Adenoid-Cystic Carcinoma of Nasal Septum and Pterygo-Palatine Fossa

Introduction: Adenoid cystic carcinoma (ACC) is an uncommon malignant tumour arising from salivary glands. It is more common in minor salivary glands, palate being the commonest site. Local recurrence, distant metastasis and perineural invasion are the common clinico-pathological features of this tumour. ACC of nasal septum and pterygo-palatine fossa is extremely rare entity with a very few reported cases in the available literature. Case Report: We report a case of ACC of nasal septum and pterygo-palatine fossa with its surgical management through endoscopic approach and follow up in the background of available literature. The article is aimed to highlight the clinical features, diagnosis, endoscopic approach for surgical management and long term post operative follow up of this rare entity. Discussion: Adenoid cystic carcinoma (ACC) itself is rare in nasal cavity; where lateral nasal wall is the commonest site of occurrence. ACC arising in the pterygo-palatine fossa is further rare entity; only 3 or 4 cases have been reported till date. ACC arising in these two sites simultaneously is a unique occurrence.