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Chondrosarcoma is a cancer of cartilage-forming cells in bone. It is nearly immune to chemotherapy and radiotherapy, so complete surgery is the treatment, with proton or carbon-ion beams for skull base and spine tumours that cannot be fully removed. Half of conventional tumours carry an IDH mutation, and the IDH1 blocker ivosidenib is in a phase 3 trial.
Chondrosarcoma arises in bone from cartilage-producing cells and is graded 1 to 3; grade 1 tumours of the limbs were renamed atypical cartilaginous tumours in 2013 because they almost never metastasise, while grade 3 and dedifferentiated tumours spread early to the lungs. About half of conventional and most dedifferentiated tumours carry a mutation in IDH1 or IDH2, the same enzymes mutated in glioma and acute myeloid leukaemia, and mesenchymal chondrosarcoma carries a HEY1-NCOA2 fusion. The tumour's low cell turnover, poor blood supply and abundant cartilage matrix make it resistant to conventional chemotherapy and to standard-dose radiotherapy.
Surgery is the only established curative treatment: curettage for atypical cartilaginous tumours of the limbs, wide resection for higher grades and for all pelvic and axial tumours, with limb-salvage reconstruction where feasible. Skull base and spinal tumours that cannot be resected completely are treated with high-dose proton or carbon-ion radiotherapy, which achieves local control in most cases. Dedifferentiated chondrosarcoma is treated like osteosarcoma with doxorubicin and cisplatin-based chemotherapy despite uncertain benefit, and mesenchymal chondrosarcoma with Ewing-type regimens.
The 2011 discovery of IDH mutations made chondrosarcoma a targetable disease. In the phase 1 study of ivosidenib in IDH1-mutant chondrosarcoma, most patients had stable disease as their best response with durable control in some, and the placebo-controlled phase 3 CHONQUER trial (NCT06127407) in conventional chondrosarcoma is now recruiting. Immunotherapy has shown occasional responses in dedifferentiated tumours, and there is no approved systemic therapy for any form.
The commonest primary bone sarcoma of adults, typically diagnosed between 40 and 70 in the pelvis, proximal femur, shoulder girdle and ribs; low-grade tumours are cured by surgery alone, while dedifferentiated tumours are among the most lethal of all sarcomas.
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.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Intralesional curettage with local adjuvant, or observation of asymptomatic lesions; no chemotherapy or radiotherapy.
Wide en bloc resection with limb-salvage or pelvic reconstruction in a bone sarcoma centre; radiotherapy only for positive margins.
Maximal safe surgery followed by high-dose proton or carbon-ion radiotherapy.
Surgery plus osteosarcoma-type (doxorubicin, cisplatin) or Ewing-type (doxorubicin, ifosfamide) chemotherapy, benefit uncertain.
Ivosidenib within the CHONQUER phase 3 trial or compassionate access; no approved systemic therapy.
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The chondrosarcoma page's recommendations for curettage of low-grade limb lesions, wide resection for higher grades and particle therapy for skull base tumours follow this guideline.
IDH mutation testing helps distinguish chondrosarcoma from chondroblastic osteosarcoma and other mimics, and IDH inhibitors are being tested in advanced chondrosarcoma.
Query for this cancer: (TITLE:"Chondrosarcoma" OR ABSTRACT:"Chondrosarcoma" OR TITLE:"Cartilage sarcoma" OR ABSTRACT:"Cartilage sarcoma" OR TITLE:"Atypical cartilaginous tumour grade 1, limbs" OR ABSTRACT:"Atypical cartilaginous tumour grade 1, limbs" OR TITLE:"Dedifferentiated chondrosarcoma" OR ABSTRACT:"Dedifferentiated chondrosarcoma" OR TITLE:"Mesenchymal chondrosarcoma" OR ABSTRACT:"Mesenchymal chondrosarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Chondrosarcoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Fever, cough or breathlessness, rapid weight gain or swelling, bone pain, low blood pressure or reduced urine; the labels say to start steroids and monitor at the first suspicion, and the syndrome has been fatal.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
See all on the product pages:CisplatinDoxorubicinIfosfamideIvosidenib·Printable cards in the navigator
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