ABSTRACT
Perivascular Epithelioid Cell Tumor (PEComa) is part of an extremely rare family of mesenchymal tumors. We present a case of Peritoneal Pecomatosis with pulmonary metastases in a 35-year-old woman, with previous multiple myomectomies, uterine artery embolization and severe pos-partum hemorrhage. Given previous medical history, two years after birth a routine transvaginal ultrasound was performed which showed nonspecific, multiple, highly vascularized solid nodules in the uterus, peritoneum and rectus abdominis, confirmed by Magnetic Resonance Imaging. The nodules were surgically removed and diagnosed as multifocal PEComa with positive expression for estrogen receptors. The disease progressed with pulmonary metastasis and it was decided to start systemic therapy with Tamoxifen (20 mg daily). During the following two-year close surveillance, the patient was clinically and imagiologicaly stable, without disease progression. PEComas are very unusual tumors and the differential diagnosis include fibroids. In estrogen receptor positive tumors, Tamoxifen may be considered as a therapeutic option.