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EPEN-13. PRIMARY EXTRADURAL SACROCOCCYGEAL SUBCUTANEOUS MYXOPAPILAR EPENDYMOMA MISDIAGNOSED AS PILONIDAL CYST IN A 7 YEAR-OLD BOY: A CASE REPORT

BACKGROUND: Ependymomas occur in the brain or spinal cord and rarely as an extraspinal variety at the sacrococcygeal region, separated from the spinal cord. This rare presentation is thought to originate from a group of heterotopic ependymal cells called the coccygeal medullary vestige. There are fe...

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Detalles Bibliográficos
Autores principales: del Campo, Regina M Navarro-Martin, Tavares-Macias, Geronimo M, Pozos-Ochoa, Luis Ivan, Orozco-Alvarado, Ana L, Sanchez-Zubieta, Fernando, Arredondo-Navarro, Luis Angel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7715149/
http://dx.doi.org/10.1093/neuonc/noaa222.152
Descripción
Sumario:BACKGROUND: Ependymomas occur in the brain or spinal cord and rarely as an extraspinal variety at the sacrococcygeal region, separated from the spinal cord. This rare presentation is thought to originate from a group of heterotopic ependymal cells called the coccygeal medullary vestige. There are few reports of this occurrence in children. CLINICAL CASE: A 7-year-old male presented with a history of a soft mass arising in the sacrococcygeal area 3 years earlier, diagnosed as pilonidal cyst at primary level and treated with surgery twice, as this mass recurred the boy was sent to our hospital, a 3(rd) surgery was performed, all tumoral tissue was removed, no attachment with dural space was founded, pathology revealed myxopapilar ependymoma with positivity for PS100, EMA and Vimentin. After surgery a Follow up MRI of cranium and spine showed absence of disease, no radiotherapy neither chemotherapy was implemented. He has been on surveillance from 3 years now without recurrence. CONCLUSION: This report highlights the fact that pediatric ependymoma can have an extradural presentation and can be confounded with pilonidal cyst, total resection is needed to control the disease. Potential for recurrence or metastatic disease can continue 20 years from the time of primary tumor, so prolonged surveillance is important.