Primitive Neuroectodermal Tumour of the Larynx: A Rare Case Report

Ewing’s sarcoma/primitive neuroectodermal tumour is a malignant small round blue cell tumour of neuroectodermal origin that affects bones and soft tissue in children and young adults. These are aggressive malignant tumours, and long-term survival rates following diagnosis remain poor. Occurrence of primitive neuroectodermal tumours in the head and neck region is uncommon. It is extremely rare in the larynx with only 12 cases reported in the literature. We report a case of primary primitive neuroectodermal tumour of the larynx in a 28-year-old female. She presented with hoarseness of voice, and her computed tomography and laryngoscopy revealed a mass at the laryngeal inlet. A biopsy followed by histopathology and immunohistochemistry confirmed the diagnosis of a primitive neuroectodermal tumour. She initially defaulted from treatment and subsequently presented with stridor for which an emergency tracheostomy was done. Her metastatic workup did not reveal any lesion elsewhere. So, a diagnosis of a primary primitive neuroectodermal tumour of the larynx was made, and the patient was started on treatment.