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

(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 ?
Se realizo la embolizacion del tumor cerebral sin reseccion quirurgica en un paciente que acudio a consulta con sintomatologia de vertigo a causa de un tumor cerebral sugestivo de hemangioblastoma evidenciado en estudios de imagen (Tomografia computarizada); el paciente rechazo la reseccion quirurgica, aceptando unicamente la embolizacion con Onyx.
The differential diagnosis included neurofibroma or schwannoma, as well as other rare dumbbell-shaped tumors of the spine (such as meningiomas and hemangiomas), malignant peripheral nerve sheath tumors, hemangioblastomas, or metastases.
Further clinical developments during follow-up, hemangioblastoma of CNS in the first case and pancreatic neuroendocrine tumor (PNET) in the second case, led to the diagnosis of VHL disease.
Von-Hippel Lindau (VHL) syndrome is a rare autosomal dominant disease and is characterized by the presence of benign and/or malign tumors in various tissues and organs such as the central nervous system and retinal hemangioblastoma, pheochromocytoma, renal cyst, and renal cell carcinoma (6,7).
They have a high risk of RCC and also present with both retinal and CNS hemangioblastomas; however, pheochromocytomas are uncommon [9].
Roggendorf, "Histogenesis of stromal cells in cerebellar hemangioblastomas. An immunohistochemical study," American Journal of Pathology, vol.
(17,20 ) A French study on 1 315 cases found most discrepancies were in gliomas, hemangioblastomas, and metastatic tumors.
(4) Genetic predisposition to the development of CCRCC (termed VHL syndrome) is associated with germline VHL mutations, and patients with the VHL gene often develop numerous, bilateral CCRCCs beginning at an early age (5); they may also develop paragangliomas, pancreatic tumors, hemangioblastomas, endolymphatic sac tumors, and papillary cystadenomas of the epididymis/ broad ligament.
Treatment of intramedullary hemangioblastomas, with special attention to von Hippel-Lindau disease.
Graph 2: Distribution of Intra-Axial Lesions in Adults METASTASIS 8 40% HEMANGIOBLASTOMAS 5 25% ASTROCYTOMA 3 15% IV EPIDERMOID 2 10% CPP 1 5% MEDULLOBLASTOMA 1 5% Note: Table made from bar graph.
For the central nervous system (CNS), it has been reported that screening hemangioblastomas in asymptomatic individuals was not indicated since surgery was considered only in symptomatic subjects.
Both were born with the gene that can cause VHL (Von Hippel-Lindau syndrome, a hereditary condition associated with Hemangioblastomas, which are blood vessel tumours of the brain, spinal cord, and eye).