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

Author:

Silva Aldo José Ferreira da12ORCID,Ruela Pires Ana Carolina de Carvalho3ORCID,Costa Auxiliadora Damianne Pereira Vieira da3ORCID,Amaral Fabiana Lopes3ORCID,Bomfim Rodrigo C.4,Bomfim Filho Ângelo M. S.4,Feitosa Alessandra Lamenha5,Buarque Igor Lima6ORCID

Affiliation:

1. Division of Pediatric Neurosurgery, Hospital Geral do Estado, Maceió, Brazil

2. Pediatric Neurosurgery Division, Santa Monica Teaching Maternity, Universidade Estadual de Ciências da Saúde de Alagoas, Maceió, Brazil

3. Division of Pediatric, Hospital Geral do Estado, Maceió, Brazil

4. Intervention and Diagnostic Imaging Service (Angioneuro), Hospital Veredas, Maceió, Brazil

5. Department of Pediatric Oncology, Santa Casa de Misericórdia de Maceió, Maceió, Brazil

6. CESMAC University Center, Maceió, Brazil

Abstract

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.

Publisher

Georg Thieme Verlag KG

Subject

Neurology (clinical),Surgery

Reference14 articles.

1. Extra osseous primary Ewing's sarcoma;S A Ali;J Ayub Med Coll Abbottabad,2010

2. Adult primary spinal epidural extraosseous ewing's sarcoma: a case report and review of the literature;M Bustoros;Case Rep Neurol Med,2016

3. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation;M D Murphey;Radiographics,2013

4. Ewing sarcoma of the adrenal gland: a case report and review of the literature;H Eddaoualline;J Med Case Reports,2018

5. Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases;S Jiang;Cancer Manag Res,2018

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