title: Intracranial intraaxial cerebral tufted angioma: case report.
abstract: Tufted angioma (TA) is a rare, slow-growing, vascular lesion that commonly presents as a solitary macule, papule, or nodule arising in the soft tissues of the torso, extremities, and head and neck in children and young adults. Adult-onset cases have been infrequently reported. While typically benign, TAs may be locally aggressive. Complete physical examination and hematological workup are recommended in patients with TA to exclude the presence of Kasabach-Merritt phenomenon (KMP). The authors describe the case of a 69-year-old man with a contrast-enhancing frontal lobe lesion, with surrounding vasogenic edema, which was treated by gross-total resection. Characteristic histological features of a TA were demonstrated, with multiple cannonball-like tufts of densely packed capillaries emanating from intraparenchymal vessels in cerebral cortex and adjacent white matter. Tumor recurrence was detected after 4 months and treated with adjuvant Gamma Knife radiosurgery. To the extent of the authors' knowledge, this case illustrates the first report of TA presenting in an adult as an intracranial intraaxial tumor without associated KMP. The fairly rapid regrowth of this tumor, requiring adjuvant treatment after resection, is consistent with a potential for locally aggressive growth in a TA occurring in the brain.
authors.affiliation: Departments of1Neurological Surgery.
authors.forename: Randy S
authors.initials: RS
authors.lastname: D'Amico
authors.affiliation: 2Pathology and Cell Biology.
authors.forename: George
authors.initials: G
authors.lastname: Zanazzi
authors.affiliation: 2Pathology and Cell Biology.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Hargus
authors.affiliation: Departments of1Neurological Surgery.
authors.forename: Timothy
authors.initials: T
authors.lastname: Dyster
authors.affiliation: 3Radiology, and.
authors.forename: Shirley
authors.initials: S
authors.lastname: Chan
authors.affiliation: 3Radiology, and.
authors.forename: Angela
authors.initials: A
authors.lastname: Lignelli-Dipple
authors.affiliation: 4Radiation Oncology, Columbia University Medical Center, New York, New York.
authors.forename: Tony J C
authors.initials: TJC
authors.lastname: Wang
authors.affiliation: 2Pathology and Cell Biology.
authors.forename: Phyllis L
authors.initials: PL
authors.lastname: Faust
authors.affiliation: Departments of1Neurological Surgery.
authors.forename: Guy M
authors.initials: GM
authors.lastname: McKhann
mesh_terms: D000368:Aged; D001929:Brain Edema / Q000209:etiology; D001932:Brain Neoplasms / Q000000981:diagnostic imaging* / Q000601:surgery*; D002196:Capillaries / Q000000981:diagnostic imaging; D006391:Hemangioma / Q000000981:diagnostic imaging* / Q000601:surgery*; D006801:Humans; D059885:Kasabach-Merritt Syndrome / Q000000981:diagnostic imaging / Q000601:surgery; D008297:Male; D009364:Neoplasm Recurrence, Local; D019635:Neurosurgical Procedures / Q000379:methods*; D016634:Radiosurgery / Q000379:methods*; D012878:Skin Neoplasms / Q000000981:diagnostic imaging* / Q000601:surgery*; D016896:Treatment Outcome
keywords: GFAP = glial fibrillary acidic protein; GKRS = Gamma Knife radiosurgery; KHE = kaposiform hemangioendothelioma; KMP = Kasabach-Merritt phenomenon; Kasabach-Merritt phenomenon; TA = tufted angioma; brain tumor; intracranial; kaposiform hemangioendothelioma; tufted angioma; vascular disorders