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The embryonic kidney, or Wilms' tumor, is a kidney cancer that most often affects children from 3 to 4 years of age. Even though it is a malignant tumor, the prognosis is very good - most patients recover. What are the causes and symptoms of nephroma? How is the treatment going?

Embryonic kidney(Latinnephroblastoma ), otherwiseWilms tumor , fetal nephroblastoma or Malignant nephroblastoma is the most common malignant neoplasm of the kidneys in children. It accounts for about 97 percent. tumors of this organ and 7-8 percent. malignant tumors in the youngest. It affects 1 in 10,000 children under 15 years of age, but is most commonly diagnosed in those between 3 and 4 years of age. Occasionally, the disease is diagnosed in adolescents and is practically absent in adults. In most cases (90-95%), the tumor only affects one kidney.

Germinal kidney (Wilms' tumor): causes

The embryonic kidney is formed from the remains of the embryonic nephrogenic tissue (hence the name embryonic nephrotic tissue), i.e. the surviving fetal tissue. These residues are detected in about 1% of he althy newborns and they most often disappear. Mutations in the WT1 and WT2 genes (located on chromosome 11), which play an important role in the development of the kidney and gonads, are believed to predispose this type of remnant to survive after birth and tumor development.

There is also a familial form of Wilms' tumor, which accounts for 1-2% of cases of these tumors. Then the disease is inherited autosomal dominantly.

Germinal kidney (Wilms' tumor): symptoms

  • stomach pains
  • nausea and vomiting
  • fever for no specific reason
  • recurrent urinary tract infections (refractory to treatment)
  • hematuria
  • hypertension
  • constipation
  • a painless tumor in the abdomen that can be felt when touching the abdomen
  • significant enlargement of the abdominal circumference

U about 10 percent patients develop malformations, in particular cryptorchidism (when the testicles have not descended into the scrotum, but stopped in the abdominal cavity) and hypospadias (when the opening of the urethra is located on the abdominal side of the penis).

In addition, Wilms' tumor may coexist with other congenital syndromes such as syndromeBeckwith-Wiedemann's syndrome, WAGR syndrome, Denys-Drash syndrome, Perlman syndrome and Edwards syndrome.

Germinal kidney (Wilms' tumor): diagnosis

If there is a suspicion of embryonic nephroblastoma, ultrasound of the abdominal cavity and kidneys is performed first. When the result of this examination is doubtful or inconclusive, a CT scan or magnetic resonance imaging can be performed.

In addition, the doctor may order urography to detect a tumor located inside the kidney, or renoscintigraphy (isotope examination of the kidneys). A biopsy may also be recommended (but it cannot be performed, among others, in children under 3 months of age).

Chest X-ray is recommended, preferably lung computed tomography (as the tumor usually metastasizes to this organ).

Germinal kidney (Wilms' tumor): treatment

Treatment of nephroma consists of several stages:

1. Preoperative chemotherapy - 4-6 weeks before the surgery, chemotherapy is performed, thanks to which the risk of metastases can be reduced or eliminated, as well as the size of the tumor. Then it is easier for doctors to completely excise the neoplastic tissue. Chemotherapy also prevents the tumor from rupturing. 2. Nephrectomy, i.e. surgical excision of the tumor along with the kidney. 3. Postoperative chemotherapy - if Wilms' tumor is still present after surgery, chemotherapy is re-administered, which lasts from 4 to 27 weeks (depending on the stage of the tumor). In patients with a high degree of advancement, radiotherapy is additionally used.

Follow-up is required after the cure. Ultrasound is performed every 3 months until the age of 7 and computed tomography 3 months after the operation.

Embryonic kidney (Wilms' tumor) - prognosis

In most cases (stages I and II) it is possible to heal up to 90%. patients. In patients with stage III-V (who are usually associated with bilateral kidney involvement, lung metastases or lymph node metastases), the chances of recovery are 70%.

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Category:

Oncology