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[Malignant peritoneal mesothelioma occurring in periodic disease: apropos of a case]
G Belange1, H Gompel, Y Chaouat
1Service de médecine interne, Fondation A-de-Rothschild, Paris, France.
Introduction:
Peritoneal mesothelioma is a rare malignant neoplasm that might be linked to chronic peritoneal inflammation. As well, the association peritoneal mesothelioma-familial Mediterranean fever is uncommon.
Exegesis:
We report the case of a 60-year-old man who presented for 30 years with standard periodic familial Mediterranean fever accompanied by acute abdominal episodes, sensitive to colchicine. Between 1988 and 1995, acute abdominal episodes were accompanied by more and more profuse recurrent ascites, partially resolving under colchicine treatment. In 1995, the last episode was severe (with loss of weight and inability to tolerate feeding) and conducted to the patient's death due to peritoneal mesothelioma, as confirmed by the biopsy.
Conclusion:
Profuse and recurrent ascites is unusual in standard periodic familial Mediterranean fever. Asbestos exposure at the origin of peritoneal mesothelioma is not well documented. Furthermore, the disease clinical and paraclinical features are misleading, and the diagnosis is based on histology. The prognosis is severe, and treatment is usually disappointing. Our observation clearly demonstrates the interconnection between an unusual form of profuse and relapsing ascites that occurred in the course of a periodic disease and peritoneal mesothelioma. The potential role of recurrent peritonitis related to familial Mediterranean fever in the pathogenesis of the tumor is discussed.
Insights
Familial Mediterranean fever can present with unusual ascites, potentially linked to peritoneal mesothelioma. This rare cancer, often diagnosed late, highlights the need to consider inflammatory conditions in its pathogenesis.
Area of Science:
- Oncology
- Gastroenterology
- Genetics
Background:
- Peritoneal mesothelioma is a rare malignancy potentially associated with chronic peritoneal inflammation.
- The co-occurrence of peritoneal mesothelioma and familial Mediterranean fever (FMF) is infrequent.
Observation:
- A 60-year-old man with a 30-year history of familial Mediterranean fever experienced recurrent, profuse ascites unresponsive to colchicine.
- The patient's condition worsened, leading to death from peritoneal mesothelioma, confirmed by biopsy.
Findings:
- Unusual profuse and recurrent ascites is atypical for standard familial Mediterranean fever.
- The diagnosis of peritoneal mesothelioma is challenging due to misleading clinical features and relies on histology.
- Asbestos exposure, a common cause of peritoneal mesothelioma, is not well-documented in this case.
Implications:
- This case underscores a potential link between recurrent peritonitis in familial Mediterranean fever and the development of peritoneal mesothelioma.
- The findings suggest a possible role for chronic inflammation in familial Mediterranean fever pathogenesis of peritoneal tumors.
- Further research is warranted to explore the interplay between inflammatory conditions and peritoneal malignancies.