LYMPHANGIOMYOMATOSIS OF THE UTERUS ASSOCIATED WITH TUBEROUS SCLEROSISAND MALIGNANT NEOPLASIA OF THE FEMALE GENITAL-TRACT - A REPORT OF 2 CASES

Lymphangiomyomatosis (LAM) is a rare disease that does not generally a ffect the female genital tract. We report two cases of uterine involve ment by LAM in young women with tuberous sclerosis and renal angiomyol ipomas. In both, the uterine lesions were grossly inapparent and were discovered during microscopic examination of hysterectomy specimens re moved during surgical treatment for a primary ovarian adenocarcinoma w ith peritoneal and lymph node metastases in one case and a retroperito neal lymphangiomyoma in the other. In one case, an area of uterine LAM with atypical features was interpreted as focal sarcomatous transform ation. This patient also had pelvic and paraaortic lymph node involvem ent by typical lymphangiomyomas, a small uterine angiomyoma, and an oc cult primary endometrial adenocarcinoma. Immunostains for HMB-45 were strongly positive in the uterine LAM in both cases, the retroperitonea l and lymph node lymphangiomyomatous lesions, the uterine angiomyoma, and a resected rend angiomyolipoma. Although LAM is a rare uterine les ion, it must be distinguished from a variety of uterine smooth-muscle tumors.