Primitive neuroectodermal tumor presenting as a neck mass

Summary 

Primitive neuroectodermal tumors (PNET) comprise a family of the small blue round cell tumors. These neural crest origin tumors affect central nervous system (CNS) or peripheral tissues and are characterized by the set of unique chromosomal translocations and MIC-2 antigenic expression. PNET of the CNS are the most common malignant nervous system tumors of childhood. Reports of the peripheral PNET, which can present in the head and neck region are extremely limited. We present a case of the pediatric peripheral primitive neuroectodermal tumor manifested as an acute onset of neck swelling and ascending flaccid paralysis after routine first year immunizations.

Keywords: Primitive neuroectodermal tumor (PNET), CNS tumors, Small blue round cell tumors, Ewing's sarcoma, Ewing's sarcoma/PNET