Table 1.
Characteristics of common pediatric solid tumors.
| Disease | Incidence | Avg. age of onset | Clinical features | Genetic causes | Current treatment | Citations |
|---|---|---|---|---|---|---|
| Brain tumors | MB: Approx. 1 in 170,000 pHGG: 1 in 125,000 |
MB: 2–6 years of age pHGG: 0–14 years of age |
Increased intracranial pressure | MB: chromosomal abnormalities, structural variants (differs for each of the 4 subgroups) pHGG: homozygous inactivation of p53 (TP53) and histone 3.3 (H3F3A), amplification of receptor tyrosine kinase PDGFRA |
Surgery, combination chemotherapy (e.g., vincristine, cyclophosphamide, cisplatin, carboplatin, etoposide), radiation therapy | (3–7) |
| Neuroblastoma | Approx. 1 in 17,000 | Between 18 and 22 months | Abdominal mass | MYCN amplification, chromosomal segment alterations (e.g., deletion at 1p, 11q, and gain at 17q), mutations in anaplastic lymphoma kinase (ALK) | Surgical resection, induction chemotherapy (cisplatin, cyclophosphamide, vincristine, doxorubicin, etoposide), radiation therapy | (3, 8, 9) |
| Wilms’ tumor | 1 in 10,000 | >80% of children diagnosed before the age of 5 | Asymptomatic abdominal mass | Chromosomal abnormalities (e.g., gene deletions at 11p13 and 11p15), mutations in the WT1 gene | Nephrectomy, chemotherapy (vincristine and dactinomycin), radiation therapy | (3, 10) |
| Bone sarcomas | EwS: Approx. 1 in 650,000 OS: Approx. 1 in 220,000 (children and young adults) |
EwS: 10–19 years of age OS: 10–14 years of age |
EwS: pain and swelling in affected bone OS: localized pain or swelling |
EwS: chromosomal translocation between a member of FET family of RNA-binding proteins and a member of ETS family of TFs OS: aneuploidy, chromothripsis, kataegis, mutations in TP53 |
Induction chemotherapy (EwS: vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide OS: doxorubicin, cisplatin or carboplatin, methotrexate), surgery |
(3, 11–18) |
| Rhabdomyosarcoma | Approx. 1 in 230,000 | Approx. 70% diagnosed before the age of 10 | Soft tissue mass found in the extremities or head/neck | Alveolar RMS (ARMS): balanced chromosomal translocations between chromosome 1 or 2 and chromosome 13, PAX3::FOXO1 and PAX7::FOXO1 gene fusions Embryonic RMS (ERMS): chromosomal gains and losses, alterations of RAS family genes |
Surgery, chemotherapy (vincristine, actinomycin D, cyclophosphamide), radiation therapy | (3, 19–22) |
MB, medulloblastoma; pHGG, pediatric high-grade glioma; EwS, Ewing sarcoma; OS, osteosarcoma; TFs, transcription factors.