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Peritoneal cystic mesothelioma
Peritoneal cystic mesothelioma is a rare benign tumor characterized by the formation of intra-abdominal multilocular cystic masses.
ORPHA:168816Classification level: Disorder
- Benign multicystic peritoneal mesothelioma
- Multicystic mesothelioma
- Multilocular peritoneal inclusion cyst
- Prevalence: Unknown
- Inheritance: Unknown
- Age of onset: Adult
- ICD-10: C45.1
- OMIM: -
- UMLS: C0206680
- MeSH: -
- GARD: 10777
- MedDRA: -
Fewer than 150 cases have been reported to date. It occurs more frequently in women of child-bearing age.
Clinical features include lower abdominal or pelvic pain, presence of a palpable mass, amenorrhea, dysuria, dyspareunia, and more rarely weight loss. In women, the masses are generally located on the peritoneal surface of the uterus and rectum, while in men they are generally located on the peritoneal surface of the bladder and rectum. The tumor can also spread into the upper portions of the peritoneal cavity.
Peritoneal cystic mesothelioma seems to originate from the peritoneal mesothelium. However, etiology is unknown. There is no relation with asbestos exposure. Peritoneal cystic mesothelioma occurs frequently in women with previous history of surgery, pelvic inflammation or endometriosis. Chronic peritoneal irritation may be an aggravating factor.
Diagnosis is based on abdominal and pelvic ultrasound, abdominal computerized tomography (CT) and laparoscopic examination that reveal abdominal multicystic separated grapelike structures. Biopsy shows vascularized, translucent fluid-filled, thin-walled cysts composed of loose connective tissue. Immunohistochemistry confirms the diagnosis by showing the mesothelial origin of the parietal cells (this examination must be reviewed by an expert pathologist).
The main differential diagnoses include cystic peritoneal lymphangioma, pseudomyxoma peritonei (see this term), and other cystic neoplastic lesions.
Management and treatment
The treatment approach is multidisciplinary 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. Treatment is surgical and is based on ablation of cysts. In case of recurrence or even in first-line treatment, certain teams have proposed combination of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) (off-label use) in specific patients (young patients with good general status).
Prognosis is usually good. 5-year survival is 100% when the combined treatment is given. With surgery alone, disease inevitably recurs or persists. Invasive or malignant progression has been described.
A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011) Greek (2011, pdf)