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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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