cancersCancer

Chondrosarcoma

aka Cartilage sarcoma, Atypical cartilaginous tumour (grade 1, limbs), Dedifferentiated chondrosarcoma, Mesenchymal chondrosarcoma

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.

Part ofSarcomas (soft tissue, bone, GIST)

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.

State of the art

Particle therapy gives durable local control of skull base chondrosarcoma that surgery alone cannot achieve.
IDH1 inhibition with ivosidenib produced durable stable disease in the phase 1 study and is being tested against placebo in the CHONQUER phase 3 trial.
Grading by an expert bone pathologist, not imaging alone, decides between curettage and wide resection.

Red cards

From the labels and guidelines behind the standard of care. Your team's thresholds win.

Emergency services now
Bleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Cisplatin Doxorubicin Ifosfamide source
Emergency services now
Differentiation syndrome
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.
Ivosidenib source
Check before combining
Food and drink: Doxorubicin
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Doxorubicin source
Check before combining
Heart rhythm (QT): Ivosidenib
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
Ivosidenib source
Check before combining
Kidneys: Cisplatin
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Cisplatin source
Check before combining
Liver: Doxorubicin
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Doxorubicin source

See all on the product pages:Cisplatin Doxorubicin Ifosfamide Ivosidenib·Printable cards in the navigator

Anatomy and lymph node drainage

Bone and soft tissue (limb cross-section): subsites and node stations · wireframe schematic, not to scale

Organ atlas →

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.

Subsites

Metaphysis, near the growth plate (osteosarcoma)Conventional central chondrosarcoma, grades 1 to 3 (IDH1/IDH2-mutant in about half) · Dedifferentiated chondrosarcoma (high-grade component; treated like osteosarcoma)
Shaft (Ewing sarcoma)Mesenchymal chondrosarcoma (HEY1-NCOA2; Ewing-type chemotherapy)
Deep soft tissue compartmentConventional central chondrosarcoma, grades 1 to 3 (IDH1/IDH2-mutant in about half) · Atypical cartilaginous tumour (grade 1 of the limbs; curettage) · Peripheral (secondary) chondrosarcoma arising in osteochondroma · Dedifferentiated chondrosarcoma (high-grade component; treated like osteosarcoma) · Mesenchymal chondrosarcoma (HEY1-NCOA2; Ewing-type chemotherapy) · Clear cell chondrosarcoma (epiphyseal, low grade) · Skull base and spinal chondrosarcoma (particle therapy)
Skeletal muscle (rhabdomyosarcoma)
Neurovascular bundle (limb salvage decision)

Regional lymph nodes

Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.

Same organ: 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)

Who gets it and what has changed

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.

Group

sarcoma

What is being done about this

Finding cancer earlier

and how the field plans to fix it →

Available now

MRIStandard of care

In trials

Nothing recorded yet.

Ideas and roadmaps

Nothing recorded yet.

Also on OnCo: Symptoms and red flags · Early detection roadmap.

Cases by country

Cases by country · GLOBOCAN 2022

All countries →

No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.

Standard of care

5top

Lines of therapy by subgroup →Staging and risk scores →

Decisions you may face

5 with more than one option · full decision page →

One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.

Atypical cartilaginous tumour of the limbs3 Conventional grade 2 to 3, resectable2 Skull base and spine, incompletely resectable3 Dedifferentiated and mesenchymal3 Advanced IDH1-mutant conventional2

Atypical cartilaginous tumour of the limbs

Intralesional curettage with local adjuvant, or observation of asymptomatic lesions; no chemotherapy or radiotherapy.

Limb-salvage surgery and endoprosthetic reconstruction Active surveillance MRI

Conventional grade 2 to 3, resectable

Wide en bloc resection with limb-salvage or pelvic reconstruction in a bone sarcoma centre; radiotherapy only for positive margins.

Limb-salvage surgery and endoprosthetic reconstruction IMRT / IGRT (modern external beam)

Skull base and spine, incompletely resectable

Maximal safe surgery followed by high-dose proton or carbon-ion radiotherapy.

Proton therapy Carbon-ion therapy IMRT / IGRT (modern external beam)

Dedifferentiated and mesenchymal

Surgery plus osteosarcoma-type (doxorubicin, cisplatin) or Ewing-type (doxorubicin, ifosfamide) chemotherapy, benefit uncertain.

DoxorubicinCisplatinIfosfamide

Advanced IDH1-mutant conventional

Ivosidenib within the CHONQUER phase 3 trial or compassionate access; no approved systemic therapy.

IvosidenibIvosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen IDH inhibitors

Subtypes & biomarkers

top

Subtypes

Conventional central chondrosarcoma, grades 1 to 3 (IDH1/IDH2-mutant in about half)
Atypical cartilaginous tumour (grade 1 of the limbs; curettage)
Peripheral (secondary) chondrosarcoma arising in osteochondroma
Dedifferentiated chondrosarcoma (high-grade component; treated like osteosarcoma)
Mesenchymal chondrosarcoma (HEY1-NCOA2; Ewing-type chemotherapy)
Clear cell chondrosarcoma (epiphyseal, low grade)
Skull base and spinal chondrosarcoma (particle therapy)

Biomarkers clinicians test

IDH1 or IDH2 mutation (ivosidenib eligibility)
Histological grade 1 to 3 and dedifferentiation
HEY1-NCOA2 fusion (mesenchymal)
COL2A1 mutations
Site (limb versus pelvis and axial skeleton)

How often this target appears

History

5top

1943Lichtenstein and Jaffe separate chondrosarcoma from osteosarcoma
2011IDH1 and IDH2 mutations found in chondrosarcoma and enchondroma
IDH1 / IDH2
2013WHO renames grade 1 limb tumours atypical cartilaginous tumours
2020Phase 1 ivosidenib in IDH1-mutant chondrosarcoma reports durable stable disease
Ivosidenib
2023CHONQUER phase 3 trial of ivosidenib opens
Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen

What changed

6top

Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.

All 6 changes by month →

FollowWatch this cancer and Saved shows what changed since you last looked.Follow by feed

In development

4top

What is in development for Chondrosarcoma, drawn from the whole corpus: 1 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.

Trials under way · 1

Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen · phase 3 · Servier Bio-Innovation LLC

Open problems and what is being done

No systemic therapy has ever been proven to help.
Dedifferentiated chondrosarcoma kills most patients within two years.
Distinguishing enchondroma from grade 1 chondrosarcoma remains unreliable.

Trials

top

Trials recruiting now

Recruiting trials near you · live from ClinicalTrials.gov

Chondrosarcoma

condition: Chondrosarcoma

Open on ClinicalTrials.gov →

within

Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.

Landmark trials

Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen

Expert centres

top

Where the expertise is

Expert centres

120 centres in 42 countries · 0 with a trial for this cancer on record · second-opinion finder →

Choose a region from the globe in the header to mark centres near you.

120 centres


MD Anderson Cancer Center Houston · cancer center	United States	NCI comprehensive	6,724	95,007	CAR-NK & CAR-macrophage Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems Gamma Knife +2	#2
Samsung Medical Center Seoul · hospital	South Korea	none recorded	1,312	17,172	Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems Gamma Knife Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)	#3
Mayo Clinic Rochester, MN · hospital	United States	NCI comprehensive	4,511	44,748	Carbon-ion therapy Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems Gamma Knife +1	#5
Johns Hopkins Hospital / Sidney Kimmel Comprehensive Cancer Center Baltimore · cancer center	United States	NCI comprehensive	2,955	41,449	Multi-cancer early detection (MCED)Liquid biopsy (ctDNA)Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems +2	#10
Charité Universitätsmedizin Berlin Berlin · university	Germany	none recorded	1,563	17,749	Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems CyberKnife robotic radiosurgery Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)	#12
Massachusetts General Hospital Cancer Center Boston · hospital	United States	NCI comprehensive	3,582	54,857	Proton therapy Small-molecule kinase inhibitors Proton therapy machines: cyclotrons, synchrotrons and single-room systems Gamma Knife +1	#16
Heidelberg University Hospital / NCT / DKFZ Heidelberg · cancer center	Germany	none recorded	3,456	45,745	FAPI PET Targeted alpha therapy Carbon-ion therapy DNA methylation profiling +5	#18
Cleveland Clinic Taussig Cancer Institute Cleveland · hospital	United States	NCI comprehensive	2,264	29,412	Off-the-shelf cancer vaccines Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems Gamma Knife +1	#20
Institut Curie Paris · cancer center	France	none recorded	1,065	15,111	Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems	#21
Severance Hospital, Yonsei University Seoul · hospital	South Korea	none recorded	464	3,248	Carbon-ion therapy Robotic & minimally invasive surgery Carbon-ion synchrotrons and facilities Gamma Knife +1	#22
The Christie NHS Foundation Trust Manchester · cancer center	United Kingdom	none recorded	104	2,145	Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems MR-guided adaptive radiotherapy TomoTherapy and Radixact (helical radiotherapy)	#23
Fudan University Shanghai Cancer Center Shanghai · cancer center	China	none recorded	1,678	18,354	TROP2 PET Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems Carbon-ion therapy +1	#55
Abramson Cancer Center, University of Pennsylvania Philadelphia · cancer center	United States	NCI comprehensive	3,148	54,267	CAR-T cell therapy In vivo CAR-T PARP PET Personalised neoantigen (mRNA) vaccines +2	-
University of Michigan Rogel Cancer Center Ann Arbor, MI · cancer center	United States	NCI comprehensive	2,991	29,686	PROTACs & molecular glues (targeted protein degradation)Proton therapy Proton therapy machines: cyclotrons, synchrotrons and single-room systems	-
University College London Hospitals / UCL Cancer Institute London · hospital	United Kingdom	none recorded	2,376	28,940	Proton therapy CAR-T cell therapy Liquid biopsy (ctDNA)Whole-exome & whole-genome sequencing +5	-

(showing the first 15)

These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Chondrosarcoma but is thinly recorded here will look small.

Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost. The second-opinion finder explains how referral works in your country.

Questions to ask

top

Bring to your appointment

Questions to ask your oncologist about Chondrosarcoma

Generated from this cancer's standard of care, biomarkers, and pipeline · 17 questions

Newly diagnosed

What is my exact diagnosis, stage, and grade, and which tests established them?
Why: Everything else follows from an accurate stage and subtype.
Which biomarkers have been tested on my tumour (for example IDH1 or IDH2 mutation, Histological grade 1 to 3 and dedifferentiation, HEY1-NCOA2 fusion, COL2A1 mutations, Site), and what were the results?
Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
Which subtype is my cancer, and does that change the recommended treatment?
Why: Recognised subtypes for this cancer include Conventional central chondrosarcoma, grades 1 to 3, Atypical cartilaginous tumour, Peripheralchondrosarcoma arising in osteochondroma.
Is germline (inherited) genetic testing recommended for me or my family?
Why: Inherited variants can change treatment and matter for relatives.

Atypical cartilaginous tumour of the limbs

For my situation (atypical cartilaginous tumour of the limbs), which of the standard options do you recommend and why?
Why: Guideline options include: Intralesional curettage with local adjuvant, or observation of asymptomatic lesions; no chemotherapy or radiotherapy.

Conventional grade 2 to 3, resectable

For my situation (conventional grade 2 to 3, resectable), which of the standard options do you recommend and why?
Why: Guideline options include: Wide en bloc resection with limb-salvage or pelvic reconstruction in a bone sarcoma centre; radiotherapy only for positive margins.

Skull base and spine, incompletely resectable

For my situation (skull base and spine, incompletely resectable), which of the standard options do you recommend and why?
Why: Guideline options include: Maximal safe surgery followed by high-dose proton or carbon-ion radiotherapy.

Dedifferentiated and mesenchymal

For my situation (dedifferentiated and mesenchymal), which of the standard options do you recommend and why?
Why: Guideline options include: Surgery plus osteosarcoma-type (doxorubicin, cisplatin) or Ewing-type (doxorubicin, ifosfamide) chemotherapy, benefit uncertain.
Am I a candidate for Doxorubicin, Cisplatin, Ifosfamide, and what side effects should I expect?
Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Advanced IDH1-mutant conventional

For my situation (advanced idh1-mutant conventional), which of the standard options do you recommend and why?
Why: Guideline options include: Ivosidenib within the CHONQUER phase 3 trial or compassionate access; no approved systemic therapy.
Am I a candidate for Ivosidenib, and what side effects should I expect?
Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
How do the results of Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen apply to someone like me?
Why: Trial populations differ from individual patients; ask how closely you match.

Any stage

Are there clinical trials I could join, for example of Ivosidenib, Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment Regimen, IDH inhibitors, Carbon-ion therapy?
Why: Trials are how the next standard of care is set; asking early keeps options open.
Would a second opinion at a high-volume centre change anything, and can you help arrange it?
Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
Why: Supportive care improves quality of life and helps patients complete treatment.
I read that “No systemic therapy has ever been proven to help”. How does that affect my plan?
Why: Open problems are where trials and second opinions matter most.
I read that “Dedifferentiated chondrosarcoma kills most patients within two years”. How does that affect my plan?
Why: Open problems are where trials and second opinions matter most.