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Neuroblastoma Staging, from Campbell's

International Neuroblastoma Risk Group classification, from Campbell's

Wilms tumor COG staging, from Campbell's

Neuroblastoma

• Most common extracranial solid tumor of childhood, 50-70% present with metastatic disease
• Low stage tumors may spontaneously regress
• Stroma poor tumors have poor prognosis (< 10%)
• Presentation: abdominal pain or mass, bone/joint pain, ecchymosis, cough, neurologic symptoms, retention, constipation
• Catecholamine secretion: paroxysmal hypertension, palpitations, flushing headache
• VIP secretion: diarrhea and hypokalemia
• Acute myoclonic encephalopathy: myoclonus, opsoclonus, ataxia, can lead to prolonged neurologic impairment in 70-80%
• Labs: elevated urinary catecholamine metabolites (VMA/HVA) found in 90-95%
• Marrow aspirate x2 and biopsy x2 are recommended for staging
• Management: immediate surgery for localized tumors, otherwise reasonable to treat with neoadjuvant chemotherapy

Nephroblastoma (Wilms tumor)

• Most common primary malignant renal tumor of childhood
• If high risk (WAGR, Denys-Drash, Fanconi anemia, Perlman, Mosaic variegated aneuploidy), recommend screening renal US
• Nephrogenic rests: seen in 1/3 Wilms, increase need for surveillance, can form thick kidney rind
• 85% present with mass, pain, or hematuria
• Labs: obtain CBC, CMP, calcium, UA, coags (5-10% have von Willebrand disease), urine catecholamines (if concern for neuroblastoma)
• Imaging: usually discovered with US, obtain CT/MR A/P with PO+IV contrast and CT with contrast, bone scan only if bone pain or elevated AlkPhos
• Management: radical nephrectomy with regional LN dissection, unless surgically inoperable (per surgeon)
• Surgical contraindications: tumor thrombus above hepatic veins, invasion into local structures requiring extensive resection, pulmonary compromise, severe risk morbidity/mortality
• Bilateral tumors: up to 5%, give chemotherapy then bilateral partial nephrectomy
• Chemotherapy: vincristine, dactinomycin, +/- doxorubicin, cyclophosphamide, etoposide
• Recurrence: most occur within 2yr treatment, lung most common site

Other renal tumors

• Clear cell sarcoma: 3% of renal tumors, 50% present with bone metastases (evaluate with bone scan), 30% at risk for relapse, may require XRT, managed with doxorubicin and surgery
• Rhabdoid tumor: 2% of renal tumors, high risk for brain mets (screen with brain imaging), aggressive, resistant to chemo/XRT, high mortality rate
• Congenital mesoblastic nephroma: most common renal tumor in infants and diagnosed via antenatal ultrasound, usually managed with surgery only
• Solitary multilocular cyst, cystic partially differentiated nephroblastoma: benign tumor with multiple various sized cysts compressing normal parenchyma, considered on spectrum with nephroblastoma, managed with surgery
• Renal cell carcinoma: higher incidence of pRCC, most common renal tumor of second decade, manage with surgery
• Angiomyolipoma (AML): rare in children, treat if > 3-4cm, everolimus then embolization/surgery