Wilms tumor

Visão geral 
Teoria 
Diagnóstico 
Tratamento 
ACOMPANHAMENTO 
Recursos 

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Última revisão: 30 Apr 2025

Última atualização: 05 Apr 2023

Wilms tumor is the most common renal malignancy in children.

Usually presents as a unilateral, painless, abdominal/flank mass; less commonly presents bilaterally.

Metastatic disease occurs in <10% of patients and needs to be carefully excluded using ultrasound and computed tomography/magnetic resonance imaging.

Long-term survival approaches 90% in localized disease.

Treatment is with nephrectomy, chemotherapy, and radiation therapy.

Definição

Wilms tumor, or nephroblastoma, is the most common form of renal malignancy in childhood.[1] It usually occurs in the first 5 years of life.[2] The risk for developing Wilms tumor is increased in certain congenital overgrowth syndromes, congenital nonovergrowth syndromes, and children with congenital urogenital anomalies.[3] Long-term survival approaches 90% in localized disease.[2]

História e exame físico

Principais fatores diagnósticos

upper abdominal/flank mass or swelling
congenital urogenital anomalies
congenital syndromes

Detalhes completos

Outros fatores diagnósticos

abdominal distension
abdominal pain
hypertension
hematuria
poor appetite or weight loss
fever
pallor
shortness of breath
hepatomegaly
varicocele
family history of Wilms tumor
hypoglycemia in infancy
features of paraneoplastic syndrome

Detalhes completos

Fatores de risco

age <5 years
congenital urogenital anomalies
congenital syndromes
family history of Wilms tumor
prenatal exposure to harmful environmental factors

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

CBC
renal function
liver function tests
urinalysis
serum total protein/albumin
coagulation studies
serum calcium level
abdominal ultrasound with Doppler
CT or MRI abdomen and pelvis with and without contrast
CT chest with and without contrast
chest x-ray

Detalhes completos

Investigações a serem consideradas

tumor histology
genetic testing
loss of heterozygosity (LOH) studies

Detalhes completos

Algoritmo de tratamento

AGUDA

COG criteria

SIOP criteria

CONTÍNUA

tumor recurrence

Colaboradores

Autores

Sandeep Batra, MD

Clinical Assistant Professor

Riley Hospital for Children

Department of Pediatrics

Indiana University School of Medicine

Indianapolis

Declarações

SB declares that he has no competing interests.

Revisores

Nadine Deannie Lee, MD

Pediatric Hematologist-Oncologist

Hematology/Oncology

Riley Children's Hospital

Indiana University Health

Indianapolis

Declarações

NDL declares that she has no competing interests.

Norbert Graf, MD

Direktor

Klinik für Pädiatrische Onkologie und Hämatologie

Universitätsklinikum des Saarlandes

Homburg

Germany

Declarações

NG declares that he has no competing interests.

Zelig Tochner, MD

Associate Professor

Radiation Oncology

Children's Hospital of Philadelphia

Philadelphia

Declarações

ZT declares that he has no competing interests.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Spreafico F, Fernandez CV, Brok J, et al. Wilms tumour. Nat Rev Dis Primers. 2021 Oct 14;7(1):75.Texto completo Resumo

Lopyan NM, Ehrlich PF. Surgical management of Wilms tumor (nephroblastoma) and renal cell carcinoma in children and young adults. Surg Oncol Clin N Am. 2021 Apr;30(2):305-23. Resumo

Vujanić GM, Parsons LN, D'Hooghe E, et al. Pathology of Wilms' tumour in International Society of Paediatric Oncology (SIOP) and Children's Oncology Group (COG) renal tumour studies: similarities and differences. Histopathology. 2022 Jun;80(7):1026-37. Resumo

van den Heuvel-Eibrink MM, Hol JA, Pritchard-Jones K, et al. Position paper: rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol. 2017 Dec;14(12):743-52.Texto completo Resumo

Dome JS, Mullen EA, Dix DB, et al. Impact of the first generation of Children's Oncology Group clinical trials on clinical practice for Wilms tumor. J Natl Compr Canc Netw. 2021 Aug 1;19(8):978-85.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Neuroblastoma
- Clear cell sarcoma of the kidney
- Renal cell carcinoma
Mais Diagnósticos diferenciais
Diretrizes
- Wilms tumor (nephroblastoma)
- SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low income setting
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice

O uso deste conteúdo está sujeito ao nosso aviso legal

Wilms tumor

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

COG criteria

SIOP criteria

tumor recurrence

Colaboradores

Autores

Sandeep Batra, MD

Declarações

Revisores

Nadine Deannie Lee, MD

Declarações

Norbert Graf, MD

Declarações

Zelig Tochner, MD

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice