Abstract
Connective Tissue Oncology Group Annual Meeting 2018 took place in Rome from 4 to 17 November 2018, and the 39th Plenary Meeting of the Scandinavian Sarcoma Group was held in Bergen from 8 to 10 May 2019. These both large international conferences brought together an overwhelming majority of molecular and clinical specialists in the sarcoma field, especially concerning soft tissue sarcoma.
Topics discussed on the conference included, among others, the issues of sarcoma genetics, clinical and molecular subclassification, targeted therapy, clinical prognostication and new experimental sarcoma models. In the field of germinal sarcoma genetics, a large ongoing international study has been presented; the interim analysis of results revealed a very complex nature of sarcoma genetic predisposition, and, surprisingly, a rather prominent place among the predisposing genes occupying those coding for structural telomere constituents.
Fusion oncogenes dominate somatic sarcoma genetics, especialy as to their origine and impact on sarcoma clinical behaviour; this topic is especially relevant for karyotypically simple, mostly pediatric sarcomas. A crucial issue in karyotypically complex sarcomas is the effort for their subclassification beyond the basic pathology, based either on their clinical characteristics (uterine leiomyosarcoma versus soft tissue leiomyosarcoma) or specific gene expression profiles (molecular subtypes in undifferentiated pleiomorphic sarcoma).
These examplex also showed that molecular characterization can open the way for subtype specific therapies. Other examples of such a strategy includes gastrointestinal stromal tumour, infantile fibrosarcoma or inflammatory myofibroblastc tumour, where in all cases targeted therapy could be conceived based on the actionable mutation identified.
Attempts into this direction have been made also for clear cell sarcoma and dedifferentiated liposarcoma, albeit here their effectiveness is still poor, and the progress is osteosarcoma is still rather slow as well. Actually, the Platelet-Derived Growth Factor signalling system holds a prominent position in search for targeted therapies, not only among rare sarcoma types, where it is activated by a mutation (a small part of gastrointestinal stroma tumours, infantile hereditary myofibromatosis, dermatofibrosarcoma protuberans), but also across other, distinctly more usual sarcoma types, where specific blocking anti-PDGFRα-antibody olaratumab could be successfully integrated into combinatorial chemotherapeutic regimens.
In the field of clinical prognostication, we could see a remarkable progress in sarcoma nomograms. Interesting results have also been presented in the area of new experimental sarcoma models.