Wilms Tumor (Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, PA: LWW; 2011;861–885)
• Overview/Epidemiology
• Malignant tumor of kidney arising in nephrogenic rests; 500 new U/S cases annually
• 2nd most common abdominal tumor in childhood; occurs early (mean age 3.5 yr)
• Usually sporadic but ↑ risk in several genetic d/o 2/2 involvement of WT1 tumor suppressor gene (Beckwith–Wiedemann, Denys–Drash, WAGR, hemihypertrophy, aniridia)
• May be bilateral (especially if genetic predisposition)
• Classic histology: Triphasic (“favorable”)
• Histologic variants: Focal or diffuse anaplasia (more aggressive)
• Clinical presentation/Evaluation
• Most common is asymptomatic abdominal mass
• Also: Hematuria, fever, hypertension, abd pain, anemia from intratumoral hemorrhage
• Metastasizes to regional lymph nodes, lung, liver
• Imaging: CXR, abdominal CT or MRI, U/S to assess for IVC thrombus, chest CT
• Treatment/Outcome
• Surgery is crucial: Nephrectomy up-front unless bilateral or unresectable
• Radiation post-operatively for stage III and IV disease
• Chemotherapy with 2 drugs (stage I/II) or 3 drugs (stage III/IV): Vincristine, actinomycin D, +/− doxorubicin
• Prognosis generally excellent for all stages unless more aggressive histology
Rhabdomyosarcoma (Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, PA: LWW; 2011;923–953)
• Overview/Epidemiology
• Most common soft tissue sarcoma (STS) in children; ½ of STS, 3% of childhood CA
• Malignant tumor arising from muscle (primitive mesenchymal cell) occurs anywhere
• 350 new cases in the United States per year; 2/3 of cases under 6 yr of age
• Clinical presentation/Evaluation
• Symptoms depend on site involved
• Most common sites: Head & neck, esp orbit, GU, trunk, extremities
• 2 major histologies: Embryonal (favorable), alveolar
• Metastasizes to lung, bone, bone marrow
• Imaging: CT or MRI primary site, chest CT, bone scan and/or PET
• Treatment/Outcome
• Multi-modality with chemotherapy (most common regimen is VAC: Vincristine, actinomycin D, cyclophosphamide) plus local control with surgery and/or radiation
• Prognosis related to age (infants and teens higher risk), histology, site, stage
Ewing Sarcoma (Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, PA: LWW; 2011;987–1014)
• Overview/Epidemiology
• 2nd most common primary malignant bone tumor
• Family of tumors thought to be of neural crest origin. Includes Ewing sarcoma of the bone, soft tissue, Askin tumor of thoracic wall, and primitive neuroectodermal tumor
• 200 new cases in the United States per year; >50% occur in 2nd decade (mean age = 15 yr)
• More common in Caucasians; very rare in African-Americans
• Pathology: Small, round, blue-cell tumor, 85% with translocation EWSR1/FLI1 t(11;22)
• Clinical presentation/evaluation: Pain, swelling, tenderness over involved bone or soft tissue. Can affect any bone, but most commonly pelvis and femur. If more advanced/metastatic may have systemic sxs (fever, weight loss, etc)
• Imaging: X-ray may show osteolysis, detachment of the periosteum from the bone (Codman triangle) given an “onion-skinning” appearance of the affected bone. MRI of primary site, chest CT, bone scan +/– PET scan
• Treatment/Outcome
• Multi-modality w/ chemo (most common regimen; vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide) plus local control with surgery and/or radiation
• Prognosis: Better prognosis in pts w/o mets, tumor size <200 mL, good response to chemo, extremity lesions vs. axial), younger children, type I transcript (EWSR1/FLI1)