Queen's University Student Editing Initiative

edit

Hello, we are a group of medical students from Queen's University. We are working to improve this article over the next month and will be posting or planned changes on this talk page. We look forward to working with the existing Wikipedia medical editing community to improve this article and share evidence. We welcome feedback and suggestions as we learn to edit. Thank you. Patguy202 (talk) 18:11, 1 October 2018 (UTC)

We have compiled a list of suggestions to improve this article and would appreciate community feedback before we proceed with these edits. Here is a list of our suggestions:

1. We propose to add the following citations to the Introduction sections: Approximately 650 cases are diagnosed in the U.S. annually.[1] The majority of cases occur in otherwise normal children; however, a minority of children with Wilms tumor have an associated congenital syndrome.[1]

2. We propose to add the following introductory content to the Pathogenesis section: Wilms Tumor has many causes, which can broadly be categorized as syndromic and non-syndromic.[2] Syndromic causes of Wilms Tumor occur as a result of alterations to genes such as the Wilms Tumor 1 (WT1) or Wilms Tumor 2 (WT2) genes, and the tumor presents with a constellation of other signs and symptoms. Non-syndromic Wilms Tumor is not associated with other symptoms or pathologies.[2]

3. We propose to add the following content to the Pathogenesis section pertaining to the role of nephrogenic rests in the development of the tumor: Many, but not all, cases of Wilms Tumor develop from nephrogenic rests, which are fragments of tissue in or around the kidney that develop before birth and become cancerous after birth.[2] In particular, cases of bilateral Wilms Tumor, as well as cases of Wilms Tumor derived from certain genetic syndromes such as Denys-Drash syndrome, are strongly associated with nephrogenic rests.[2]

4. We propose to add the following content to the Diagnosis section: The majority of patients with Wilms’ tumor present with an asymptotic abdominal mass which is noticed by a family member or healthcare professional.[2] Renal tumors can also be found during routine screening in children who have known predisposing clinical syndromes.[2] The diagnostic process includes taking a medical history, a physical exam, and a series of tests including blood, urine, and imaging tests.[3]

Once Wilms’ tumor is suspected, an ultrasound scan is usually done first to confirm the presence of an intrarenal mass.[3] A computed tomography scan or MRI scan can also be used for more detailed imaging.[4] Finally, the diagnosis of Wilms’ tumor is confirmed by a tissue sample. In most cases, a biopsy is not done first because there is a risk of cancer cells spreading during the procedure.[4] Hence, nephrectomy (in North America) or chemotherapy followed by nephrectomy (in Europe) is performed instead.[4] A definitive diagnosis is obtained after examining the nephrectomy specimen.[2][4]

5. We propose to expand the Epidemiology Section and add context with the introduction sentence: Wilms tumor is the most common malignant renal tumor in children. There are a number of rare genetic syndromes that have been linked to an increased risk of developing Wilms Tumor.[5] Screening guidelines vary between countries; however health care professionals are recommending regular ultrasound screening for patients with associated genetic syndromes[6].

Syndrome Name Associated Genetic Variant Risk for Wilms tumor Description of Syndrome
WAGR syndrome (Wilms tumor, aniridia, genital anomalies, retardation) Gene deletion that includes both WT1 and PAX6 45-60% Characterized by Wilms tumor, aniridia (absence of iris), hemihypertrophy (one side of body larger other), genitourinary abnormalities, ambiguous genitalia, intellectual disability. (Dome, 2016)
Denys-Drash syndrome (DDS) WT1 (exon 8 and 9) 74% Characterized by kidney diseases since birth leading to early-onset kidney failure, ambiguous genitalia (intersex disorders). (Dome, 2016)
Beckwith-Wiedemann Syndrome Abnormal regulation of chromosome 11p15.5 7% Characterized by macrosmia (large birth size), macroglossia (large tongue) hemihypertrophy (one side of body larger than other), other tumors in body, omphalocele (open abdominal wall) and visceromegaly (enlargement of organs inside abdomen). (Dome, 2016)

6. We propose to remove: Beckwith-Wiedemann syndrome, Denys-Drash syndrome, W, and WAGR syndrome from the see also section as per the Wikipedia manual of style as this section should be avoided when possible and they have been referenced about.

We welcome any feedback or other suggestions from the community. Thank you!

  1. ^ a b Wilms Tumor and Other Childhood Kidney Tumors Treatment". National Cancer Institute. Retrieved 2018-11-05
  2. ^ a b c d e f g PDQ Pediatric Treatment Editorial Board. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Health Professional Version. 2018 Apr 2. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK65842)
  3. ^ a b Chintagumpala M, Muscal JA, Pappo AS, Kim MS. Presentation, diagnosis, and staging of Wilms tumor. Pappo AS. 2013.
  4. ^ a b c d Szychot E, Apps J, Pritchard-Jones K. Wilms’ tumor: biology, diagnosis and treatment. Translational pediatrics. 2014 Jan;3(1):12.
  5. ^ Sonn, G., & Shortliffe, L. M. (2008). Management of Wilms tumor: current standard of care. Nature Reviews Urology, 5(10), 551.
  6. ^ Kalish, J. M., Doros, L., Helman, L. J., Hennekam, R. C., Kuiper, R. P., Maas, S. M., ... & Rednam, S. (2017). Surveillance recommendations for children with overgrowth syndromes and predisposition to Wilms tumors and hepatoblastoma. Clinical Cancer Research, 23(13), e115-e122.