PNET/ES Tumor with Paraneoplastic Features: A Case Report

Peripheral primitive neuroectodermal tumors (PNET) are classified as Ewing sarcoma family of tumors (ESFT) and are most common in young adults and children. PNETs originated from chest wall are previously named Askins’ tumors. Histopathologic exam reveals round blue cells with high N/C ratio and high mitotic rate. Immunohistochemistry demonstrates CD99, NKX2.2 and S100 on tumor cells’ surface. Chromosomal translocation t (11; 22) (q24; q12) is usually considered for definite diagnosis.

Herein, a forty-five-year-old patient with thoracic peripheral PNET is reported with extended local invasion. Moreover, the patient developed non-islet cell tumor hypoglycemia (NICTH) and paraneoplastic proteinuria which was successfully controlled by corticosteroids administration