Malignant gonadal mesothelioma

Malignant mesothelioma of the gonads is a rare and highly lethal disease. Most of these tumors arise from the tunica vaginalis, which is a continuation of the mesothelium similar to the pleura and the peritoneum. However, intratesticular and ovarian mesotheliomas have also been described. Occasionally, patients with localized disease at the time of detection have been known to survive for more than 10 years; however, the majority will not live beyond 5 years, with median survival being approximately 23 months. The principle reasons for this are difficulty in making a preoperative diagnosis and advanced stage at the time of treatment. Surgery forms the mainstay of management for all stages of the tumor. Adjuvant therapy in the form of chemotherapy, immunotherapy, or radiotherapy has negligible benefit. It is essential to diagnose this rare entity at the early stages to allow complete surgical extirpation. For the management of localized disease, we suggest the following protocol: initial staging of suspected cases with computed tomographic scan of the abdomen and pelvis; radical inguinal orchiectomy or hemiscrotectomy; retroperitoneal lymph node dissection in cases with positive nodes on scan or biopsy; and inguinal node dissection in cases requiring hemiscrotectomy. For advanced or recurrent disease, we suggest local radical resection with chemotherapy, including high-dose cisplatin and doxorubicin for two cycles of 5 days each; add local radiotherapy for uncontrolled locally advanced disease.