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Pediatric_Oncology_A_Comprehensive_Guide.pdf
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11 Nephroblastoma (Wilms Tumor)

133

 

 

11.14.3.1 Pathology

Tumor origin of epithelial tissue of the proximal tubule

11.14.3.2 Clinical Manifestations

Pain, intra-abdominal tumor, hematuria

Diagnostic procedure as in nephroblastoma (see above); often tumor calcifications

Metastatic spread often in lung, liver, regional lymph nodes, and bone

11.14.3.3Therapy

Complete resection

In high-risk patients after surgery, combination treatment with interferon-a, interleukin-2, or high-dose radioand chemotherapy, since renal cell carcinoma is only moderately sensitive to radioand chemotherapy. Treatment with TK inhibitors for renal cell carcinoma seems to be effective

11.14.3.4Prognosis

In localized tumor 60% survival

In metastatic disease: <40% survival

11.14.4 Renal Rhabdoid Tumor

Rare tumor

Mainly in children <2 years of age

Cytogenetic alterations at chromosome 22q11 and SMARCB1 (INI 1) mutation

Despite aggressive treatment survival is less than 40%

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