Wilms tumor - symptoms and treatment
Wilms tumor is an embryonic tumor of the kidney, consisting of blastomic, stromal and epithelial elements. Genetic disorders are important in the pathogenesis, but family inheritance is only 1-2% of cases. The diagnosis is made on the basis of ultrasound and CT of the abdominal cavity and is confirmed by biopsy. Treatment may include surgical resection, chemotherapy and radiation therapy.
Wilms' tumor usually occurs in children under 5 years old, but occasionally in older children and extremely rare in adults. Wilms' tumor accounts for approximately 6% of tumors in children under 15 years of age. Bilateral synchronous tumors develop in about 4% of cases, with bilateral damage most often found in very young children, especially girls. Chromosomal degradation of WT1, the Wilms tumor suppressor gene, has been identified in some cases. Other associated genetic abnormalities include a deletion of WT2, a deletion of chromosome 16, and a duplication of chromosome 12.
In about 12% of cases, there are other congenital anomalies, especially of the genitourinary system, but hemihypertrophy is also common. WAGR syndrome is a combination of Wilms' tumor, developmental disorders of the urogenital system and mental retardation.
Wilms Tumor - Symptoms and Signs
The most frequent manifestation is a painless palpable mass in the abdominal cavity. Less common signs are abdominal pain, hematuria, fever, anorexia, nausea and vomiting. Hematuria indicates involvement in the pathological process of the collecting system. Hypertension can develop if compression of the renal vessels or parenchyma causes renal ischemia.
Wilms tumor - diagnosis
Ultrasound examination of the abdominal cavity determines the acid or solid structure of the formation, as well as damage to the renal vein or portal vein. An abdominal CT scan is necessary to determine the size of the tumor and its spread to the regional lymph nodes, the opposite kidney or liver. The diagnosis is confirmed by education biopsy. Renal arteriography, portography, retrograde urography, or excretory urography are rarely required.Chest X-ray is necessary for the detection of metastases in the lungs at the time of detection of the disease.
Wilms tumor - treatment
The prognosis depends on the histological picture, the stage of the disease at diagnosis and the age of the patient. Outcomes in children with Wilms tumor are favorable. The percentage of cure in children with the initial stage of the lesion ranges from 85 to 95%. Even in children with more advanced stages, the prognosis is quite good, with a cure rate varying from 60% to 90%. Relapses occur, usually within the first 2 years after the disease is detected. Recovery is possible in children with recurrent tumors. The outcome after relapse is better in children who had the initial stages of the disease, the tumors recurred in places that had not been irradiated, in which relapse occurred more than a year after the first manifestations and they received less intensive treatment of the primary tumor.
Wilms' National Tumor Study Group has established criteria for determining the stage of the disease and treatment guidelines. The corresponding diagnostic operation is shown to clarify the potentially resected tumor, with the study of another kidney.If the tumor is unilateral and limited to the kidney or if the spread is minimal, total resection is performed by nephrectomy, followed by therapy with vincristine and actinomycin D. If the tumor is unilateral, but has metastases, or if the tumor is bilateral, chemotherapy with actinomycin D and vincristine is performed with or without radiation therapy . Children with more severe stages also receive doxorubicin. Other commonly used drugs include cyclophosphamide, ifosfamid, and etoposide. Children with very large non-resectable tumors or bilateral lesions are candidates for chemotherapy, followed by re-examination and possibly resection.
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