Tubulocystic renal cell carcinoma

Last revised by Daniel J Bell on 8 Jul 2021

Tubulocystic renal cell carcinomas are a rare subtype of renal cell carcinoma (RCC) with predominantly cystic appearance.

Tubulocystic renal cell carcinoma was first identified as a distinct histopathologic entity in 2005, and subsequently acknowledged as an independent disease category in the 2016 WHO classification of renal tumors 1,3.

Peak incidence occurs in the 6th through 8th decades.

Males are much more commonly affected than females (7:1 ratio).

Most patients are asymptomatic and the tumor is discovered incidentally.

Macroscopically, the tumors are well-circumscribed lesions comprising extensive tiny cysts with numerous thin septa, resulting in a spongy or "bubble wrap" appearance. Microscopically, the tumor is composed of tubular and cystic spaces separated by fibrous stroma.

Limited information from genetic studies suggest gene expression similar to papillary RCC 4.

  • variable attenuation so may appear solid or cystic
  • C+: contrast enhancement is usually mild or indeterminate given small amount of solid tissue
  • T2: hyperintense due to cystic component, with septa
  • T1 C+ (Gd): mild enhancement in small solid components or wall

Surgical resection is curative.

Recurrence and/or metastasis occur in less than 10% of cases.

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