Abstract
Malignancies arising from the external auditory canal are rare, with just 0.2% of all head and neck cancers. Only 4% of the external ear cancers arise from the external auditory canal. The most common histological variant is Squamous cell carcinoma, while neuroendocrine tumours are quite rare, with just 8 cases previously reported in EAC and few others in pinna. To the best of our knowledge, ours is the first reported case of poorly differentiated NET of EAC, which is involving pinna. The patient was a 56-year-old lady who presented with complains of right ear mass, blood-stained purulent discharge, decreased hearing and pain. Biopsy showed WHO grade III Neuroendocrine tumour. She was operated (lateral temporal bone resection with superficial parotidectomy and neck dissection with cervicofacial flap reconstruction). However, due to surgical site infection and flap failure, wound was again reconstructed with supraclavicular flap. She was planned for radiotherapy, but never turned for followup. The case represents the challenges in diagnosis, management and follow-up of such a rare entity.