Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity
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...
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Thieme Revinter Publicações Ltda.
2021-03-01
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doaj-4832da24be5f4c6ab2ea7c81307747032021-06-28T22:41:17ZengThieme Revinter Publicações Ltda.Brazilian Neurosurgery0103-53552359-59222021-03-01400108609010.1055/s-0040-1718426Gangliocytic Paraganglioma of the Filum Terminale. A Rare EntitySofia Isabel Carneiro Pereira Guerra Tavares0Gonçalo Maria Sengo Agante Guerreiro Costa1Department of Neurosurgery, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalDepartment of Neurosurgery, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalParagangliomas 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0040-1718426paragangliomagangliocyticfilum terminalelow back pain |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sofia Isabel Carneiro Pereira Guerra Tavares Gonçalo Maria Sengo Agante Guerreiro Costa |
spellingShingle |
Sofia Isabel Carneiro Pereira Guerra Tavares Gonçalo Maria Sengo Agante Guerreiro Costa Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity Brazilian Neurosurgery paraganglioma gangliocytic filum terminale low back pain |
author_facet |
Sofia Isabel Carneiro Pereira Guerra Tavares Gonçalo Maria Sengo Agante Guerreiro Costa |
author_sort |
Sofia Isabel Carneiro Pereira Guerra Tavares |
title |
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity |
title_short |
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity |
title_full |
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity |
title_fullStr |
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity |
title_full_unstemmed |
Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity |
title_sort |
gangliocytic paraganglioma of the filum terminale. a rare entity |
publisher |
Thieme Revinter Publicações Ltda. |
series |
Brazilian Neurosurgery |
issn |
0103-5355 2359-5922 |
publishDate |
2021-03-01 |
description |
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. |
topic |
paraganglioma gangliocytic filum terminale low back pain |
url |
http://www.thieme-connect.de/DOI/DOI?10.1055/s-0040-1718426 |
work_keys_str_mv |
AT sofiaisabelcarneiropereiraguerratavares gangliocyticparagangliomaofthefilumterminalearareentity AT goncalomariasengoaganteguerreirocosta gangliocyticparagangliomaofthefilumterminalearareentity |
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1721355749586632704 |