Primary spinal extradural extraosseous primitive neuroectodermal tumor/Ewing's sarcoma: A critical analysis and review

Abstract

Primary spinal extradural Ewing's sarcoma/primitive neuroectodermal tumor (PNET) is rare malignant tumor of childhood and early adulthood. The World Health Organization classifies PNET as an undifferentiated round cell tumor arising from primitive neuroepithelial cell. It can be central or peripheral PNET depending on site of presentation. Usually, the presenting symptoms are chronic back pain and myelopathy. Overall prognosis and survival are dismal in spite of total surgical resection and adjuvant therapy. Because of the rarity and malignant behavior, definite management of spinal PNET has never been described. After review of medical record at Acharaya Vinoba Bhave Rural Hospital, Sawangi, India, we identified four patients of spinal PNET and were included in our study. Age at diagnosis ranging from 15 to 26 years old with mean age of 20 years old. All four cases were epidural in location, two of which were of Askin type tumor with spinal cord compression. Rural population with low literacy and financial constraints were the key reasons of late presentations at our hospital. Counseling and proper education regarding the disease are a must for early case detection and early treatment of those living in rural areas and suffers from financial constraints. Due to rarity of the disease and its poor prognosis, a well-organized multicentric controlled trial is required to formulate a standard guidelines in the management of this disease.