Primary perivascular epithelioid cell tumor (pecoma) of the ovary: A case report and review of the literature

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.