[Cervical medullary metastasis in a patient with Von Hippel-Lindau disease]

K K Doe1, A Diaz, S Taha

  • 1Service de Neurochirurgie, CHU La Milétrie, 350, avenue Jacques-Coeur, 86021 Poitiers. doekarl@yahoo.fr

Neuro-Chirurgie
|December 4, 2003
PubMed

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.