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About Wilms Disease
Wilms disease (Also known as nephroblastoma) is the most common pediatric renal cancer and the 4th most common pediatric cancer. Fundamentally, Wilms disease is cancer of the kidney which can lead to abdominal pain, gross hetauria, urinary tract infections, fever, and hyper-tension/hypo-tension. Wilms disease has no established cause, but has been linked to mutations in genes WT1, CTNB1, and WTX. Other diseases associated with Wilms disease including WAGR, Drash Syndrome, and Beckwith-Wiedmann syndrome. [1]
Wilms Disease has varied prognosis/histology; Favorable histology has a triphasic pattern of blastema, epithelial, and stroma tissue. Tissues that exhibit anaplasia, the presence of marked nuclear enlargement, are typically indicative of unfavorable histology. |
What is WT1?
WT1 is a gene responsible for development of kidneys and gonads before birth. Found on the 11th chromosome, it produces a protein (also known as WT1) that plays a role a cell differentiation and apoptosis as a transcription factor- binding to other sections of DNA and regulating their cell expression. While the link between WT1 and Wilms disease hasn't been fully explored, it has been established that mutations in the zinc-finger domain (DNA binding domain) lead to LoF mutations that may lead to Wilms disease. [2] While previous research characterized WT1 as a tumor suppressor gene, newer research has shown increased expression trends in other neoplasms indicative of an oncogene. [3]
Stages of Wilms Disease
Wilms Tumor can be broken up into different stages based on treatment outcomes/current growth. Stage 1 involves a removable cancer massive in only one kidney and the cancer has not grown out of the kidney. Stage 2 involves the tumor spreading to other tissues, however it can still be removed by surgery. Stage 3 involves the tumor spreading to lymph nodes inside the abdomen or pelvis and can no longer be removed in it's entirely as the cancer has spread to the lining of the abdominal wall. Stage 4, the cancer has spread toward organs such as lungs, liver, brain, or bones.
Learn More about Wilms Tumor
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Wilms Disease crash course [4]
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Cluster Map
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References:
1. Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. [Updated 2023 May 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK442004/
2. Al-Hussain, Turki MD*; Ali, Afshan MD†; Akhtar, Mohammed MD, FCAP, FRCPA, FRCPath*. Wilms Tumor: An Update. Advances In Anatomic Pathology 21(3):p 166-173, May 2014. | DOI: 10.1097/PAP.0000000000000017
3. Ariyaratana S, Loeb DM. The role of the Wilms tumour gene (WT1) in normal and malignant haematopoiesis. Expert Reviews in Molecular Medicine. 2007;9(14):1-17. doi:10.1017/S1462399407000336
4. Medical Central. (2022, October 25). Wilms' Tumor: Everything You Need To Know [Video]. YouTube. https://www.youtube.com/watch?v=udQZjNfJgUw
1. Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. [Updated 2023 May 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK442004/
2. Al-Hussain, Turki MD*; Ali, Afshan MD†; Akhtar, Mohammed MD, FCAP, FRCPA, FRCPath*. Wilms Tumor: An Update. Advances In Anatomic Pathology 21(3):p 166-173, May 2014. | DOI: 10.1097/PAP.0000000000000017
3. Ariyaratana S, Loeb DM. The role of the Wilms tumour gene (WT1) in normal and malignant haematopoiesis. Expert Reviews in Molecular Medicine. 2007;9(14):1-17. doi:10.1017/S1462399407000336
4. Medical Central. (2022, October 25). Wilms' Tumor: Everything You Need To Know [Video]. YouTube. https://www.youtube.com/watch?v=udQZjNfJgUw