Leiomyomatosis peritonealis disseminata. Report of a case and review of the literature

Abstract

The eighth documented case of leimoyomatosis peritonealis disseminata (LPD) is presented. Histologic evidence for concomitant intravenous leiomyomatosis and early development of a leiomyoma in a vessel wal was noted in the present case. Electronmicroscopic studies proved the smooth muscle origin of the tumors in this case. Intriguing features of this disease are its grossly malignant appearance, relatively benign histology, and generally favorable clinical behavior. Because women of reproductive age, especially in the fourth decade, are affected, hormones may play a role in the etiology of LPD. Intraoperative diagnosis requires frozen-section examination and treatment has included extirpation of involved tissues, wherever possible. Also advocated is surgical castration which may remove the hormonal stimulus for the growth of any residual tumor. Since LPD bears a close resemblance to genital leiomyosarcoma, it warrants proper recognition and management.