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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2017; 36(04): 247-250
DOI: 10.1055/s-0037-1607358
Case Report | Relato de Caso
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Embryonic Tumor with Abundant Neuroepithelium and True Rosettes (ETANTR): Case Report and Literature Review

Tumor embrionário com abundante neuroepitélio e verdadeiras rosetas (ETANTR): relato de caso e revisão da literatura
Cleciton Braga Tavares
1   Department Neurosurgery, Hospital São Marcos, Teresina, Piauí
,
Francisca das Chagas Sheyla Almeida Gomes-Braga
2   Hospital Universitário, Universidade Federal do Piauí, Teresina, Piauí
,
Emerson Brandão Sousa
1   Department Neurosurgery, Hospital São Marcos, Teresina, Piauí
,
Camilla Aniele Areia Ferreira
3   Universidade Federal do Piauí, Teresina, Piauí
,
José Nazareno Pearce de Oliveira Brito
1   Department Neurosurgery, Hospital São Marcos, Teresina, Piauí
› Author Affiliations
Further Information

Publication History

17 July 2017

14 September 2017

Publication Date:
23 October 2017 (online)

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Abstract

The embryonal tumor with abundant neuropil and true rosettes (ETANTR) is an extremely rare variant of the primitive neuroectodermal tumor (PNET). About 80 cases have been reported since its first description in the literature, in 2000. The ETANTR occurs in very young patients, especially children under 6 years of age. It is found mainly in the cerebral cortex. Headache, focal neurological signs, seizures, increased head circumference and psychomotor developmental delay are the most frequent symptoms of ETANTR. Histologically, it displays the features of an ependymoblastoma and a neuroblastoma, showing areas of neuroepithelium fibrillar rosettes with ependymoblastic zones and interposed undifferentiated neuroepithelial cells. The ETANTR is distinguishable from other embryonal tumors due to the abundance of neuroepithelium. Genetic studies have demonstrated the presence of polysomy of chromosome 2 and chromosome 19q13.42 amplification. This is an extremely aggressive tumor with a mean survival ranging from 9 to 48 months. We present the first report in Brazil, published in indexed literature, of an ETANTR case involving a young child.

Resumo

O tumor embrionário com neurópilo abundante e rosetas verdadeiras (TENARV) é uma variante muito rara do tumor neuroectodérmico primitivo (TNEP), com cerca de 80 casos publicados desde a sua primeira descrição na literatura, em 2000. O TENARV ocorre em pacientes muito jovens, especialmente crianças abaixo de 6 anos de idade, atingindo principalmente o córtex cerebral. Cefaleia, sinais focais, crise convulsiva, aumento do perímetro cefálico e atraso do desenvolvimento psicomotor são os sintomas mais frequentes da TENARV. Histologicamente, este tipo de tumor apresenta as características combinadas de um ependimoblastoma e um neuroblastoma, demonstrando áreas de neuroepitélio fibrilar com rosetas ependimoblásticas de permeio e zonas de células neuroepiteliais indiferenciadas. O TENARV é distinguível de outros tumores embrionários pela abundância de neuroepitélio. Estudos genéticos demonstram a presença de polissomia do cromossomo 2 e amplificação do cromossomo 19q13.42. Trata-se de um tumor extremamente agressivo, com sobrevida média entre 9 e 48 meses. Apresentamos o primeiro relato brasileiro, publicado em literatura indexada, de um caso de TENARV acometendo uma criança jovem.

Keywords

primitive neuroectodermal tumors - ependymoblastoma - neuroblastoma

Palavras-chave

tumores neuroectodérmicos primitivos - ependimoblastoma - neuroblastoma
 

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