From the archives of the AFIP: primary peritoneal tumors: imaging features with pathologic correlation

Abstract

Primary peritoneal tumors are uncommon lesions that arise from the mesothelial or submesothelial layers of the peritoneum. Primary malignant mesothelioma, multicystic mesothelioma, primary peritoneal serous carcinoma, leiomyomatosis peritonealis disseminata, and desmoplastic small round cell tumor are the most prominent of these rare lesions. Primary malignant mesothelioma is a highly aggressive malignancy that occurs most commonly in older men and that has a strong association with high levels of asbestos exposure. It manifests most often as diffuse sheetlike or nodular thickening of the peritoneal surfaces, but it may occasionally be a localized mass. Multicystic mesothelioma occurs most frequently in women and has benign or indolent biologic behavior in the majority of patients. It is a multilocular cystic mass that arises from the pelvic peritoneal surfaces. Primary peritoneal serous carcinoma occurs almost exclusively in women. It is histologically identical to ovarian serous carcinoma and may be indistinguishable from metastatic ovarian carcinoma at imaging studies. Leiomyomatosis peritonealis disseminata is a rare, benign proliferative process that also occurs exclusively in women and is characterized by multiple smooth muscle nodules throughout the peritoneum. Desmoplastic small round cell tumor is a highly aggressive malignancy of unknown origin that occurs most often in the peritoneal cavity of young men. This unusual group of tumors is linked together by a common site of origin and imaging manifestations that mimic those of peritoneal carcinomatosis. Knowledge of the spectrum of imaging findings in this group of primary peritoneal tumors, along with their clinical and pathologic characteristics, is important in the evaluation of patients with diffuse peritoneal disease.