TY - JOUR T1 - Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Ovary: A Case Report and Review of the Literature JF - Anticancer Research JO - Anticancer Res SP - 4483 LP - 4488 DO - 10.21873/anticanres.15258 VL - 41 IS - 9 AU - ANGIOLO GADDUCCI AU - CLARA UGOLINI AU - STEFANIA COSIO AU - FABIO VISTOLI AU - EMANUELE FEDERICO KAUFFMANN AU - UGO BOGGI Y1 - 2021/09/01 UR - http://ar.iiarjournals.org/content/41/9/4483.abstract N2 - Background: Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms located at various anatomic sites, which usually express both melanocytic and myogenic markers. Case Report: A 60-year-old woman underwent laparotomy for a huge, heterogeneous, right ovarian mass. The histological examination of the surgical specimen revealed a neoplasm consisting of both cells with clear or eosinophilic cytoplasm and spindle cells in a myxoid stroma. Immunostaining was positive for human melanoma black-45, h-caldesmon, desmin, actin, and transcription factor 3. Cell atypias were moderate, mitoses were 4/10 high power fields (HPF) and margins were focally infiltrative. These findings pointed to a diagnosis of ovarian PEComa. Twenty-five months later, two subcutaneous lesions were surgically removed on the left trapezius muscle and the median subumbilical area, respectively. The former was a desmoid fibromatosis, whereas the latter was a recurrence of PEComa with greater nuclear pleomorphism and higher number of mitoses (26/50 HPF) compared to the primary tumor. The patient was free of disease 11 months later. Conclusion: A long-term follow-up of gynecological PEComas is strongly recommended. ER -