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DOI: 10.1055/s-0042-1742710
Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child
Sarcoma de Ewing extraesquelético primário no espaço epidural toracolombar: Raro relato de caso em criança
Abstract
Introduction Ewing sarcomas are a family of tumors that can be of skeletal or extraskeletal origin. We report a rare case of a child with extraskeletal Ewing sarcoma in the thoracolumbar epidural space.
Case Report The patient was a 1-year-old female child with sphincter alteration, flaccid paraplegia, and areflexia. A magnetic resonance imaging (MRI) scan showed a large extensive epidural lesion with compression of the dural sac in the D6–L2 segment, and a left paravertebral extension through the L1–L2 foramen. Laminotomy was performed, with subtotal resection of the lesion. The histopathological and immunohistochemical analyses indicated Ewing sarcoma. Due to the child's age, radiotherapy was not performed, only chemotherapy, due to the aggressiveness of the neoplasm. The patient showed rapid tumor recurrence and ended up dying.
Discussion Extraskeletal Ewing sarcoma can appear in different locations in the body. They are aggressive tumors with local recurrence and distant metastases. In our case, a combination of MRI and positron-emission tomography–computed tomography scan presented a clearer result, especially in the presence of metastasis. In the histopathological analysis, small blue cells with a clear cytoplasm and indistinct nucleoli were observed. In the immunohistochemical analysis, CD99 (MIC2) expression is highlighted. The best treatment outcome would have been surgical resection with chemotherapy and radiotherapy.
Conclusion We reported a rare case of thoracolumbar epidural Ewing sarcoma in which, despite surgery and chemotherapy, the tumor behaved very aggressively, leading to an unfavorable prognosis.
Resumo
Introdução Os sarcomas de Ewing constituem uma família de tumores que podem ser de origem óssea ou extraesquelética. O caso aqui relatado é o de uma criança com raro sarcoma de Ewing extraesquelético no espaço epidural toracolombar.
Relato de caso Criança do sexo feminino, de 1 ano de idade, com quadro de alteração de esfincter, paraplegia flácida, e arreflexia. O exame de ressonância magnética (RM) revelou volumosa lesão expansiva epidural com compressão do saco dural no segmento de D6-L2, e extensão paravertebral esquerda através do forame L1-L2. Realizou-se laminotomia, com ressecção subtotal da lesão. O exame histopatológico e a imunohistoquímica indicavam sarcoma de Ewing. Por conta da idade da criança, ela não foi submetida a radioterapia, apenas a quimioterapia, e, devido à agressividade da neoplasia, a paciente evoluiu com rápida recidiva tumoral e terminou falecendo.
Discussão O sarcoma de Ewing extraesquelético pode aparecer em diferentes locais do corpo. São tumores agressivos com recorrência local e metástase à distância. Na investigação, a combinação de RM e tomografia por emissão de pósitrons–tomografia computadorizada apresenta melhor resultado, principalmente na presença de metástase. No exame histopatológico, observaram-se pequenas células azuis com citoplasma claro e nucléolos indistintos. Na imunohistoquímica, se destaca a expressão principalmente do CD99 (MIC2). A conduta com melhor resultado no tratamento seria ressecção cirúrgica, com quimioterapia e radioterapia.
Conclusão Relatamos um caso raro de uma criança com sarcoma de Ewing epidural na coluna, e apesar da realização da cirurgia e de quimioterapia, o tumor se comportou de modo extremamente agressivo, o que levou a um prognóstico desfavorável.
Publikationsverlauf
Eingereicht: 06. Februar 2021
Angenommen: 20. Dezember 2021
Artikel online veröffentlicht:
04. April 2022
© 2022. 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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