Uropathology

Renal cell carcinoma with fibromyomatous stroma

Definition A group of renal cell carcinoma (RCC) with prominent smooth muscle–rich stroma that invests tumor cells, probably a heterogeneous group of RCCs with similar morphology. Considered an emerging/provisional entity in the World Health Organization (WHO) classification Clinical features Epidemiology…

Clear cell papillary renal cell carcinoma

Definition An indolent variant of renal cell neoplasm consists of tubular and papillary structures lined by bland clear cells with a distinct immunophenotype Clinical features Epidemiology Accounts for 1%–4% of all renal tumors Mean patient age, 60 years (range, 18–88…

Thyroid-like follicular renal cell carcinoma

Definition A recently described, rare variant of renal cell carcinoma (RCC) with a morphology that closely mimics well-differentiated thyroid follicular neoplasms Also termed thyroid-like follicular carcinoma of the kidney or thyroid follicular carcinoma-like renal cell carcinoma Clinical features Epidemiology Rare,…

Tubulocystic carcinoma

Definition A renal carcinoma with predominantly tubulocystic structures Clinical features Epidemiology A rare variant of kidney epithelial neoplasm, <1% of renal cell carcinomas Patients range in age from 30 to 80 years (mean age, 57.2 years) Male predominance with an…

Mucinous tubular and spindle cell carcinoma

Definition Low-grade renal epithelial neoplasm composed of tubules, spindle cells, and extracellular mucin Clinical features Epidemiology Usually occurs in adults, with a wide age range of 17–82 years (mean, 53 years) Occurs predominantly in female patients (male-to-female ratio, 1:4) You’re…

Succinate dehydrogenase-deficient renal cell carcinoma

Definition A variant of renal cell carcinoma (RCC) defined by the loss of protein expression of succinate dehydrogenase B on immunohistochemistry Clinical features Epidemiology Rare, <0.2% of all RCCs Mean age, 38 years (range, 14–76) Male to female ratio, 1.8:1 Majority…

Mit family translocation renal cell carcinoma

Definition Subtype of renal cell carcinoma harboring gene fusions involving two members of MiT family transcription factors, TFE3 (with Xp11 translocation), and TFEB (with t(6;11) translocation) Clinical features Epidemiology The majority of cases are reported in children and young adults.…

Renal medullary carcinoma

Definition Highly aggressive type of renal cell carcinoma centered in the medulla, associated with the sickle cell trait and hemoglobinopathies Clinical features Epidemiology Affects young adults in their second to third decades of life, with male-to-female ratio of 2:1 Most…

Collecting duct carcinoma

Definition A highly aggressive type of renal carcinoma originating from collecting duct epithelium; also known as carcinoma of the collecting ducts of Bellini Clinical features Epidemiology Less than 1% of renal cancers Occurs mostly in adults; rare cases reported in…