Primary extraskeletal peripheral primitive neuroectodermal tumor of subcutaneous tissue neck in a young adult: A rare case report

Abstract

Primitive neuroectodermal tumors (PNETs) are poorly differentiated small round cell neoplasms which primarily affect childhood age and very rarely seen in adults. Peripheral PNET (pPNET) cases are very rare compared to central PNET, and most of them originate from neural crest cells located outside the central nervous system. We report a case of large extraosseous pPNET arising from subcutaneous tissue of left side neck in a young male patient. Despite aggressive inherent nature of histology, structural complexity of neck area and poor predictors like tumor size >6 cm, extraosseous nature, this case advocate that complete resolution is possible with aggressive multimodal treatment including surgery, radiation therapy, and chemotherapy. Other major concerns in such cases of pPNET are difficulty in diagnosis due to low incidence, unpredictable site involvement, histological similarity with other round cell tumors and lack of established treatment guidelines.