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Malignant peritoneal mesothelioma
Malignant peritoneal mesothelioma is a primary peritoneal malignancy occurring in the lining cells (mesothelium) of the peritoneal cavity.
ORPHA:168811Classification level: Disorder
- Diffuse malignant peritoneal mesothelioma
- Primary malignant peritoneal mesothelioma
- Prevalence: 1-9 / 100 000
- Inheritance: Multigenic/multifactorial or Not applicable
- Age of onset: Adult
- ICD-10: C45.1
- ICD-11: 2C51.2
- OMIM: -
- UMLS: C0346109
- MeSH: -
- GARD: -
- MedDRA: 10056558
Peritoneal mesothelioma accounts for 10 to 30% of all malignant mesotheliomas. The annual incidence is approximately 1/500,000 in France but reaches 1/200,000 in some parts of Europe (Italy). Men are predominantly affected.
The tumors are usually diagnosed in late adulthood (median age: 55 years). Typical presenting features are abdominal distention, abdominal pain, presence of an abdominal mass, impaired general state, weight loss, and ascites. Dyspnea, coagulation disorders, edema of the lower limbs and intestinal occlusion may be observed.
The relationship between peritoneal mesothelioma and asbestos exposure is unclear, especially in women, and has not been established unlike in malignant pleural mesothelioma. Other causes have been reported such as exposure to erionite, viral infection and vaccine products and/or genetic factors.
Diagnosis is based on imaging techniques, such as ultrasound and chest-abdominal-pelvic computed tomography (CAP-CT). Diagnosis is confirmed histologically on tissue biopsy and by relevant immunostaining results (positive for calretinin and negative for carcinoembryonic antigen (CEA)), and should be performed by two experts.
Differential diagnosis includes peritoneal carcinomatosis secondary to colorectal or gastric cancer and primary peritoneal carcinoma (see this term).
Management and treatment
Treatment strategies require a multidisciplinary approach and must be discussed by a panel of physicians in a specialized center. There are currently no validated recommendations on clinical management and no cytotoxic agents have been granted a European Marketing Authorization (MA) in this indication. Currently proposed treatment with curative intent involves a combination of cytoreductive surgery (visceral resections and peritonectomy procedures) with hyperthermic intraperitoneal chemotherapy (HIPEC) (off-label use) in specific patients (young, good general status, low tumor volume). Systemic chemotherapy (off-label use) is sometimes used in palliative treatment.
With palliative treatment (systemic chemotherapy), median survival does not reach 1 to 2 years. Following cytoreductive surgery and HIPEC, median survival of more than 50 months, and 5-year survival of more than 50% may be obtained.
A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011) Greek (2011, pdf)
- Clinical practice guidelines
- English (2021) - Eur J Surg Oncol
: produced/endorsed by FSMR(s)