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Nephrogenic rests and nephroblastomatosis

Definition Nephrogenic rests are abnormally persistent embryonal cells beyond 36 weeks of gestation and are associated with and capable of developing into nephroblastomas (Wilms tumor). Nephroblastomatosis is defined as the presence of diffuse and multifocal nephrogenic rests. Clinical features Epidemiology Encountered in 40% of patients with unilateral nephroblastoma and nearly 100% of patients with bilateral nephroblastomas Observed in 1% of infant autopsies You’re Reading a Preview…

Wilms tumor

Definition A renal tumor derived from nephrogenic blastemal cells that recapitulate the histology of the developing kidney at various stages with divergent patterns of differentiation; also known as nephroblastoma Clinical features Epidemiology Affects 1/8000 to 1/10,000 children, with no sex predilection The most common renal tumor in children The most common genitourinary cancer in children Accounts for 8% of all pediatric cancers Ninety-eight percent of patients…

Metanephric stromal tumor

Definition Rare benign pediatric spindle cell renal tumor that is histologically identical to the stromal component of metanephric adenofibroma Clinical features Epidemiology Rare; fewer than 50 cases reported Mean age, 24 months (range, neonates to 15 years old) You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Metanephric adenoma

Definition A benign tumor recapitulating differentiation toward early embryonic metanephric tubules; also known as embryonal adenoma or nephrogenic nephroma Clinical features Epidemiology Female predominance Wide age range, from infants to elder individuals You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Renal cell neoplasms in Birt-Hogg-Dubé (BHD) syndrome

Definition An autosomal dominant syndrome characterized by a constellation of benign skin follicle tumors, multiple renal cell neoplasms, and spontaneous pneumothorax Clinical features Epidemiology Rare autosomal dominant disease with an incidence of 1/36,000 to 1/45,000 Life risk for renal cell carcinomas (RCCs) 14%–35% You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member.…

Renal cell carcinoma in von Hippel-Lindau disease

Definition An autosomal dominant disease caused by germline mutations in the von Hippel-Lindau ( VHL ) tumor suppressor gene on 3p25.3 and characterized by a constellation of tumors including renal cysts and clear cell renal cell carcinoma Clinical features Epidemiology Autosomal dominant condition with an incidence of 1:36,000 to 1:45,000 Renal cell carcinomas (RCCs) develop in ~60% of patients. Mean age of RCC onset is 39…

Renal cell carcinoma, unclassified type

Definition A group of renal cell carcinomas (RCCs) whose morphology or growth pattern does not readily fit any of the RCC subtypes recognized by the current World Health Organization classification Clinical features Epidemiology Represents 3%–5% of all RCCs The incidence will decrease with the detection of genetic changes characteristic of RCC subtypes using next-generation sequencing. You’re Reading a Preview Become a Clinical Tree membership for Full…

Renal cell carcinoma with somatic mutations in TSC/mTORC1 genes

Definition A group of recently described renal cell carcinomas with morphology similar to tumors in patients with tuberous sclerosis complex (TSC) and harboring somatic mutations in the genes of the TSC and mTORC1 pathways. They may have diverse morphological patterns. Clinical features Epidemiology Incidence currently unknown but rare; many were diagnosed as “unclassified RCC” or “unclassified renal oncocytic tumors.” You’re Reading a Preview Become a Clinical…

Eosinophilic solid cystic renal cell carcinoma

Definition A recently described variant of renal cell carcinoma (RCC) defined morphologically by solid and cystic areas composed of tumor cells with voluminous eosinophilic cytoplasm and basophilic coarse cytoplasmic granules and positive cytokeratin 20 staining Clinical features Epidemiology Incidence currently unknown but rare; many were diagnosed as “unclassified RCC” or “unclassified renal oncocytic tumors” ~10% occur in patients with tuberous sclerosis complex (TSC); majority sporadic Mean…

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 Rare You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in…

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 years), with no sex preference Most cases are sporadic and some are associated with end-stage renal disease and von Hippel-Lindau syndrome. You’re Reading a Preview Become…

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, with 39 cases reported Female-to-male ratio is nearly 2:1 Ages range from 19 to 83 years (median, 35 years) You’re Reading a Preview Become a Clinical…

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 overall male-to-female ratio of 7:1 Rarely in patients with hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), especially when the tumor has “poorly differentiated” foci You’re…

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 Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

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 of cases occur in the setting of germline mutations in one of the succinate dehydrogenase (SDH) genes, most commonly SDHB You’re Reading a Preview Become a…