Renal Cell-Like Adenocarcinoma of Nasal Cavity: A Rare Entity Deserving Elucidation

Sino-nasal malignancies are extremely rare accounting < 1% of all malignancies and < 3% of aero-digestive tract tumors. Their surgical pathology is extremely challenging due to the enormous heterogeneity. Few of these sino-nasal tumors may possibly present histologically as clear cell neoplasm. This mechanism is described as 'histological mimicry'. Renal cell-like adenocarcinoma (RCLA) is amongst the top contenders in that list of tumors including squamous cell carcinoma and mucoepidermoid carcinoma. RCLA affecting the nasal cavity is an extremely rare malignancy to have been reported in the literature. It was first described by Zuhr et al. in 2002 and till as recent as 2017 only 15 such cases had been reported worldwide. We present a rare case report of a 45 years old female who presented with unilateral nasal obstruction but was found harboring a histopathological surprise. We wish to highlight the diagnostic and therapeutic challenge faced in the management of this case and create awareness amongst the ORLs, pathologists and medical oncologists about this unaccustomed entity.

The online version contains supplementary material available at 10.1007/s12070-024-05275-6.