A CASE OF PRIMITIVE NEUROECTODERMAL TUMOR (PNET) PRESENTING AS ANTERIOR MEDIASTINAL MASS

Abstract

Peripheral primitive neuroectodermal tumors (pPNET) are a group of highly malignant tumors composed of small round cells of neuroetodermal origin that affect soft tissue and bone ,most commonly present in the thoracopulmonary region (Askin tumor), abdomen , pelvis and rarely in the head and neck. They usually present in the second decade of life, with slight male preponderance and account for 4-17% of all soft tissue tumors. Clinical symptoms depend on the site of presentation but invariably include pain and swelling of surrounding structure due to mass effect. Current recommendations advocate complete surgical resection whenever possible, adjuvant or neoadjuvant chemotherapy and radiotherapy. Here we present a 20 year old male patient who presented with chest pain and breathlessness and on investigation found to have an anterior mediastinal mass which further evaluation found to be a primitive neuroectodermal tumor