Sinonasal Renal Cell Like Adenocarcinoma; A Report of Rare Entity with Emphasis on its Association with Von Hippel Lindau Syndrome

Abstract

Abstract Background Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare entity that was introduced to head and neck tumors classification on 2017 as an emerging entity. Clinically, it could be asymptomatic or show non-specific symptoms like epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires utilization of clinical, radiological, histopathological and ancillary tests for accurate characterization of the lesion. There is around 20 cases reported in the literature with 3 cases associated with VHL syndrome. Case Presentation A 41 years old female with a nasal mass for two years ago that presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and Inhibin while were negative for CK20, P63, CK 5/6, CD10, RCC, TTF1, PAX8, CEA, and GATA3. Proliferation index (KI67) was less than 5% (Figure.3). The diagnosis was consistent with sinonasal renal cell-like adenocarcinoma. The patient has no recurrence and no symptoms after 1 year. Conclusion: Our study report a rare case of SNRCLA with discussion of the histological features and its association with VHL syndrome.