt2
1Unit of Colorectal and Peritoneal Surface Malignancy, Department of Surgical Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
Abstract:
Primary peritoneal mesothelioma (PM) is a rare and aggressive malignancy that arises from the peritoneum and classified into diffuse malignant peritoneum mesothelioma (DMPM) and borderline variants, viz. multicystic peritoneal mesothelioma (MCPM) and well-differentiated papillary peritoneal mesothelioma (WDPPM). The borderline variants are rarer than conventional DMPM, are less aggressive form accounting for 3-5% of all cases of peritoneal mesothelioma. In this narrative review article, we have discussed the pathogenesis, clinical presentation, natural history, and management of these rarer variants of PM, viz. MCPM and WDPPM. Histologically, MCPM typically consists of small cysts composed of mesothelial epithelium with benign bland cuboidal cells with clear fluids; cells lack cellular atypia and have increased number of mitoses. WDPPM has specific papillary component with myxoid plump cores and single layer of bland mesothelial cells. Both the variants commonly present as incidental finding or symptoms of chronic abdominal pain, chronic pelvic inflammatory disease, pelvic mass, and infertility. In the absence of treatment, these diseases are slow growing with major concerns being that both the variants have malignant transformation capabilities and a high rate of recurrence. In the light of current evidences, it is recommended that MCPM and WDPPM patients should be offered a complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy consisting of cisplatin and doxorubicin. Collaborative multi-institutional studies are needed to generate more data and formulate robust guidelines.
Insights
Multicystic peritoneal mesothelioma (MCPM) and well-differentiated papillary peritoneal mesothelioma (WDPPM) are rare, less aggressive peritoneal mesothelioma variants. Complete cytoreductive surgery with hyperthermic intraperitoneal chemotherapy is recommended for these conditions.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Primary peritoneal mesothelioma (PM) encompasses diffuse malignant peritoneal mesothelioma (DMPM) and rarer borderline variants: multicystic peritoneal mesothelioma (MCPM) and well-differentiated papillary peritoneal mesothelioma (WDPPM).
- These borderline variants constitute 3-5% of PM cases, exhibiting less aggressive behavior than DMPM.
- Understanding their distinct histological features, clinical presentation, and natural history is crucial for effective management.
Purpose of the Study:
- To provide a comprehensive narrative review of the pathogenesis, clinical presentation, natural history, and management of MCPM and WDPPM.
- To highlight the histological characteristics differentiating these rare peritoneal mesothelioma variants.
- To discuss current evidence-based treatment recommendations for MCPM and WDPPM.
Main Methods:
- Systematic literature review of primary peritoneal mesothelioma, focusing on multicystic peritoneal mesothelioma and well-differentiated papillary peritoneal mesothelioma.
- Analysis of histological findings, clinical manifestations, and treatment outcomes reported in existing studies.
- Synthesis of current evidence to guide management strategies.
Main Results:
- MCPM is characterized by small cysts of mesothelial cells lacking atypia, while WDPPM features a papillary component with bland mesothelial cells.
- Both variants often present incidentally or with symptoms like chronic abdominal pain, pelvic mass, or infertility.
- Despite slower growth, MCPM and WDPPM possess malignant transformation potential and high recurrence rates.
Conclusions:
- Complete cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (cisplatin and doxorubicin) is the recommended treatment approach for MCPM and WDPPM.
- Further collaborative, multi-institutional studies are essential to generate robust data and establish definitive clinical guidelines.
- Early diagnosis and appropriate surgical and chemotherapeutic intervention are key to improving outcomes for patients with these rare peritoneal mesothelioma variants.
