Wilms tumor

Wilms tumor, or nephroblastoma, is a malignant kidney tumor of childhood that develops from immature kidney cells. It is the most common childhood kidney cancer.

Nephrogenic rest: A focus of persistent embryonic kidney tissue that can precede Wilms tumor. These residual cell clusters are considered precursors for many Wilms tumors.
Wilms tumor staging: A system that classifies Wilms tumor by its extent and surgical findings. Stage determines treatment intensity and helps estimate prognosis.
Max Wilms: A German surgeon and pathologist who published an influential description of nephroblastoma in 1899. The tumor bears his name because of his early account of the disease.
Clear cell sarcoma of the kidney: A rare malignant childhood kidney tumor distinct from Wilms tumor. It can resemble Wilms tumor clinically but has different pathology and metastatic behavior.
WT1: A tumor-suppressor gene on chromosome 11 that helps regulate kidney and gonadal development. WT1 alterations can disrupt kidney development and contribute to Wilms tumor.
Nephrectomy: Surgical removal of all or part of a kidney. Removal of the affected kidney is central to treatment in many protocols.
Denys-Drash syndrome: A rare disorder involving WT1 variants, kidney disease, and increased Wilms tumor risk. It links a defined genetic condition to tumor predisposition.
Malignant rhabdoid tumor of the kidney: An aggressive kidney cancer of young children, often associated with SMARCB1 loss. Its age distribution and aggressive course distinguish it from typical Wilms tumor.
WNT signaling pathway: A cell-signaling network that regulates development, tissue renewal, and cell fate. WNT pathway changes, including CTNNB1 mutations, occur in a subset of tumors.
Wilms tumor histology: The microscopic classification of Wilms tumor, including favorable and anaplastic patterns. Anaplasia can signal resistance to chemotherapy and alter treatment.

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AniridiaRelated: Its risk is elevated in WAGR syndrome, making systemic assessment important when that diagnosis is suspected.