Wilms' tumor overview

Jump to navigation Jump to search
https://https://www.youtube.com/watch?v=Ode2LYAS5CA%7C350}}

Wilms' tumor Microchapters

Home

Patient Information

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Wilms' Tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

Echocardiography or Ultrasound

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Wilms' tumor overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Wilms' tumor overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Wilms' tumor overview

CDC on Wilms' tumor overview

Wilms' tumor overview in the news

Blogs on Wilms' tumor overview

Directions to Hospitals Treating Wilms' tumor

Risk calculators and risk factors for Wilms' tumor overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

Wilms' tumor or nephroblastoma is a tumor of the kidneys that typically occurs in children, rarely in adults. It was first described by Dr. Osler in 1814. The tumour typically arises from mesodermal precursors of the renal parenchyma (metanephros). On microscopic histopathological analysis, tubules, solid sheets of small round cells, and stroma are characteristic findings of wilms' tumor. It may be caused by either genetic mutations or chromosomal alterations. The risk factors of wilms' tumor include family history, congenital anomalies, and associated syndromes. The complications of wilms' tumor include metastasis, high blood pressure, and kidney damage. Prognosis is generally good. The 5-year survival rate for Wilms tumor in children is around 90%, whereas older patients suffer worse outcome. The common symptoms include swelling, abdominal pain, fever, hypertension, and hypercalcemia. On abdominal examination a painless mass may be palpated in the flank. CT scan, MRI, Ultrasound, PET, and biopsy may be helpful in the diagnosis. The predominant therapy for wilms' tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.

Historical Perspective

Wilm's Tumor was first discovered by Dr. Osler, in 1814.The association between Wilm's tumor 1 (WT1) and Wilm's tumor was made in 1990. Wilm's tumor is named after Dr. Max Wilms (1867-1918) who is a surgeon and pathologist from Germany. In 1969, the National Wilms Tumor Study (NWTS) group devised a therapy regimen for wilms tumor. 

Pathophysiology

Wilms tumor has a triphasic appearance. It is comprised of 3 types of cells which are stromal, epithelial and blastemal. All the 3 types are not required for the diagnosis of wilms tumor. Primitive tubules and glomeruli are often seen comprised of neoplastic cells. Beckwith and Palmer reported in NWTS the different histopathologic types of wilms tumor to categorize them based on prognosis. Lesions comprising of nephrogenic rests can lead to wilms tumor. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. Based on a study wilms tumor is divided into 2 pathologic categories: favorable and anaplastic. Wilms tumor (hereditary or sporadic) appears to result from changes in one or more of at least ten genes. The changes may be somatic or germline. Aberrations in germline or clonal WT1, WT2, and Wnt activation when combined with stage of development of the nephron, characterize different subsets of wilms tumor that can be differentiated by using gene expression profiling. This genetic/ontogenic categorization describes some of the heterogeneity among wilms tumors.

Causes

Wilms' tumor may be caused by either genetic mutations or chromosomal alterations.

Wilms' tumor differentiating from other disease

Wilms' tumor must be differentiated from neuroblastoma, cystic nephroma, and angiomyolipoma.

Epidemiology and Demographics

Wilms tumors are the most common pediatric renal mass. The incidence of Wilms tumor is estimated to be 0.71 cases per 10,00,000 children younger than 15 years. There is no significant gender predilection among unilateral cases, but the male to female ratio in bilateral cases is 0.60:1.00. It typically occurs in early childhood with peak incidence between 3 and 4 years of age. Asian individuals are less likely to develop wilms tumor.

Risk factors

Common risk factors in the development of wilms tumor include familial wilms tumor, congenital anomalies, WT-1 related syndromes and WT2-related syndromes. Less common risk factors in the development of wilms tumor include perlman syndrome, simpson-golabi-ehemel syndrome, Sotos syndrome, 9q22.3 microdeletion syndrome, Bloom syndrome, li-fraumeni syndrome and Alagille syndrome.  

Screening

Screening can be very helpful to diagnose wilms tumor in high risk patients.Screening is done with serial abdominal ultrasonography.  

Natural history, Complications and Prognosis

The complications of wilms' tumor include metastasis, high blood pressure, and kidney damage. Prognosis is generally good. The 5-year survival rate for Wilms tumor in children is around 90%, whereas older patients suffer worse outcome. The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years. Also, according to the different stage, anaplastic tumors have the relatively poorer outcome.

Staging

There are 5 stages of wilms' tumor based on both the results of the imaging studies and the surgical and pathologic findings at nephrectomy.

History and Symptoms

The common symptoms of wilms' tumor include swelling, abdominal pain, fever, hypertension, and hypercalcemia.

Physical Examination

On abdominal examination a painless mass may be palpated in the flank.

CT

CT scan may be helpful in the diagnosis of wilms' tumor.

MRI

MRI may be helpful in the diagnosis of wilms' tumor.

Ultrasound

Ultrasound may be helpful in the diagnosis of wilms' tumor.

Other Diagnostic Studies

18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT and biopsy may be helpful in the diagnosis of wilms' tumor.

Medical therapy

The predominant therapy for wilms' tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.

Surgery

Surgery is the mainstay of treatment for wilms' tumor.

References

Template:WH Template:WS