A dormant yet belligerent tumour, the sinonasal adenoid cystic carcinoma- A case report

Abstract

Adenoid cystic carcinoma, also known as cribriform tumour or Cylindroma owing to the cellular arrangement or type of tissue that it originates from, is rare and shows perineural invasiveness as a pathognomic feature making treatment challenging. The tumour is known to arise in the salivary glands and seldom seen in the nasal cavity or paranasal sinuses. We here present a rare case of ACC occurring beyond its usual premise.A male patient aged 32 presented with unilateral nasal obstruction and epistaxis and on examination a growth in the right nasal cavity was noted to peculiarly arise from beneath the mucosa of the nasal floor, extending posteriorly up to the choana, not involving the turbinates or septum. DNE with biopsy was done and CECT PNS was done for further evaluation and the mass was diagnosed to be stage III sinonasal adenoid cystic carcinoma.This tumour originates from minor salivary glands and its presentation in the nose and paranasal sinuses has been reported to be very sparse. This is perhaps a reason for it to be missed out in daily practice due to clinical features being similar to several inflammatory and neoplastic diseases of the nose and paranasal sinuses. Histopathological cribriform and tubular subtypes are less aggressive than solid form and that determine the prognosis. It is a disease of 6th and 7th decade, slow growing and locally invasive very unusual to find it in a young patient such as ours.Though uncommon the ACC mustn’t go undiagnosed and must be differentiated from commoner nasal masses. Otorhinolaryngologists need to work in collaboration with pathologists and oncologists to accurately diagnose and treat this belligerent tumour in a multifaceted approach. Complete surgical excision with post-operative radiotherapy is the most accepted treatment plan