Revista argentina de neurocirugía
versión On-line ISSN 1850-1532
MANZONE, Patricio et al. Neurofibromatosis tipo 1 y tumores raquídeos del contenido. Rev. argent. neurocir. [online]. 2006, vol.20, n.2, pp. 61-68. ISSN 1850-1532.
Objetive: To assess prevalence, prognosis and therapeutic possibilities of patients with type 1 neurofibromatosis (NF-1) and spinal tumors. Method: All patients with NF-1admitted to our Hospital settlings since January 1993 to December 2002 were analized, the clinical records and image studies of those with spinal tumors as a cause of admission were retrospectively reviewed. Karnofsky scale, Functional World Health Organization (WHO) scale, Frankel grade and Visual Analogical Scale (VAS) for pain were used at hospital admission, postoperatively and at follow-up. Surgical staging was reviewed with the Weinstein Boriani Bacci system. Results: In this 10 years period we admitted 44 patients who fully satisfied NIH criteria for NF-1. Twelve of them (27.3%) presented symptomatic spinal tumors: 9 males and 3 females, in average 25.2 years old. Spinal tumors were 9 benign (all neurofibromas) and 3 malign. Mean follow up was 2.8 years. Fourteen surgical procedures were performed in 11 patients. Eleven complications occurred in 6 operated cases (50% of patients), but 2 of 3 (67%) malignant spinal tumors cases had complications. Two of 8 benign tumors operated (25%) recurred an average of 3.3 years after surgery. All cases showed improved postoperative functional score at follow-up (Frankel, VAS, WHO and Karnofsky), including those cases who finally died. Conclusion: 1) Symptomatic spinal tumors should be taken into account in the general population patients with NF-1. 2) Benign tumors in this series were more aggressive than those arising in patients without NF. 3) Postoperative improvement of the functional status and the quality of life, supports the surgical approach among patients with NF-1 and symptomatic spinal tumors, despite the technical difficulties.
Palabras clave : Neurinomas; Neurofibroma; Neurofibromatosis; Spinal tumors.