Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child
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, flacc...
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| Format: | Article |
| Language: | English |
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Thieme Revinter Publicações Ltda.
2022-06-01
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| Series: | Brazilian Neurosurgery |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742710 |
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| author | Aldo José Ferreira da Silva Ana Carolina de Carvalho Ruela Pires Auxiliadora Damianne Pereira Vieira da Costa Fabiana Lopes Amaral Rodrigo C. Bomfim Ângelo M. S. Bomfim Filho Alessandra Lamenha Feitosa Igor Lima Buarque |
| author_facet | Aldo José Ferreira da Silva Ana Carolina de Carvalho Ruela Pires Auxiliadora Damianne Pereira Vieira da Costa Fabiana Lopes Amaral Rodrigo C. Bomfim Ângelo M. S. Bomfim Filho Alessandra Lamenha Feitosa Igor Lima Buarque |
| author_sort | Aldo José Ferreira da Silva |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T21:34:39Z |
| format | Article |
| id | doaj.art-d090de2835cb4b8f9646ca40f82f1bec |
| institution | Directory Open Access Journal |
| issn | 0103-5355 2359-5922 |
| language | English |
| last_indexed | 2024-04-13T21:34:39Z |
| publishDate | 2022-06-01 |
| publisher | Thieme Revinter Publicações Ltda. |
| record_format | Article |
| series | Brazilian Neurosurgery |
| spelling | doaj.art-d090de2835cb4b8f9646ca40f82f1bec2022-12-22T02:29:02ZengThieme Revinter Publicações Ltda.Brazilian Neurosurgery0103-53552359-59222022-06-014102e183e18610.1055/s-0042-1742710Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a ChildAldo José Ferreira da Silva0Ana Carolina de Carvalho Ruela Pires1Auxiliadora Damianne Pereira Vieira da Costa2Fabiana Lopes Amaral3Rodrigo C. Bomfim4Ângelo M. S. Bomfim Filho5Alessandra Lamenha Feitosa6Igor Lima Buarque7Division of Pediatric Neurosurgery, Hospital Geral do Estado, Maceió, BrazilDivision of Pediatric, Hospital Geral do Estado, Maceió, BrazilDivision of Pediatric, Hospital Geral do Estado, Maceió, BrazilDivision of Pediatric, Hospital Geral do Estado, Maceió, BrazilIntervention and Diagnostic Imaging Service (Angioneuro), Hospital Veredas, Maceió, BrazilIntervention and Diagnostic Imaging Service (Angioneuro), Hospital Veredas, Maceió, BrazilDepartment of Pediatric Oncology, Santa Casa de Misericórdia de Maceió, Maceió, BrazilCESMAC University Center, Maceió, BrazilIntroduction 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742710ewing sarcomathoracolumbarepiduralradiotherapychemotherapy |
| spellingShingle | Aldo José Ferreira da Silva Ana Carolina de Carvalho Ruela Pires Auxiliadora Damianne Pereira Vieira da Costa Fabiana Lopes Amaral Rodrigo C. Bomfim Ângelo M. S. Bomfim Filho Alessandra Lamenha Feitosa Igor Lima Buarque Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child Brazilian Neurosurgery ewing sarcoma thoracolumbar epidural radiotherapy chemotherapy |
| title | Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child |
| title_full | Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child |
| title_fullStr | Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child |
| title_full_unstemmed | Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child |
| title_short | Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child |
| title_sort | primary extraskeletal ewing sarcoma of the thoracolumbar epidural space rare case report in a child |
| topic | ewing sarcoma thoracolumbar epidural radiotherapy chemotherapy |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0042-1742710 |
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