What is the Wilms Tumor

(Wilms’ tumor, nephroblastoma)

Understanding the Wilms Tumor
Cancer is categorized typically when a group of cells in the body begin to grow out of control often with detrimental impact. Cells in practically any section of the body can grow cancerous and can spread to other areas of the body.

Wilms tumor (also called Wilms’ tumor or nephroblastoma) is a type of cancer that originates in the kidneys. It is the most prevalent type of kidney cancer in children. It is named after Max Wilms, a German doctor who drafted one of the first medical articles concerning the disease in 1899.

The effect on the Kidneys
The kidneys are two bean-shaped organs that are appended to the back wall of the abdomen. One kidney is just to the left and the other only to the right of the backbone. The lower rib cage protects the kidneys.

Small glands called adrenal glands sit on top of each of the kidneys. Each kidney and adrenal gland is surrounded by fat and a thin, fibrous capsule (known as Gerota’s fascia).

illustration showing the kidneys in relation to the renal artery and vein, adrenal gland, ureter, bladder, and urethra with a window showing greater detail including adrenal gland, gerota’s fascia, renal pelvis, renal artery, and vein, ureter, and fat
The main priority of the kidneys is to filter blood proceeding from the renal arteries to alleviate the body of excess salt, water, and unneeded waste products. These elements become urine. Urine then exits the kidneys through long, slender conduits named ureters that link to the bladder. Urine flows down the ureters into the bladder and is collected there until the person urinates.

The kidneys also have other jobs:

They assist regulating blood pressure by creating a hormone called renin.
They help ensure the body has enough red blood cells by producing a hormone called erythropoietin. This hormone tells the bone marrow to create more red blood cells.
Our kidneys are essential, but the body actually requires only one entire kidney to do all of its essential functions. Many people across the globe live normal, healthy lives with just one kidney.

Wilms tumors
Wilms’ tumor is an embryonal carcinoma of the kidney from

blastocytic

stromal and epithelial fractions. Genetic disorders are suspected in the pathogenesis, but a family inheritance there is only 1-2% of cases. The diagnosis is made by ultrasound, abdominal computed tomography or MRI Treatment consists of surgical resection, chemotherapy, and radiation.

Wilms’ tumor usually occurs in children <5 years, but sometimes can also occur in older children or rare in adults. Wilms’ tumor accounts for about 6% of cancers in children from <15 years. Bilateral synchronous tumors occur in about 5% of cases.

Wilms’ tumor is an embryonal carcinoma of the kidney from

blastocytic

stromal and epithelial fractions. Genetic disorders are suspected in the pathogenesis, but a family inheritance there is only 1-2% of cases. The diagnosis is made by ultrasound, abdominal computed tomography or MRI Treatment consists of surgical resection, chemotherapy, and radiation.

Anatomy of the Kidneys

Wilms’ tumor usually occurs in children <5 years, but sometimes can also occur in older children or rare in adults. Wilms’ tumor accounts for about 6% of cancers in children from <15 years. Bilateral synchronous tumors occur in about 5% of cases. A chromosomal deletion of WT1, a Wilms tumor suppressor gene has been identified in some cases. Another associated gene abnormality includes a deletion of WT2 (a second Wilms’ tumor suppressor gene), loss of heterozygosity of 16q and 1p, and inactivation of the WTX gene.

Approximately 10% of cases have congenital malformations, especially urogenital malformations, but also a hemihypertrophy (asymmetry of the body). The WAGR syndrome is a combination of Wilms tumor (WT1 with deletion), aniridia, urogenital malformations (z. B. renal hypoplasia, renal cysts, hypospadias, cryptorchidism), and mental retardation. Symptoms and signs.

The most common finding is a painless, palpable abdominal tumor. Less common are abdominal pain, hematuria, fever, anorexia, vomiting, and nausea find. Hematuria can be microscopic or gross. Hypertension may occur and is of varying severity. Diagnosis Abdominal ultrasound and CT or MRI By Abdominal sonography can be determined whether the tumor is cystic or solid nature and whether the renal veins or vena cava are infiltrated.
Abdominal computed tomography or -MRT is necessary to clarify the extent of the tumor and spread to the lymph nodes, the contralateral kidney or liver. A chest x-ray and possibly a chest computed tomography are needed to detect pulmonary metastasis at initial diagnosis. The diagnosis of Wilms’ tumor is typically allegedly based on the results of imaging studies. Therefore nephrectomy instead of a biopsy in most patients instead is the time of diagnosis.

A biopsy is not performed because of the risk of contamination by peritoneal tumor cells, as they spread cancer and would result in a higher stage, requiring more intensive therapy. During surgery locoregional lymph nodes for pathological and surgical staging are sampled (see also National Cancer Institute Stages of Wilms tumor).

Prognosis The prognosis for Wilms ‘tumor depends on the histology (favorable or unfavorable) the time of diagnosis the patient’s age (older age is associated with a worse prognosis) The outcomes for children with Wilms’ tumor is excellent. The cure rate for the low stages of the disease (only the involvement of the kidney) is between 85% and 95%. Even children with the more advanced disease have a 60% (in the case of poor histology) and 90% (with good histology) good therapeutic results.

Relapses occur within two years after diagnosis. A cure is possible in children with recurrent carcinoma. Had success rates of recurrence of the tumor are better in children who initially had a low stage whose tumor originated on one side that were not irradiated, again ill later than 1 year after the initial diagnosis and the originally received a less aggressive therapy , Treatment Surgery and chemotherapy radiotherapy in patients with a higher level of risk disease Initial treatment of unilateral Wilms tumor occurs primarily by surgical resection followed by adjuvant chemotherapy.

A select group of younger patients with small tumors can be cured by surgery alone. The type of cytotoxic drugs and length of treatment depend on histology and stage of the tumor. Chemotherapy depends on the risk group but is usually from actinomycin D and vincristine with or without doxorubicin.

In more aggressive tumors intensive multi-agent chemotherapy may be used. Children with large, non-resectable tumors are candidates for chemotherapy, followed by a re-evaluation and subsequent resection. Children who are iller or who have tumors that affect the regional lymph nodes received radiotherapy. Further information Stages of Wilms tumor (National Cancer Institute)

Health Life Media Team

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