Definition A rare tumor differentiating toward specialized smooth muscle cells of the glomerular afferent arteriole in the juxtaglomerular apparatus; associated with renin production and hypertension Clinical features Epidemiology Rare tumor with fewer than 100 cases published Affects adolescents and young…
Definition Angiomyolipoma (AML) composed predominantly, at least 80%, of epithelioid cells Clinical features Epidemiology Eight percent of AMLs in surgical cohorts have an epithelioid component (10% or more of the tumor). Mean age, 50 (range 30–80) years Both genders are…
Definition A mesenchymal neoplasm composed of variable proportions of abnormal blood vessels, cells with smooth muscle features and adipose tissue; believed to be derived from perivascular epithelioid cells (PEC); hence, also named PECOMA Clinical features Epidemiology Angiomyolipoma (AML) accounts for…
Definition Intracaliceal mass composed of osteoid trabeculae, osteoblast-like cells, and spindle cells Clinical features Epidemiology Extremely rare Exclusively in infancy (age, 6 days to 17 months) Male predominant Left side predominant You’re Reading a Preview Become a Clinical Tree membership…
Definition Highly malignant mesenchymal tumor of pediatric kidney, also termed bone-metastasizing renal tumor of childhood for its striking predilection to metastasize to bone Clinical features Epidemiology Accounts for 3% of pediatric renal tumors, with approximately 20 new cases diagnosed annually…
Definition Highly aggressive malignant tumor composed of cells with rhabdoid morphology in pediatric patients Clinical features Epidemiology Affects young children, with mean age at diagnosis 1 year; the majority diagnosed before 2 years of age; extremely uncommon beyond 3 years…
Definition Rare low-grade fibroblastic neoplasm of infancy arising in the renal sinus Clinical features Epidemiology Two to four percent of pediatric renal tumors Ninety percent occur in the first year of life. Nearly all reported cases within less than 30…
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…
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…
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…