Primary Primitive Neuroectodermal Tumor (PNET) of the Spine With t(11;22): Report of 3 Cases and Review of Literature

Ashley Margol, Chenue Abongwa, Girish Dhall
Journal of Pediatric Hematology/oncology 2021 January 20
Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. The authors also compare the clinical course and outcome of therapy with primary PNET of the central nervous system and Ewing sarcoma family of tumors. In addition, this report highlights the risk for leptomeningeal dissemination at recurrence and discusses the importance of central nervous system-targeted therapy for durable disease control.

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