A 7-year-old girl is diagnosed with medulloblastoma following resection of a cerebellar mass. Molecular subtyping is performed to guide risk stratification and subsequent therapy. The identification of which molecular subgroup is associated with the most favorable prognosis and would likely qualify the patient for reduced-intensity therapy to minimize long-term sequelae?
A 3-year-old child undergoes a radical nephrectomy for a large renal mass, confirmed to be a Wilms tumor with favorable histology. As part of the risk-stratification protocol, the tumor is sent for genetic analysis. The combined loss of heterozygosity (LOH) at which two chromosomal loci is considered a significant adverse prognostic factor and would warrant treatment intensification, even with favorable histology?
A 14-year-old female is evaluated for concerns about her growth and development. Her history is significant for the subacute development of obesity, a decreased linear growth velocity over the past two years, and delayed puberty. An ophthalmology exam reveals bitemporal hemianopsia. These findings are most suggestive of which type of childhood central nervous system tumor?
A 17-year-old female with no past medical history presents with abdominal pain and a large liver mass. Biopsy confirms hepatocellular carcinoma. She has no evidence of cirrhosis or viral hepatitis. Further molecular analysis of the tumor tissue reveals a distinct DNAJB1-PRKACA translocation. Which histologic variant of hepatocellular carcinoma is associated with this molecular finding and clinical profile?
A 1-month-old male is found to have a large, solid renal mass on an ultrasound performed for abdominal distention. Polyhydramnios was noted during the prenatal period. Radical nephrectomy is performed for the mass. Which of the following is the most likely pathologic diagnosis?
A 2-year-old girl is found to have a mass in her distal extremity. Biopsy confirms infantile fibrosarcoma, and molecular testing reveals a t(12;15)(p13;q25) translocation. Given this specific molecular finding, which of the following therapeutic approaches should be considered as a first-line therapy?
An 18-month-old prepubertal male undergoes a radical inguinal orchiectomy for a testicular mass. Pathology confirms a stage I, pure yolk sac tumor that was completely resected. Staging evaluation, including imaging and serum tumor markers, is negative for any residual or metastatic disease. What is the most appropriate subsequent management for this patient?
A 2-year-old boy presents with a large abdominal mass. Imaging reveals a large, unifocal liver tumor in the right lobe, and his α-fetoprotein level is greater than 100,000 ng/mL. A biopsy confirms hepatoblastoma with mixed epithelial and mesenchymal elements. The surgical team deems the tumor unresectable at diagnosis. What is the most appropriate initial management step?
A 16-year-old boy, who is notably taller than his peers, presents with a three-month history of persistent pain and swelling in his distal femur. Radiographs show a “sunburst” pattern consistent with osteosarcoma. Several genetic conditions are known to predispose patients to osteosarcoma. Which of the following conditions is NOT listed as a predisposing factor for this patient’s suspected diagnosis?
A 6-year-old boy with a confirmed diagnosis of multi-system Langerhans cell histiocytosis involving skin and bone fails to respond to initial therapy with vinblastine and corticosteroids. The care team is considering second-line therapies and sends tumor tissue for molecular analysis. The results reveal a BRAF V600E pathogenic variant. Which of the following therapeutic agents directly targets the identified aberrant signaling pathway in this patient’s disease?
