Case 1 − A case of gynecological PEComa

Abstract

Perivascular epithelioid cell tumors (PEComas) are a heterogeneous group of rare mesenchymal neoplasms composed of epithelioid cells which express melanocytic and myogenic markers, such as HMB-45, desmin and actin. In March 2013, we visited a postmenopausal 51-year-old woman with a suspected diagnosis of uterine PEComa diagnosed by a dilation and curettage of the lining of the uterus. After the histological revision of the formalin-fixed paraffin-embedded (FFPE) material by our expert pathologist, we confirmed the diagnosis and referred the patient for complete primary surgery. On November 2013, the patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with no macroscopically detectable residual cancer. However, in January 2014, due to voiding dysfunction and inconstant vaginal bleeding, the patient underwent magnetic resonance imaging (MRI) which documented multiple irregular lesions in the pelvis suspected as recurrent PEComa. Considering the early relapse of PEComa after optimal primary surgery, we suggested a systemic treatment with the combination of gemcitabine and docetaxel. For logistic reasons, the patients started the chemotherapy in her district hospital. After two cycles of chemotherapy the patient died due to treatment-related complications.