Management of Primitive Neuro-ectodermal Tumor of the Vagina in a Sickle Cell Disease Patient

Primitive neuro-ectodermal tumor (PNET) is rare comprising 1% of soft tissue sarcomas. PNET is considered an entity of Ewing’s family of tumors. Ewing’s family of tumors is not a single condition but a group of closely related tumors. These tumors have a similar natural history, prognosis, immunohistochemical and cytogenetic profiles. These tumors are defined as round cell sarcoma that shows varying degree of neuro-ectodermal differentiation. Primitive neuro-ectodermal tumors can be classified into central and peripheral PNETs according to the cells of origin. Central PNETs originate from the neural tube, which includes the brain and spinal cord, while peripheral PNETs arise from the neural crest, which includes sympathetic nervous system, bones or soft tissues.