RT Journal Article SR Electronic T1 Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Ovary: A Case Report and Review of the Literature JF Anticancer Research JO Anticancer Res FD International Institute of Anticancer Research SP 4483 OP 4488 DO 10.21873/anticanres.15258 VO 41 IS 9 A1 ANGIOLO GADDUCCI A1 CLARA UGOLINI A1 STEFANIA COSIO A1 FABIO VISTOLI A1 EMANUELE FEDERICO KAUFFMANN A1 UGO BOGGI YR 2021 UL http://ar.iiarjournals.org/content/41/9/4483.abstract AB 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.