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Pathological and Clinical Features and Management of Central Nervous System
Hemangioblastomas in von Hippel-Lindau Disease
#MMPMID28326249
Kanno H
; Kobayashi N
; Nakanowatari S
J Kidney Cancer VHL
2014[]; 1
(4
): 46-55
PMID28326249
show ga
Central nervous system (CNS) hemangioblastoma is the most common manifestation of
von Hippel-Lindau (VHL) disease. It is found in 70-80% of VHL patients.
Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting
for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS
hemangioblastomas are typically located in the posterior fossa and the spinal
cord. VHL patients usually develop a CNS hemangioblastoma at an early age.
Therefore, they require a special routine for diagnosis, treatment and follow-up.
The surgical management of symptomatic tumors depend on many factors such as
symptom, location, multiplicity, and progression of the tumor. The management of
asymptomatic tumors in VHL patients are controversial since CNS hemangioblastomas
grow with intermittent quiescent and rapid-growth phases. Preoperative
embolization of large solid hemangioblastomas prevents perioperative hemorrhage
but is not necessary in every case. Radiotherapy should be reserved for
inoperable tumors. Because of complexities of VHL, a better understanding of the
pathological and clinical features of hemangioblastoma in VHL is essential for
its proper management.
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