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Abdominal Cancer Alliance

What is Peritoneal Mesothelioma?

Peritoneal mesothelioma is a malignancy that originates in the lining of the peritoneum, the thin membrane that bounds the abdominal and pelvic cavities. Though rare and difficult to diagnose, aggressive treatment has been shown to extend life and quality of life in peritoneal mesothelioma patients.

The Basics

Malignant mesothelioma is a rare, aggressive form of cancer that develops in the mesothelial cells that form the lining of the pleura (lungs), peritoneum (abdominal cavity), pericardium (heart), and testicles (testes).

Peritoneal mesothelioma is the second most common type of mesothelioma and forms in the lining of the abdominal cavity. Only about 500 cases of peritoneal mesothelioma are diagnosed annually in the United States accounting for 15-30% of all mesotheliomas. [see References: 1,2] The average survival for patients with peritoneal mesothelioma is approximately one year; however, this can extend to 3-5 years with aggressive treatment.[3-9]

Peritoneal mesothelioma is caused by exposure to asbestos, a fibrous material woven into fabrics for fire-resistant and insulating materials. There are two suggested routes of asbestos exposure into the abdominal cavity: 1) through ingestion and 2) inhaled and transferred through the lymphatic system. Peritoneal mesothelioma is slow-growing and can take up to 10-50 years from exposure for symptoms to develop.[10]

Abdominal Cancer Alliance

Risk Factors

  • Asbestos Exposure: Working at or living near an asbestos mine or processing plant; Working in high-risk occupational setting, such as automotive industry; Serving on military ships and facilities built with asbestos; Disturbing old asbestos products during home renovation

  • Gender: occurs more commonly in men

  • Age: diagnosis occurs around age 65 and older

  • Genetics: associated with a rare BAP1 mutation

  • Smoking: combined with asbestos exposure

  • Radiation: the exact relationship is not fully understood

  • Simian Virus 40: exposure to this virus may increase risk; however, the exact relationship is not fully understood

Stomach Ache

Signs & Symptoms

  • Abdominal pain

  • Nausea and vomiting

  • Change in bowel habits

  • Abdominal swelling/ascites

  • Early satiety

  • Unintentional weight loss

Key Facts

Asbestos

Peritoneal mesothelioma is most commonly linked to environmental exposure to asbestos.

Difficult Diagnosis

It is considered a very lethal disease that is difficult to diagnose due to vague symptoms.
 

Improved Treatment

Significant advances have been made in the treatment of peritoneal mesothelioma, improving both survival (median survival increased from less than 1 year to 3-5 years) and tolerability.

CRS/HIPEC

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is the treatment of choice for peritoneal mesothelioma, with the best outcomes achieved when all visible disease is removed.

Diagnosis and Prognosis

Malignant peritoneal mesothelioma includes three subtypes: epithelioid, sarcomatoid, and biphasic (mixed epithelioid and sarcomatoid). Epitheliod is the most common type, accounting for 50-70% of cases, and associated with the best survival outcomes. Pure sarcomatoid tumors are extremely rare, with most data limited to single case reports.[13] Regardless of subtype, complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is the treatment of choice. Factors independently associated with favorable overall survival include: cancer subtype, absence of lymph node metastases, completeness of cytoreduction, and administration of HIPEC.[14]

Benign Cystic Mesothelioma & Peritoneal Inclusion Cysts

Cystic mesothelial lesions, including benign cystic mesothelioma and peritoneal inclusion cysts, pose a significant challenge for both pathologists and clinicians. While many are considered benign or reactional in nature, rather than a true neoplastic process (which forms abnormal and excessive tissue growth), the majority of patients present with multiple lesions and is associated with significant morbidity (symptoms/complications) and risk for recurrence or malignant transformation.[15] As these are very rare, with fewer than 200 cases reported in the literature, there is little information about optimal treatment. However, it is typically agreed that complete surgical resection with or without HIPEC is advisable and should be completed by specialized centers.[16]

Other Resources

More Information on Peritoneal Mesothelioma:
The Mesothelioma Center
American Cancer Society
Patient Support
Mesothelioma Applied Research Foundation
Mesothelioma Applied Research Foundation

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References

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