Leiomyomatosis peritonealis disseminata: Does malignant transformation occur? A literature review

Leiomyomatosis peritonealis disseminata (LPD) is a rare smooth muscle tumor . In the literature more than 100 cases have been described. LPD is charact erized by multiple small nodules on the peritoneal surface, mimicking a mal ignant process with metastases, but generally demonstrates benign histologi c features. Exposure to estrogen seems to play an etiologic role, Many pati ents have uterine leiomyomas as well, The diagnosis of LPD is easily made o n biopsy, Reduction of estrogen exposure is generally sufficient to cause r egression of LPD. Surgical castration or gone; dotrophin releasing hormone agonists seem good alternatives in the case of progression or recurrence of LPD. In six patients a malignant leiomyosarcoma has been described shortly after the diagnosis of LPD was made. Five of these patients did not have u terine leiomyomas or exposure to exogenous or increased endogenous estrogen . The relationship with pregnancy in the sixth patient may be coincidental. Whether malignant transformation of LPD occurs remains uncertain. Characte ristics of these patients differ from those of LPD patients and may indicat e a high malignant potential, necessitating a different approach. (C) 1999 Academic Press.