title: Extraneural hemangioblastoma: a report of 5 cases.
abstract: Hemangioblastoma is a morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease, and which involves the central nervous system in the majority of the cases. Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported. The histogenesis of this tumor remains uncertain. Various cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth muscle/myofibroblastic have been proposed for the so-called stromal cells, which are thought to represent the neoplastic component of these lesions. We report on 5 cases of hemangioblastoma arising in extraneural tissues. Two of the tumors were located in the presacral region, and one each in the maxilla, kidney, and adrenal glands. All 5 cases were morphologically indistinguishable from central nervous system hemangioblastoma. The existence of these cases suggests that the "stromal" cells of hemangioblastoma can demonstrate a variety of mature specific lineages, such as smooth muscle/myofibroblastic, or neuroendocrine, depending on the location and possibly the microenvironment.
authors: Department of Pathology, New York University Medical Center, 560 First Avenue, New York, NY 10016, USA. daisuke.nonaka@med.nyu.edu
authors: Daisuke
authors: D
authors: Nonaka
authors: Jaime
authors: J
authors: Rodriguez
authors: Juan
authors: J
authors: Rosai
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D018572:Disease-Free Survival; D005260:Female; D018325:Hemangioblastoma / Q000737:chemistry / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008875:Middle Aged; D010524:Peripheral Nervous System Neoplasms / Q000737:chemistry / Q000150:complications / Q000473:pathology* / Q000601:surgery; D014746:Vimentin / Q000032:analysis; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D014746:Vimentin