Wilms' tumor

The nephroblastoma ( Wilms' tumor also, after Max Wilms, a German surgeon, 1867-1918 ) is the most common malignant renal tumor in childhood. He goes from embryonic tissue remnants of the kidney, called the metanephric blastema, from. Normally, this tissue disappears later than the 36th week of pregnancy.

Epidemiology

The peak incidence is between the second and third year of life and affects one in 10,000 live births. Two-sided occurrence is observed in 5% of cases and may be due to a familial tumor. The genetics of familial Nephroblastoma is not yet understood. Are of importance, inter alia, the Wilms ' tumor gene WT 1 on chromosome 11p13, which plays an important role in the physiological development of the kidney and possibly also of the heart, and WT-2 at 11p15 and Wnt / beta -catenin signaling pathway or WNT / β -catenin signaling pathway.

Symptoms

The clinical symptoms are usually nonspecific: often occurs a painless increase in waist circumference on, and abdominal pain and a palpable mass of the upper abdomen occur ( visceromegaly, enlargement of organs ). The nephroblastoma associated anomalies are further aniridia ( hypoplasia of the iris ), a hemihypertrophy ( enlargement of a body) and urogenital malformations. Bloody urine ( hematuria) is only observed in 5-10 % of cases.

Histopathology

There are usually nephroblastoma tumor of soft tissue, which can weigh just 500 grams to one kilogram until the discovery. Not infrequently the tumor grows like a plug along the renal vein. Metastases are predominantly found in the regional lymph nodes and in the lungs. Under the microscope, there are small blastäre cells with a low proportion of the cytoplasm. In addition, however epithelial and stromal differentiations occur.

Therapy

The therapy consists of a combined surgical removal of the tumor, chemotherapy and radiation. The exact regimen is the tumor stage (staging) and tumor histology (grading) dependent. The German Cancer Society and the German Society for Pediatric Oncology and Hematology give to the current diagnostic and therapeutic measures out a guideline in accordance with the generally recognized state of the science.

The prognosis of the disease is good at guideline- compliant therapy. It depends on the stage of the disease and the histological subtype, 90% of patients are cured in the long term.

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