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DOI: 10.1055/s-0040-1718426
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity
Paraganglioma gangliocítico do filum terminale. Uma entidade rara
Abstract
Paragangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85–90%) or from the extra-adrenal paraganglia.
In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale.
We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level.
He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse.
These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.
Resumo
Os paragangliomas são tumores neuroendócrinos raros, benignos e de crescimento lento, que podem ter origem na medula adrenal (85–90%) ou nos paraganglia extra-adrenais.
Podem ser encontrados em diversas localizações no sistema nervoso central (SNC), mas mais frequentemente a nível da cauda equina ou no filum terminale, onde correspondem a entre 2,5 e 3,8% do número total de tumores dessa região. Existem apenas 8 casos descritos na literatura que descrevem a presença da variante gangliocítica desta entidade no filum terminale.
Apresentamos o caso de um homem com 41 anos de idade com dor lombar crônica refratária ao tratamento médico, sem qualquer déficit neurológico associado. A ressonância magnética (RM) demonstrou uma lesão oval intradural e extramedular, de contornos regulares, com captação homogénea de contraste localizada ao nível de L1.
O paciente foi submetido a intervenção cirúrgica, tendo sido feita uma resseção completa da lesão. A análise histológica revelou um paraganglioma gangliocítico do filum terminale. Aos 5 anos de seguimento, o paciente permanece assintomático e sem sinais de recidiva.
Estas são lesões com um bom prognóstico após uma remoção macroscopicamente total. Embora a taxa de recidiva seja muito baixa, a vigilância prolongada é recomendada devido ao crescimento lento destes tumores, sendo estimado que entre 1 e 4% podem recidivar mesmo após uma remoção total.
Publikationsverlauf
Eingereicht: 10. April 2020
Angenommen: 05. August 2020
Artikel online veröffentlicht:
16. Oktober 2020
© 2020. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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