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Voluminous plexiform neurofibromas of the neck region in neurofibromatosis 1

I. Pascual-Castroviejo, S.I. Pascual-Pascual, R. Velázquez-Fragua, J. Viaño, D. Quiñones-Tapia, F. López-Barea [REV NEUROL 2014;59:13-19] PMID: 24965926 DOI: https://doi.org/10.33588/rn.5901.2014043

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Volumen 59 | Number 01 | Nº of views of the article 6.503 | Nº of PDF downloads 444 | Article publication date 01/07/2014

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ABSTRACT

AIM To present the clinic, imaging and evolutive characteristics of a series of patients with neurofibromatosis 1 with voluminous plexiform neurofibromas in the neck (VPNFN) during childhood.

PATIENTS AND METHODS Nine patients (five females and four males) who were diagnosed as VPNFN at ages between 3 and 15 years. The VPNFN widespread to the posterior fossa or the upper thoracic region in some cases. The diagnosis was based on the clinical, imaging and histological findings.

RESULTS One of the tumors was intralaryngeal and caused respiratory difficulties. The other eight patients had the origin of the tumor in several spinal roots of one or both sides and could growth to the posterior fossa and to the upper thoracic region in some cases with displacement of the surrounding organs, especially in three patients, all girls, in whom the tumor reached a voluminous size on one side, that was observed only until 10 to 11 years when the growth ceased.

CONCLUSIONS The VPNFN are histologically benign tumors. Those located in the larynx must be removed because of the respiratory problems, but it is not necessary in cases with other locations despite the voluminous size that can reach in some patients with great displacement of the surrounding organs. The analysis of the results of our series may demonstrate that al least the extralaryngeal tumors only grow to 11-12 years of age. This possibility may make recommendable to retard the surgical treatment as much as possible in cases that it is not necessary. KeywordsMediastinum plexiform neurofibroma Neurocutaneous disorders Neurofibromatosis 1 Pharynx plexiform neurofibroma Voluminous plexiform neurofibromas in the neck

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Original

Voluminous plexiform neurofibromas of the neck region in neurofibromatosis 1