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Article: Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child

Silva, Aldo José Ferreira da / Ruela Pires, Ana Carolina de Carvalho / Costa, Auxiliadora Damianne Pereira Vieira da / Amaral, Fabiana Lopes / Bomfim, Rodrigo C. / Bomfim Filho, Ângelo M. S. / Feitosa, Alessandra Lamenha / Buarque, Igor Lima

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

2022  Volume 41, Issue 02, Page(s) e183–e186

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 ... ...

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.
Keywords Ewing sarcoma ; thoracolumbar ; epidural ; radiotherapy ; chemotherapy ; sarcoma de Ewing ; toracolombar ; epidural ; radioterapia ; quimioterapia
Language English
Publishing date 2022-04-04
Publisher Thieme Revinter Publicações Ltda.
Publishing place Stuttgart ; New York
Document type Article
ZDB-ID 2824608-1
ISSN 2359-5922 ; 0103-5355 ; 2359-5922
ISSN (online) 2359-5922
ISSN 0103-5355 ; 2359-5922
DOI 10.1055/s-0042-1742710
Database Thieme publisher's database

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