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Título

WILMS TUMOR: A LITERATURE REVIEW

Resumo

INTRODUCTION: The Wilms tumor is the main kidney neoplasm in the pediatric population, corresponding to about 5% of all childhood neoplasms.
OBJECTIVE: Describe the main features of the Wilms tumor.
METHODOLOGY: A literature review was carried out using Pubmed databases in the period of February to first half of March 2022, using the descriptors ''Wilms tumor'' and ''Review'' with filter for publications from the last ten years.
RESULTS: The Wilms Tumor corresponds to a malignant embryonic tumor, so most of the cases are diagnosed in childhood, making adult cases extremely rare. Certain syndromes are related to an increased risk of developing the neoplasm, since in about 9-17% of cases there is a previous syndrome such as WAGR Syndrome (Wilms Tumor, aniridia, genitourinary anomalies, mental retardation), Beckwith-Wiedemann Syndrome, Denys-Drash Syndrome, among others. In addition, the presentation occurs mostly unilaterally, but in 10% of cases there is bilateral or multicentric involvement. In general, post-treatment survival has increased in recent years, reaching about 90%, with recurrence occurring in 15% of cases. However, there are cases of metastasis resulting from Wilms Tumor, with the lung being the main organ affected. The main clinical manifestation is the presence of an isolated abdominal mass. Other manifestations include: abdominal pain, hematuria, anemia, hypertension, subcapsular hemorrhage. The definitive diagnosis is made by means of biopsy and histological examination, but initially ultrasound is used to visualize the renal tumor. Treatment is preferably by a surgical approach aiming to save renal function, but it depends on the tumor extension and the existence of metastasis.
CONCLUSION: The Wilms tumor corresponds to the main renal tumor in the pediatric age group, but it has a high survival rate if diagnosed and properly managed. In a number of cases, there is a close relationship with previous genetic syndromes, and screening through imaging tests, such as ultrasound, is important in these specific cases. Clinical manifestations are diverse, ranging from nonspecific urinary signs and symptoms to an abdominal mass and subcapsular hemorrhage. Therefore, Wilms' tumor is an important differential diagnosis in childhood urological complaints, especially in children with genetic syndromes.

Palavras Chave

Wilms Tumor; Review; Kidney Neoplasm

Área

Tumores raros

Instituições

Universidade Luterana do Brasil - Rio Grande do Sul - Brasil

Autores

ANDREI LEONARDO SCHUSTER, BÁRBARA FRANCCESCA BRANDALISE BASSANI, ELSON ROMEU FARIAS