Malignant peritoneal mesothelioma

Last revised by Mohammad Taghi Niknejad on 22 Apr 2024

Malignant peritoneal mesothelioma is an uncommon primary tumor of the peritoneal lining. It shares epidemiological and pathological features with - but is less common than - its pleural counterpart, which is described in detail in the general article on mesothelioma. Other abdominal subtypes (also discussed separately) include:

Approximately 20-30% of all mesotheliomas arise from the peritoneal serosal lining. As with pleural mesothelioma, there is also a strong association with asbestos exposure (primarily the crocidolite variety), and this occupational hazard probably accounts for disease predominance in middle-aged to older males (most common age group is 50 to 70 years, and it may also account for the slightly increased male predilection).

Despite this, ~50% of cases have no history of exposure to asbestos. However, prior radiation exposure, exposure to thorium, talc, erionite, or mica, as well as in patients affected by familial Mediterranean fever and diffuse lymphocytic lymphoma, have also been reported.

The overall incidence is approximately 1-2 cases per million.

Typically patients present with weight loss, anorexia, abdominal pain and/or abdominal distension, pyrexia of unknown origin.

There can be symptoms associated with hypercoagulability particularly due to a paraneoplastic syndrome associated with mesothelioma.

There is a reported correlation between the clinical presentation and CT findings, with subgrouping into 2:

  • dry-painful type: presenting with abdominal pain, CT features of peritoneal mass(es) and little to no ascites

  • wet type: presenting with abdominal distension, CT findings of ascites and widespread nodules without a predominant mass

  • mixed type: both pain and ascites present

As with pleural mesothelioma, three histologic subtypes exist:

  • epithelioid (most frequent, 60 to 80%)

  • sarcomatoid (10 to 12%)

  • biphasic (mixed epithelioid - sarcomatoid,10 to 15%)

The epithelioid type composed of columnar, cuboidal or flattened cells arranged in gland-like structures or papillary architectures like adenocarcinoma. For differentiation between adenocarcinoma and mesothelioma, IHC staining is very useful. WT1, cytokeratin 5/6, podoplanin and calretinin are positive in mesothelioma but claudin 4 is negative. This panel is diagnostic in most cases of mesothelioma in the context of morphology and clinical findings.

The sarcomatoid type likes fibrosarcoma, less likely show imunorectivity for previously IHC markers and some may be positive just for keratin. The mixed type reveals both epithelioid and sarcomatoid appearance 10.

  • variable sonographic appearances, from sheet-like hypoechoic to echogenic masses 1,4

  • omental thickening

  • ascites

CT is useful for the detection, characterization, staging, and guiding biopsy of peritoneal masses. Features include:

  • solid, heterogeneous, enhancing soft tissue mass

  • presence of calcified pleural plaques, indicative of asbestos exposure

  • in contrast, peritoneal tumoral calcification is uncommon

  • nodular, irregular peritoneal and omental thickening, progressing to omental caking

  • peritoneal or omental masses

  • variable ascites (mild to moderate)

  • direct invasion of abdominal viscera, especially liver and colon.

  • concurrent pleural involvement is not uncommon on CT images at diagnosis 7

Peritoneal mesothelioma does not tend to spread to distant organs and lymphadenopathy is usually not a feature.

Peritoneal masses and nodules demonstrate 1:

  • T1: low-intermediate signal

  • T2: high signal

  • C+ (Gd): shows enhancement

  • gallium scan: diffuse uptake throughout the peritoneal cavity 4

Treatment options include cytoreductive surgery, peritonectomy and intraperitoneal chemotherapy. However, the prognosis is very poor, with death usually within one year 6.

General considerations include:

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