A Peripheral Primitive Neuro-ectodermal Tumor (pPNET) of Larynx

Abstract

Introduction

Primitive neuroectodermal tumors (PNETs) are malignant tumors comprised of small round cells of neuro-ectodermal origin that affect soft tissue and bone. Though the occurrence of pPNETs in the head and neck region is rare, these are aggressive malignant tumors, and long-term survival rates following diagnosis remain poor.

Case Report

In the present case, a tumour was located in larynx (as globular/cystic mass of epiglottis) of the patient and was diagnosed as pPNET. Immunohistochemical analysis indicated that tumor cells were positive for CD99 and NSE, focally positive for EMA but negative for synaptophysin and chromogranin. The mass was surgically excised with negative margins. In post op period patient was planned for post-op chemotherapy and radiotherapy.

Conclusion

pPNETs are very rare in head and neck region. Significant advances in the neoadjuvant and adjuvant chemotherapeutic regimens, as well as improved facility in diagnosing these tumors through cytogenetic and immunohistochemical analysis improves the long-term disease-free survival.