hemangioblastoma

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Related to hemangioblastomas: pheochromocytoma

hemangioblastoma

 [he-man″je-o-blas-to´mah]
a benign blood vessel tumor of the cerebellum, spinal cord, or retina, consisting of proliferated blood vessel cells and angioblasts. Those arising in the cerebellum (cerebellar hemangioblastoma) may be cystic and associated with von hippel-lindau disease. Called also angioblastoma.
cerebellar hemangioblastoma hemangioblastoma of the cerebellum, often cystic; an autosomal dominant form is associated with von Hippel-Lindau disease.

he·man·gi·o·blas·to·ma

(he-man'jē-ō-blas-tō'mă),
A benign neoplasm frequently arising in the cerebellum composed of capillary vessel-forming endothelial cells and stromal cells; a slowly growing tumor that affects, primarily, middle-aged individuals; increased incidence in von Hippel-Lindau disease.

hemangioblastoma

/he·man·gio·blas·to·ma/ (he-man″je-o-blas-to´mah) a benign blood vessel tumor of the cerebellum, spinal cord, or retina, consisting of proliferated blood vessel cells and angioblasts.

hemangioblastoma

[hēman′jē·ōblastō′mə] pl. hemangioblastomas, hemangioblastomata
Etymology: Gk, haima + angeion, vessel, blastos, germ, oma, tumor
a brain tumor composed of a proliferation of capillaries and of disorganized clusters of capillary cells or angioblasts, usually occurring in the cerebellum.

Hemangioblastoma

(1) A tumour of soft tissues, which is of intermediate aggressiveness and thus preferably known as hemangioendothelioma.
(2) A vascularised cerebellar tumour, representing 2% of CNS neoplasms, which occurs in the 3rd–4th decades, either alone or associated with von-Hippel-Lindau syndrome, phaeochromocytoma, syringomyelia or erythrocythemia. 

Management
Excision.
 
Prognosis
Good.

he·man·gi·o·blas·to·ma

(hē-man'jē-ō-blas-tō'mă)
A benign cerebellar neoplasm composed of capillary vessel-forming endothelial cells and stromal cells; a slowly growing tumor that affects, primarily, middle-aged individuals; increased incidence in von Hippel-Lindau disease.
Synonym(s): angioblastoma, Lindau tumor, haemangioblastoma.

Lindau,

Arvid Wilhelm, Swedish pathologist, 1892-1958.
Lindau disease - Synonym(s): von Hippel-Lindau syndrome
Lindau tumor - a benign cerebellar neoplasm. Synonym(s): hemangioblastoma
von Hippel-Lindau syndrome - see under von Hippel
References in periodicals archive ?
Hemangioblastomas are benign (WHO grade I) but highly vascular, capillary-rich neoplasms.
42-44) Recently, inhibin A, D240, and brachyury have been found to be present in hemangioblastomas and may be helpful in differentiating them from other CCTs.
Sherman JH, Le BH, Okonkwo DO, Jane JA: Supratentorial Dural-based Hemangioblastoma not associated with Von Hippel Lindau complex.
Table 1: Focal spinal cord lesions in children Neoplastic Non-neoplastic Glial tumors: Demyelinating disease: Astrocytoma, ependymoma ADEM, MS, transverse myelitis Non-glial tumors: Vascular lesions: Hemangioblastoma, metastases, Cavernoma, AV fistula, ischemia PNET, germ cell tumor, melanoma Others: Syringohydromyelia Table 2: Differentiation between Astrocytoma and Ependymoma Astrocytoma Ependymoma Age group Pediatric Adult Location Eccentric Central Morphology Ill-defined Well-circumscribed Enhancement Patchy, irregular Focal, intense homogenous Hemorrhage/ Uncommon Common calcification Conus or filum No Yes (Myxopapillary)
The most common manifestations of VHL disease are retinal hemangioblastomas, central nervous system hemangioblastomas, and visceral tumors.
Clinical manifestations include central nervous system hemangioblastomas, retinal angiomas, and solid tumors, including clear cell RCC.
21,24) Surgical resection of the lesion is the treatment of choice for solitary hemangioblastomas.
287) von HippelLindau syndrome is a rare autosomal dominant familial cancer syndrome (retinal angiomas, hemangioblastomas, pheochromocytomas, ccRCC).
The diagnosis is based on the presence of multiple hemangioblastomas of the central nervous system (CNS), 1 hemangioblastoma and a visceral manifestation, or 1 central or visceral manifestation in a first-degree relative.
von Hippel-Lindau (VHL) syndrome is an autosomaldominant, inherited familial cancer syndrome predisposing patients to a variety of malignant and benign neoplasms, with retinal, cerebellar, and spinal hemangioblastomas, RCC, pheochromocytoma, and pancreatic tumors most frequent.
164) The VHL syndrome is a rare autosomal-dominant familial cancer syndrome (retinal angiomas, hemangioblastomas, pheochromocytomas, ccRCC).
D2-40, a novel monoclonal antibody against the M2A antigen as a marker to distinguish hemangioblastomas from renal cell carcinomas.