Case report on primary spinal extradural peripheral neuro-ectodermal tumor: Ewing sarcoma; rare but not unheard of

<p>Alvernia Neysa Binti Ujat, Prabu Rau Sriram and Angeline Madatang</p>

Case report on primary spinal extradural peripheral neuro-ectodermal tumor: Ewing sarcoma; rare but not unheard of

Alvernia Neysa Binti Ujat, Prabu Rau Sriram and Angeline Madatang

Queen Elizabeth Hospital, Malaysia

: J Spine Neurosurg

Abstract

Ewing Sarcoma (ES) and Primitive Neuro-Ectodermal Tumor (PNET) were initially described as different clinic-pathologic entity, however over recent decades, these two entities are considered as entities that are of same spectrum of neoplastic disease known as Ewing Sarcoma Family of Tumors (EFT). This EFT ranges from classic ES, to atypical ES and PNET. PNET can be further divided into central and peripheral types. PNET in overall are an aggressive childhood malignancy. Spinal PNET are of peripheral type and it is rare compared to its central type counterpart. We report a case of intra-spinal exradural PNET, a rare case in children. A 7 years old girl first presented with one month history of left lower limb ascending weakness and pain radiating from hip to the toes. Two weeks later patient started to develop pain and weakness over right lower limb and constipation and subsequently develop overflow incontinence. Urgent MRI spine was done and an extradural non enhancing lesion at the level of L3/L4 extending to L4/L5 seen causing severe spinal canal stenosis. Laminectomy and excision of tumor was done with no worsening of neurology. Post-surgery patient was started on chemotherapy. Histologically, ES and PNET shared a lot of common features such as neural differentiations, expressing neural antigens and even markers suggesting of common neural origin. Further molecular and genetic differentiation between these two entities might enlighten on its differences. Based on literature review, prognosis for spinal PNET is poor even with complete surgical resection and chemo-radiotherapy.

Biography

Alvernia Neysa Binti Ujat has her expertise in a renowned Neurosurgery Department in Kota Kinabalu, Sabah. She has been doing her training as Resident since two years. She is also actively taking part in researches, data collections and attending conferences to expose more on latest updates in neurosurgery.

E-mail: ardalia.a.neysa@gmail.com