Intraparenchymal central nervous system Ewing’s sarcoma. A case treated according to the modified Euro-EWING protocol

Marie-Amelyne Le Rouzic, Julie Valduga, Ludovic Mansuy, Emmanuelle Schmitt, Olivier Klein, Valérie Bernier, Pascal Chastagner

Abstract


Ewing's sarcoma (ES) is the second most common malignant bone tumor in children and young adults. ES may also occur as a primary soft tissue neoplasm without involvement of the bone and is then referred to as extraosseous ES (EES). The paediatric central nervous system extraosseous Ewing's sarcoma (CNS-EES) is an extremely rare entity since less than 20 cases have been reported in literature. Moreover, only less than 10 cases correspond to brain parenchyma primary tumors. These cases have been often misdiagnosed with other varieties of primary brain tumors, particularly those which were called PNET (Primitive Neuro-Ectodermal Tumor). We present a case of true intraparenchymal CNS-EES and a comparison of epidemiology, behaviour, treatment, and prognosis of CNS-EES and intracranial PNET cases.

Keywords


extraosseous Ewing’s sarcoma; central nervous system; surgery; chemotherapy; radiotherapy; child

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References


Bernstein M, Kovar H, Paulussen M, et al. Ewing's sarcoma family of tumors: current management. Oncologist 2006; 11:503-519.

Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumors of the central nervous system. Acta Neuropathol 2007; 114:97-109.

Mazur MA, Gururangan S, Bridge JA, et al. Intracranial Ewing's sarcoma. Pediatr Blood Cancer 2005; 45:850-856.

VandenHeuvel KA, Al-Rohil RN, Stevenson ME, et al. Primary intracranial Ewing’s sarcoma with unusual features. Int J Clin Exp Pathol 2015; 8:260-274.

Pekala JS, Gururangan S, Provenzale JM, et al. Central nervous system extraosseous Ewing sarcoma: radiologic manifestations of this newly defined pathologic entity. Am J Neuroradiol 2006; 27:580-583.

Kazmi SA, Perry A, Pressey JG, et al. Primary Ewing sarcoma of the brain: a case report and literature review. Diagn Mol Pathol 2007; 16:108-111.

Jay V, Zielenska M, Lorenzana A, et al. An unusual cerebellar primitive neuroectodermal tumor with t(11;22) translocation: pathological and molecular analysis. Pediatr Pathol Lab Med 1999; 16:119-128.

Bunyaratavej K, Khaoroptham S, Phonprasert C, et al. Primary intracranial peripheral primitive neuroectodermal tumor/Ewing’s sarcoma presenting with acute intracerebral hemorrhage. Clin Neuropathol 2005; 24:184-190.

Choudhury KB, Sharma S, Kothari R, et al. Primary extraosseous intracranial Ewing’s sarcoma: Case report and literature review. Indian J Med Paediatr Oncol 2011; 32:118-121.

Antunes NL, Lellouch-Tubiana A, Kalifa C, et al. Intracranial Ewing sarcoma/‘peripheral’ primitive neuroectodermal tumor of dural origin with molecular genetic confirmation. J Neurooncol 2001; 51:51-56.

Papotti M, Abbona G, Pagani A. Primitive neuroectodermal tumor of the meninges: a histology, immunohistochemical, ultrastructural and cytogenetic study. Endocr Pathol 1998; 3:275-280.

Dedeurwaerdere F, Giannini C, Sciot R, et al. Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET. Mod Pathol 2002; 15:673-678.

Katayma Y, Kimura S, Watanabe T, et al. Peripheral-type primitive neuroectodermal tumor arising in the tentorium: case report. J Neurosurg 1999; 90:141-144.

Taylor RE, Bailey CC, Robinson KJ, et al. Outcome for patients with metastatic (M2-3) medulloblastoma treated with SIOP/UKCCSG PNET-3 chemotherapy. Eur J Cancer 2005; 41:727-734.

Edelstein K, Spiegler BJ, Fung S, et al. Early aging in adult survivors of childhood medulloblastoma: long-term neurocognitive, functional, and physical outcomes. Neuro Oncol 2011; 13:536-545.

Laughton SJ, Merchant TE, Sklar CA, et al. Endocrine outcomes for children with embryonal brain tumors after risk-adapted craniospinal and conformal primary-site irradiation and highdose chemotherapy with stem-cell rescue on the SJMB-96 trial. J Clin Oncol 2008; 26:1112-1118.




DOI: http://dx.doi.org/10.22551/2017.17.0404.10116

Copyright (c) 2017 Marie-Amelyne Le Rouzic, Julie Valduga, Ludovic Mansuy, Emmanuelle Schmitt, Olivier Klein, Valérie Bernier, Pascal Chastagner

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ISSN: 2360-6975