Osteosarcoma
Osteosarcoma is the most common bone cancer, mostly in teenagers. Chemotherapy plus surgery cures about two-thirds when it has not spread; because no new drug has beaten that chemotherapy in a large trial in 30 years, the next gains are being sought in cellular therapy against GD2, HER2 and B7-H3.
Overview
Osteosarcoma is a high-grade bone sarcoma with chaotic genomes (TP53 and RB1 loss, chromothripsis, no recurrent targetable driver), arising in the metaphyses of long bones during growth spurts and in older adults after Paget disease or radiation. Germline predisposition (Li-Fraumeni, hereditary retinoblastoma, Rothmund-Thomson) accounts for a meaningful fraction.
Standard therapy since the 1980s is neoadjuvant MAP (high-dose methotrexate, doxorubicin, cisplatin), limb-salvage surgery, and adjuvant MAP; histologic response (≥90% necrosis) is prognostic but intensifying therapy for poor responders (EURAMOS-1: adding ifosfamide-etoposide) did not help, nor did interferon maintenance. Mifamurtide (liposomal MTP-PE) is approved in the EU (INT-0133) but not in the US. Lung metastases are resected whenever possible. Relapsed disease has ~20% survival; multikinase inhibitors (regorafenib in SARC024/REGOBONE, cabozantinib in CABONE, sorafenib) give short PFS gains. Novel approaches: GD2- and HER2-directed CAR-T, B7-H3 ADCs, radiopharmaceuticals (Ra-223, Sm-153), and biology from canine osteosarcoma.
State of the art
- MAP chemotherapy, unchanged since the 1980s, remains the standard; EURAMOS-1 (2,260 patients) closed the door on intensification.
- Limb salvage is possible in >90% with expandable prostheses for growing children.
- Multikinase inhibitors are the only agents with randomised evidence at relapse, and the gain is months.
- Immunotherapy has largely failed (checkpoint inhibitors inactive); cellular therapy against GD2/HER2/B7-H3 is the active frontier.
Anatomy and lymph node drainage
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)
- Deep soft tissue compartment
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
- Metaphysis, near the growth plate (osteosarcoma)Conventional high-grade (osteoblastic, chondroblastic, fibroblastic) · Telangiectatic · Low-grade central and parosteal (surgery alone) · Periosteal (intermediate grade)
- Shaft (Ewing sarcoma)
- Deep soft tissue compartment
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Preclinical models
5 cell lines, 1 mouse models and 3 repositories are listed for this cancer.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- The most common primary bone cancer; ~1,000 cases per year in the US with peaks in adolescence and over 60 (Paget disease, radiation); 5-year survival ~70% localised, ~25% metastatic.
Neoadjuvant MAP (methotrexate, doxorubicin, cisplatin) ×2 cycles, limb-salvage resection with wide margins (amputation if required), adjuvant MAP to ~29 weeks; mifamurtide added in EU.
Same chemotherapy with resection of all metastases (thoracotomy) when feasible; survival ~25-30%.
Surgical resection of recurrence; ifosfamide ± etoposide, gemcitabine-docetaxel; regorafenib or cabozantinib; clinical trials (CAR-T, ADCs).
Carbon-ion or proton radiotherapy for craniofacial and pelvic tumours; Sm-153 or Ra-223 for bone-forming metastases (investigational).
Subtypes & biomarkers
top- Conventional high-grade (osteoblastic, chondroblastic, fibroblastic)
- Telangiectatic
- Small cell
- Low-grade central and parosteal (surgery alone)
- Periosteal (intermediate grade)
- Secondary (Paget, post-radiation)
- Histologic necrosis after neoadjuvant chemotherapy (≥90% good response)
- Alkaline phosphatase and LDH
- Metastases at diagnosis (lung, bone)
- Germline TP53 / RB1 / RECQL4
- GD2, HER2, B7-H3 expression (trial eligibility)
- ctDNA copy-number burden (prognostic, emerging)
How often this target appears
- 1970Amputation alone cures <20%; lung metastases the rule
- 1972High-dose methotrexate with leucovorin rescue (Jaffe) and adriamycin (Cortes) show activity
- 1979Rosen's T-10: neoadjuvant chemotherapy and limb salvage
- 1986Randomised proof that adjuvant chemotherapy cures (Link, NEJM; MIOS)
- 2008INT-0133: mifamurtide improves overall survival; EU approval 2009
- 2016EURAMOS-1: no benefit from intensifying for poor responders or interferon for good responders
- 2019Regorafenib (SARC024, REGOBONE) and cabozantinib (CABONE) show activity at relapse
Dated changes read from the records linked to this indication: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 8 changes by month →- 2026-09-08This recordOsteosarcomaFacts on this page last checked
When this page itself was last checked or edited.
- 2019MilestoneCabozantinibRegorafenib (SARC024, REGOBONE) and cabozantinib (CABONE) show activity at relapse
A milestone in how this cancer is treated.
- 2016MilestoneOsteosarcomaEURAMOS-1: no benefit from intensifying for poor responders or interferon for good responders
A milestone in how this cancer is treated.
- 2008MilestoneOsteosarcomaINT-0133: mifamurtide improves overall survival; EU approval 2009
A milestone in how this cancer is treated.
- 1986MilestoneOsteosarcomaRandomised proof that adjuvant chemotherapy cures (Link, NEJM; MIOS)
A milestone in how this cancer is treated.
- 1979MilestoneOsteosarcomaRosen's T-10: neoadjuvant chemotherapy and limb salvage
A milestone in how this cancer is treated.
What is in development for Osteosarcoma, drawn from the whole corpus: 3 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Technologies being tested · 1
Trials under way · 1
- Lu-TARGO (177Lu-TARGeted Osteosarcoma Therapy) · phase 1/2 · Lantheus Medical Imaging
Targets under investigation · 1
Open problems and what is being done
No survival improvement since the 1980s; metastatic and relapsed disease ~20-30% survival.
What is being done about thisAdvanced and metastatic diseaseAvailable now- DocetaxelApproved
- Radioligand therapy (beta emitters)Approved
- Radium-223 dichlorideApproved
In trialsNothing recorded yet.
Ideas and roadmapsNothing recorded yet.
Also on Nuclide: Indications.
No recurrent druggable driver; genomic chaos.
Chemotherapy toxicity: cardiotoxicity, hearing loss, infertility, second cancers.
Rarity fragments trials; international cooperation (EURAMOS) needed for every question.
Trials
topTrials recruiting now
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Landmark trials
Direct links plus the targets, companies, and technologies of this cancer's products.
isotopes
2technologies
2targets
2drugs
3companies
1trials
1Latest papers
topQuery for this indication: (TITLE:"Osteosarcoma" OR ABSTRACT:"Osteosarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Osteosarcoma, not a curated reading list.
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- IdeaAlpha radioligands after ADC failure