sarcoma

NCI oncologist who led the trial that showed atezolizumab produces lasting responses in alveolar soft part sarcoma, the first approved treatment for this rare cancer.

Boston sarcoma doctor who first showed that mTOR inhibitors shrink perivascular epithelioid cell tumours and then led AMPECT, the trial that made nab-sirolimus the first approved treatment for these rare cancers.

Bordeaux oncologist who led CABONE, the trial that showed cabozantinib shrinks or stabilises osteosarcoma and Ewing sarcoma after standard treatment has failed.

Chordoma is a slow-growing bone cancer (a sarcoma) of the skull base and spine that arises from leftover embryonic notochord cells. Complete surgery followed by high-dose proton or carbon-ion radiotherapy controls most tumours, and the whole disease depends on a single transcription factor, brachyury, which vaccines and degraders are now trying to hit.

Desmoid tumours are locally aggressive growths of fibroblast-like cells, classed with soft-tissue sarcomas, driven by WNT mutations, that never spread to distant organs but can invade nerves, bowel and muscle. Many stop growing or shrink on their own, so watching first is standard; if they progress, the gamma-secretase inhibitor nirogacestat, approved in 2023, shrinks tumours and relieves pain.

Epithelioid sarcoma is a rare soft tissue cancer that has lost a gene brake called SMARCB1, leaving it dependent on the enzyme EZH2. Surgery cures localised tumours. The EZH2 inhibitor tazemetostat, the first epigenetic drug approved for a solid tumour, was approved in 2020 and withdrawn worldwide in March 2026 after secondary blood cancers in a lymphoma trial, so there is no targeted drug today.

Ewing sarcoma is a bone and soft-tissue cancer of teenagers driven by a single fusion gene, EWSR1-FLI1. Intensive chemotherapy with surgery or radiation cures most localised cases; disease that has spread at diagnosis, and relapse, remain hard to treat, and no drug against the fusion protein itself has yet succeeded.

Marseille oncologist who led REGOBONE, the trial that showed regorafenib delays progression in metastatic osteosarcoma and other bone sarcomas.

GIST is a sarcoma of the gut wall driven almost always by a KIT or PDGFRA mutation. It was the proof that a pill can control a solid tumour: imatinib turned a median survival of about a year into one of eight years or more, and the mutation now dictates which drug to use.

Led the imatinib trial in GIST that proved a targeted drug could melt away a solid tumour, and every GIST kinase inhibitor since.

IMT is a rare tumour, grouped with the sarcomas, of spindle cells mixed with inflammatory cells, most often in the lung or abdomen of children and young adults. Surgery cures most, and about half carry an ALK gene fusion, so the ALK-blocking pill crizotinib is approved for those that cannot be removed, one of the first targeted approvals for a childhood solid tumour.

Polish medical oncologist and sarcoma specialist who serves as President of the Organisation of European Cancer Institutes.

Medical oncologist and sarcoma specialist who directs Institut Bergonié, the regional cancer centre for Nouvelle-Aquitaine in Bordeaux.

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.

Paris oncologist who led LMS-04, the trial that showed doxorubicin plus trabectedin lengthens survival in advanced leiomyosarcoma compared with doxorubicin alone.

Leuven oncologist who led the trial that showed eribulin lengthens survival in advanced liposarcoma, the basis of the drug's approval for that disease.

Polish surgical oncologist who led the pooled EORTC and SWOG trials showing that imatinib shrinks dermatofibrosarcoma protuberans, a rare skin sarcoma driven by a PDGFB fusion.

A childhood soft-tissue sarcoma, a cancer of muscle-like cells found anywhere from the eye socket to the bladder. Most children are cured with chemotherapy, surgery and radiation, and a fusion gene (PAX-FOXO1) now decides how intensively to treat.

London sarcoma specialist who led TAPPAS, the trial that tested the anti-angiogenic protein TRC105 with pazopanib in angiosarcoma, and who has led many international sarcoma trials.

Led SPEARHEAD-1, the trial that made afami-cel the first engineered TCR T-cell therapy approved for a solid tumour.

German sarcoma specialist who was lead author of INTRIGUE, the trial that compared ripretinib with sunitinib as second-line treatment for GIST.

Italian oncologist who leads histology-specific trials in ultra-rare sarcomas and the Connective Tissue Oncology Society.

Paris surgeon who championed compartmental resection for retroperitoneal sarcoma and led STRASS, the trial that showed preoperative radiotherapy does not improve abdominal recurrence-free survival overall.

TGCT is a benign but destructive tumour of the joint lining, classed with soft-tissue sarcomas, in which a few cells carrying a CSF1 gene fusion recruit a crowd of normal immune cells that eat away at the joint. Surgery cures most localised cases, and for diffuse or recurrent disease two pills that block the CSF1 receptor, pexidartinib and vimseltinib, shrink tumours and restore joint function.

Paediatric oncologist from University Hospital Essen who presides over SIOP Europe and has led international Ewing sarcoma trials.

Uterine sarcomas are rare cancers of the muscle and supporting tissue of the womb, distinct from the far commoner endometrial cancer. Removing the uterus intact is the main treatment and is followed by observation for stage I disease; low-grade stromal sarcomas respond to hormone-blocking pills, while advanced leiomyosarcoma is treated with doxorubicin and trabectedin.

Vascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to the slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE depend on growth signals that the mTOR blocker sirolimus quiets, and EHE without symptoms is watched.

Sarcoma oncologist who led ANNOUNCE and the pexidartinib trial that produced the first drug for tenosynovial giant cell tumour.

Led PALETTE, the trial that made pazopanib the first targeted drug for soft-tissue sarcoma.