P.037 Disseminated leptomeningeal hemangioblastoma in a case of Von Hippel Lindau
| Autor: | P Snow, P Bartlett, N Hache, B Galway, Falah B. Maroun, JC Jacob, A Engelbrecht |
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| Rok vydání: | 2018 |
| Předmět: | |
| Zdroj: | Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques. 45:S25-S26 |
| ISSN: | 2057-0155 0317-1671 |
| Popis: | Background: Leptomeningeal dissemination of hemangioblastomas (HB), whether sporadic or associated with Von Hippel Lindau (VHL), are extremely rare. Very scanty literature is available. Methods: A 36 year old female with VHL and stable pancreatic, adrenal and renal lesions was operated upon 4 years ago for a large symptomatic cervicomedullary cystic and solid tumor. 2 years after surgery the tumour recurred and further removal was unsuccessful due to medullary adhesions. Radiation was given to the posterior fossa area and to several small nodules over the cauda equina resulting in severe pain. Serial follow up imaging revealed diffuse leptomeningeal dissemination increasing in size of the suprasellar region, ambient cistern and Sylvian fissures. Clinically, she has been stable with small dose of steroids and VP shunt insertion for papilledema. Results: Review of the literature consists of 2 series of 7 and 21 patients each with leptomeningeal dissemination involving sporadic HB and VHL associated HB. Leptomeningeal dissemination is estimated at about 4.3%. It is postulated that the tumour starts in the Pia and spreads in an extra medullary fashion throughout the subarachnoid spaces. Conclusions: Long term recurrence has been noted raising the question of aggressive treatment with some drug therapy related to angiogenesis is postulated. |
| Databáze: | OpenAIRE |
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