Onco Wiki / Disease

Osteosarcoma

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-OSTEOSARCOMA
TypeDisease
Aliases
Conventional osteosarcomaOSOsteogenic sarcomaОстеосаркома
Statusreviewed 2026-05-19 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-ESMO-BONE-SARCOMA SRC-NCCN-BONE-SARCOMA SRC-NCCN-PEDIATRIC-SARCOMA

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology9180/3
icd_o_3_topography["C40", "C41"]
icd_10C40, C41
who_classificationWHO Soft Tissue and Bone Tumours 5th ed (2020): Osteosarcoma — conventional (high-grade intramedullary), telangiectatic, small cell, low-grade central, parosteal, periosteal, high-grade surface, extraskeletal

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Notes

Osteosarcoma is the most common primary malignant bone tumor in children + adolescents. Bimodal age distribution: adolescent growth-spurt peak (10–20y; long-bone metaphyses) and elderly secondary peak (>60y; predominantly Paget disease-associated or post-radiation). Standard-of-care multi-modal treatment (full Indications pending): neoadjuvant chemotherapy → en-bloc surgical resection (limb-salvage preferred; amputation reserved for unresectable / vascular-bundle invasion) → adjuvant chemotherapy. MAP regimen (high-dose methotrexate, doxorubicin, cisplatin) is the global standard from EURAMOS-1 (good-responder arm). Histological necrosis ≥90% at resection predicts favorable outcome; poor responders (<90% necrosis) do NOT benefit from intensification per EURAMOS-1. Genetic relationships drive prevention-workstream surveillance: - Li-Fraumeni (germline TP53): osteosarcoma is the second-most common cancer in this syndrome; Toronto-protocol annual whole-body MRI + bone surveillance recommended. v0.2-B pilot. - Hereditary retinoblastoma (germline RB1) survivors: lifetime osteosarcoma risk markedly elevated, particularly within prior RT field. Long-term surveillance post-retinoblastoma...

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