Nazim Demircan, Associate Professor at Marmara university, shared on LinkedIn:
“Rare cancers may be individually uncommon, yet collectively they represent one of oncology’s most complex challenges.
Limited patient populations, biological heterogeneity, and the difficulty of generating high-level evidence continue to make progress in sarcomas and rare cancers particularly challenging.
This is precisely why every meaningful trial in this space matters.
With ESMO 2026 approaching, here are 9 sarcoma and rare cancer studies on my radar:
- TANGENT | TGCT
Phase III emactuzumab, evaluating CSF-1R-targeted therapy in a rare, locally aggressive tumor - RINGSIDE | Desmoid tumor
Phase III varegacestat, further defining the role of γ-secretase inhibition in desmoid disease - Zolucatetide | Desmoid tumor
An emerging therapeutic approach with updated early-phase data in the Sarcoma Proffered Paper session - NB003 | GIST
A next-generation selective KIT inhibitor with first-in-human data in advanced GIST - Darovasertib program | Uveal melanoma
Targeted therapy across both primary and metastatic disease – including its potential move into earlier treatment - ²¹²Pb-VMT-α-NET | Neuroendocrine tumors
Targeted alpha-particle therapy in advanced SSTR2-positive NETs – an exciting direction for radiopharmaceutical therapy - MGC026 | Soft-tissue sarcoma
A B7-H3-directed ADC moving into tumor-specific evaluation in STS - BOT + BAL | Advanced sarcoma
Real-world experience with a novel immunotherapy combination in heavily pretreated disease - EFTISARC-NEO | Localized soft-tissue sarcoma
Further insights from neoadjuvant efti + pembrolizumab + radiotherapy, now focusing on patient-reported quality of life
What stands out to me is not just any single study, but the breadth of therapeutic approaches now reaching rare cancers – targeted therapies, ADCs, immunotherapy, radiopharmaceuticals, and new strategies in diseases where conducting definitive trials has historically been difficult.
Looking forward to seeing the full data and discussing what they may mean for patients with rare cancers in Madrid.”

You can also read: OncoDaily Grand Rounds at ESMO 2026: All You Need to Know About Sarcoma
