[Cervical medullary metastasis in a patient with Von Hippel-Lindau disease].
Von Hippel-Lindau disease is an autosomal dominant disorder mainly characterized by hemangioblastoma(s) associated with kidney clear-cell carcinoma. Hemangioblastoma represents 2 to 3% of all central nervous system tumors whereas extramedullary metastasis accounts for 30 to 50% of all intravertebral column tumors. The most common sites of metastatic dissemination in order of frequency are the lung, breast, prostate and kidney. We report a case of an unusual cervical intradural extramedullary metastasis of clear-cell kidney carcinoma associated with a cervical spinal cord hemangioblastoma in a 45-year-old patient with Von Hippel-Lindau disease who had developed retinal hemangioblastomas 20 years earlier. A review of the relevant literature is proposed.
Doe
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Service de Neurochirurgie, CHU La Milétrie, 350, avenue Jacques-Coeur, 86021 Poitiers. doekarl@yahoo.fr
Diaz
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A
Taha
S
S
Lerich
B
B
Lapierre
F
F
D000328:Adult; D000368:Aged; D002548:Cerebral Revascularization / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D019299:Decompression, Surgical / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D004576:Electromyography; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005154:Facial Nerve / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D019569:Hemifacial Spasm / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016343:Monitoring, Intraoperative; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome