title: Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature.
abstract: Angiosarcoma arising in the central or peripheral nervous system has rarely been reported. Eight patients with primary angiosarcoma of the central nervous system are described here; these included five males and three females ranging in age from 2 weeks to 72 years (mean 38 years). Of the eight neoplasms, six were located in the cerebral hemispheres and one was in the meninges; the site was unknown in the other. All patients underwent surgical resection. Five of the eight patients died, four within 4 months after surgery and one after 30 months. Two of the remaining three patients were 17 and 27 years old at the time of diagnosis and were alive at follow-up review 39 and 102 months after surgery, respectively. One patient was lost to follow-up monitoring. Microscopically, all eight tumors demonstrated a well-differentiated pattern with irregular vascular channels and intraluminal papillae; in addition, four showed poorly differentiated solid areas. Immunohistochemical staining of neoplastic cells to factor VIII-related antigen and Ulex europaeus agglutinin I was performed in five tumors and was focally positive in four. No correlation could be shown between the histological features and the growth and biological behavior of the tumors.
authors.affiliation: Department of Neuropathology, Armed Forces Institute of Pathology, Washington, D.C.
authors.forename: H
authors.initials: H
authors.lastname: Mena
authors.forename: J L
authors.initials: JL
authors.lastname: Ribas
authors.forename: F M
authors.initials: FM
authors.lastname: Enzinger
authors.forename: J E
authors.initials: JE
authors.lastname: Parisi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006394:Hemangiosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009418:S100 Proteins / Q000032:analysis; D015996:Survival Rate; D014746:Vimentin / Q000032:analysis
chemical_list: D009418:S100 Proteins; D014746:Vimentin