Multifocal PEComa (PEComatosis) of the Female Genital Tract Associated With Endometriosis, Diffuse Adenomyosis, and Endometrial Atypical Hyperplasia

Many of the reported PEComas have been asso-ciated with the tuberous sclerosis complex (TSC), an inherited disorder characterized by mutation of the TSC1 and TSC2 genes.4,9 Here, we describe the clinical, histological, and immunohistochemical fea-tures of a case of severe tuberous sclerosis in which extensive uterine endometriosis and adenomyosis with complex atypical hyperplasia were found in association with PEComa involving the uterus and multiple pelvic sites. Material and Methods The surgical specimen was fixed in 10 % formalin and processed for histological examination by con-ventional methods. In addition to hematoxylin and eosin, immunohistochemical stains were performed using formalin-fixed, paraffin-embedded sections. The avidin–biotin peroxidase complex technique and the peroxidase–antiperoxidase technique were used with the following antibodies: HMB-45 (Dako, Denmark), smooth muscle actin (Thermoscientific, USA), calretinin (Swant, Switzerland), cytokeratin 7