title: Pleomorphic hyalinizing angiectatic tumor of soft parts: case report with unusual ganglion-like cells and review of the literature.
abstract: Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described, non-metastasizing tumor of uncertain lineage. This tumor distributes equally between the genders and has a predilection for the subcutaneous soft tissue, particularly in lower extremity, other locations are rare. Based on the recent literature, PHAT is suspected to encompass the morphological spectrum with other tumors such as myxoinflammatory fibroblastic sarcoma (MIFS) and hemosiderotic fibrolipomatous tumor (HFLT), although cytogenetic data remain inconsistent. We report a case of PHAT that arose in the upper arm with unusual morphology which showed ganglion-like cells similar to Reed-Sternberg-like cells found in MIFS. The tumor had strong immunohistochemical expression of CD34, CD99, and was negative for S-100. The ganglion-like cells were positive for both CD34 and CD68 but negative for CD30. The translocation between chromosome 1 and 10, a frequent finding of MIFS and HFLT, was not identified by FISH excluding the possibility of hybrid PHAT and MIFS. We conclude FISH can be a potential useful tool to separate PHAT with atypical morphology from hybrid tumor in doubted cases. Due to the rarity of PHAT and lack of consistent pathogenetic signatures, more cases and further studies will be needed to elucidate the pathogenesis and nature of this tumor.
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: ccwilly@hotmail.com.
authors.forename: Yi-Che
authors.initials: YC
authors.lastname: Changchien
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: pal.bocskai@freemail.hu.
authors.forename: Pál
authors.initials: P
authors.lastname: Bocskai
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: csupai@gmail.com.
authors.forename: Ilona
authors.initials: I
authors.lastname: Kovács
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: z.hargy@gmail.hu.
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Hargitai
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: dr.kollar.sandor@gmail.com.
authors.forename: Sándor
authors.initials: S
authors.lastname: Kollár
authors.affiliation: Department of Pathology, Kenézy Gyula County Hospital, Debrecen, Hungary. Electronic address: torok-miklos@freemail.hu.
authors.forename: Miklós
authors.initials: M
authors.lastname: Török
mesh_terms: D000368:Aged; D045888:Ganglion Cysts / Q000473:pathology; D015972:Gene Expression Regulation, Neoplastic / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence / Q000379:methods; D008297:Male; D017463:Receptors, Complement 3b / Q000378:metabolism*; D012509:Sarcoma / Q000175:diagnosis / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000175:diagnosis / Q000473:pathology*; D014178:Translocation, Genetic / Q000235:genetics
chemical_list: C509991:CR1 protein, human; D017463:Receptors, Complement 3b
keywords: CD34; Ganglion-like cells; Myxoinflammatory fibroblastic sarcoma; Pleomorphic hyalinizing angiectatic tumor; t(1 ;10)