title: Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs.
abstract: Perivascular epithelioid cell tumor (PEComas), other than angiomyolipoma, clear cell 'sugar' tumor of the lung, and lymphangioleiomyomatosis, is an uncommon mesenchymal neoplasm that arises in the soft tissue and visceral organs. We report herein two cases of sclerosing PEComa; a distinctive variant of PEComa, which is characterized by extensive stromal hyalinization, occurring in the uterus and broad ligament. The patients were 34- and 51-year-old females with no family history of tuberous sclerosis complex. Macroscopically, the tumors had white to gray cut surfaces and were microscopically composed of predominantly spindle- to polygon-shaped cells with clear to slightly eosinophilic cytoplasm and pleomorphic nuclei focally arranged in a perivascular pattern, accompanied by marked stromal hyalinization. These tumor cells were immunohistochemically positive for HMB45 and α-smooth muscle actin. Although this variant of PEComa is very rare, this entity should be considered as a potential primary neoplasm of the female genital organs.
authors: Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors: Yuichi
authors: Y
authors: Yamada
authors: Hidetaka
authors: H
authors: Yamamoto
authors: Yoshihiro
authors: Y
authors: Ohishi
authors: Kenichi
authors: K
authors: Nishiyama
authors: Masao
authors: M
authors: Fukuhara
authors: Toshiaki
authors: T
authors: Saitou
authors: Masazumi
authors: M
authors: Tsuneyoshi
authors: Yoshinao
authors: Y
authors: Oda
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001956:Broad Ligament / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D012598:Sclerosis; D014594:Uterine Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor