The Spanish Society of Medical Oncology (SEOM) emphasizes that the management of patients with sarcoma "must be carried out in multidisciplinary committees with experts in this pathology," due to "the infrequency and complexity" of these tumors.
According to the scientific society, "the treatment of these tumors must be multidisciplinary, being essential in localized disease, and should be evaluated by a committee of experts to decide the most appropriate diagnostic and therapeutic plan in each case." Furthermore, it recalls that "in recent years it has been possible to demonstrate that the survival of sarcoma patients is higher in centers expert in this pathology."
SEOM underlines that "Spain has Centers, Services, and Reference Units (CSUR) for sarcomas" and warns that "small therapeutic advances" "could have little relevance" if analyzed separately. However, it highlights that, "accumulated among themselves, they have led to a notable change in the prognosis and quality of life for many patients in many cases."
"In Oncology, every small advance counts, and it is the sum of these advances that allows us to obtain results and move forward in the prognosis of the disease," states the entity, which emphasizes that "sarcomas and, in particular, gastrointestinal stromal tumors (GIST) - technically soft tissue sarcomas but considered separately due to their special diagnostic and therapeutic characteristics - have experienced significant advances in their diagnosis and treatment in recent years."
Progress in GIST tumors
In relation to GIST, SEOM indicates that these tumors "have gone from being poorly identified and resistant to conventional chemotherapy in less than 10 years to becoming a well-understood biological and histopathological entity, distinguishable from other sarcomas." It adds that "the three drugs approved for metastatic disease - imatinib, sunitinib, and regorafenib - have increased survival from 12 months to more than five years."
The society recalls that imatinib "is also used for three years in localized high-risk disease after demonstrating increased survival" and that "new drugs continue to be approved." Among them, it mentions "ripretinib, a drug effective in GIST patients once they have progressed on all standard treatments," and "avapritinib, the first drug active against the PDGFRA D842V mutation."
All these advances are occurring in a very heterogeneous group of tumors that commemorates its International Day this Monday, July 13, and which stars in the SEOM campaign "In Oncology, every ADVANCE is written in capital letters". The organization takes the opportunity to recall that there are two main groups of sarcomas, which can originate in different tissues and in various locations of the body.
Soft tissue sarcomas (STS) originate in tissues such as muscles, tendons, or fat and account for about 1 percent of all malignant tumors, being responsible for 2 percent of cancer deaths. Bone sarcomas, on the other hand, are located in the skeleton and mainly affect children and adolescents, with an approximate incidence of one new case per year per 100,000 inhabitants.
More than 150 types of sarcomas and innovative therapies
GISTs are technically soft tissue sarcomas, although they are considered separately due to their diagnostic and therapeutic particularities. The World Health Organization (WHO) currently recognizes more than 150 varieties of sarcomas, of which approximately 60 percent are located in the extremities, 30 percent in the trunk or abdomen, and 10 percent in the head or neck.
SEOM details "two advances in synovial sarcoma with cell therapy and in leiomyosarcoma". The "most outstanding" is "afamitresgene autoleucel, an innovative T-cell receptor (TCR) therapy that in 2026 achieved full approval from the U.S. Food and Drug Administration (FDA, also by its acronym in English), expanding its use to patients aged 12 and older with advanced synovial sarcoma".
This precision treatment uses the patient's own immune cells, which are extracted and genetically modified in the laboratory to recognize the MAGE-A4 protein, present inside tumor cells. After reinfusion into the blood, these cells are capable of locating and destroying cancer in patients with a specific genetic profile who have already exhausted chemotherapy alternatives.
Regarding abemaciclib in the management of advanced or metastatic dedifferentiated liposarcoma, SEOM indicates that this oral drug "takes advantage of the tumor's genetic vulnerability by targetedly blocking the CDK4 protein - the engine that drives its cell growth -, managing to reduce the risk of disease progression or death by 61 percent and drastically extending progression-free survival from 1.5 to 9.7 months (and even to 16.4 months if used as first-line treatment), which redefines the standard of care for a cancer traditionally resistant to chemotherapy".
New options in non-GIST sarcomas
In the set of non-GIST sarcomas, the medical society highlights "important advances in their therapeutic approach". Among them, it cites that "in metastatic disease, the use of certain types of chemotherapy according to the sarcoma subtype is more widespread" and that "the use of certain drugs approved in recent years has been consolidated, such as trabectedin in L-sarcomas (liposarcoma and leiomyosarcoma), pazopanib in soft tissue sarcomas, and eribulin in adipocytic sarcomas".
"These advances have occurred in specific histotypes," notes SEOM, which points out that "it has been discovered that some sarcomas present a very specific genetic alteration, NTRK oncogene fusions." "Several clinical trials have demonstrated efficacy with larotrectinib and entrectinib, specific inhibitors of this alteration," it adds, specifying that "their use has already been approved by the European Union (EU), as well as in several European countries, although it has been rejected in Spain."
The organization also explains that "the role of immunotherapy in sarcomas is progressively being understood," and that "the use of combinations based on antiangiogenics and immunotherapy is highly effective in alveolar soft part sarcoma." "Currently, there are several clinical trials studying these concepts," it indicates, while highlighting "studies with CART in synovial sarcoma and myxoid liposarcoma, with very promising preliminary results."
Finally, SEOM mentions "some of the new drugs that have shown promising results in very rare types of sarcomas," such as "nirogacestat (gamma-secretase inhibitor) in desmoid tumors, CSF1R inhibitors (pexidartinib, vimseltinib, and pimicotinib) in tenosynovial giant cell tumor, and MDM2 antagonists in dedifferentiated liposarcomas."