Primary extrarenal papillary renal cell carcinoma presenting as a neck mass

Extrarenal primary renal cell carcinoma is an extremely rare entity with limited literature. Characteristic findings of renal cell carcinoma (RCC) at the metastatic site in the absence of a discrete radiological renal lesion can perplex both the clinician and pathologist. We report a case of metastatic primary extrarenal papillary RCC, who presented as a neck mass clinically and radiologically a paraaortic mass with normal bilateral native kidneys. The final diagnosis was aided by histopathological features, further confirmed by targeted immunohistochemical markers.