Pleural mesothelioma in a patient with Familial Mediterranean Fever: A case report

Malignant mesothelioma is a rare, lethal neoplasm of mesothelial surfaces, classically attributed to asbestos exposure. However, as regulations have reduced asbestos use, there is an increased focus on non-asbestos-related causes. Among these, the chronic serosal inflammation characteristic of Familial Mediterranean Fever (FMF) has been reported in a few cases. Although a direct causal relationship has not yet been established, such cases are critical for identifying the potential long-term risks of chronic serosal inflammation. Here, we present the fourth documented case of pleural mesothelioma associated with FMF.

A 55-year-old male patient with known FMF was admitted to the hospital with progressive shortness of breath and cough for one month. Physical examination and chest X-ray revealed a large left-sided pleural effusion. Thoracocentesis analysis demonstrated an exudative effusion with predominant lymphocytosis. A contrast-enhanced computed tomography (CT) scan showed pleural thickening with enlarged mediastinal lymph nodes. Positron emission tomography (PET) scan revealed avid pleural uptake (SUVmax = 7.5). A pleural biopsy was therefore performed, revealing epithelioid pleural mesothelioma. The patient had no known history of asbestos exposure. However, he was poorly compliant with colchicine treatment and experienced frequent episodes of serositis, occurring on average twice monthly. The patient underwent surgical resection followed by chemotherapy and is doing well 10 months after the initial presentation.

Many cases of FMF have been reported in association with peritoneal mesothelioma, but few have been linked to pleural mesothelioma. In this case, we highlight that chronic serosal inflammation, characteristic of untreated FMF, may represent a potential risk factor for non-asbestos-related malignant pleural mesothelioma. Larger-scale registry studies may be required to establish a statistically significant association.

This case reinforces the hypothesis that uncontrolled FMF may predispose patients to malignant mesothelioma. The presence of such an association would further stress the importance of early recognition and management of FMF.