Abstract

FERNANDEZ, Natalia; CASURIAGA, Ana Laura  and  GIACHETTO, Gustavo. Cervical dorsal neurofibroma in a girl with Neurofibromatosis Type 1 Neurofibroma. Anfamed [online]. 2018, vol.5, n.1, pp.118-130.  Epub June 01, 2018. ISSN 2301-1254.  https://doi.org/10.25184/anfamed2018v5n1a3.

Neurofibromatosis is one of the most frequent neurocutaneous syndrome. It is an inherited diseasewhich affects multiple systems. Three clinical presentations of the disease can be distinguished being type 1 neurofibromatosis the most common. Tumors at any body location can be associated to the usually presented cutaneous lesions. Neurofibromas are characteristic of type 1 neurofibromatosis. The aim of this study is to describe a less frequent complication of the disease which represents a therapeutic challenge due to its topography and frequency of recurrences. This is the case of a 3 year-old girl with type 1 neurofibromatosis with symptoms of multisegmental myelopathy. A cervical-dorsal tumor with medullary compression was shown in medical imaging. Tumor extraction allowed to confirm intradural, extramedullary neurofibroma. Plexiform neurofibromas are characterized by their infiltrative behavior and extensive paraspinal dissemination. Resection of these lesions usually generates morbidity associated with the involvement of extra-spinal critical structures and hinders their complete resection as occurred in the presented clinical case. It is important to perform a correct clinical and eventually imaging follow-up of patients with NF-1 to detect and treat tumors, its most important complication.

Keywords : Type 1 Neurofibromatosis; Cervical- dorsal Neurofibroma; Pediatrics..

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