The Connective Tissue Oncology Group Annual Meeting 2018 (CTOS 2018) took place in Rome from 4 to 17 November 2018, and the 39th Plenary Meeting of the Scandinavian Sarcoma Group (SSGM 2019) was held in Bergen from 8 to 10 May 2019. These two large international conferences brought together an overwhelming majority of molecular and clinical specialists in the sarcoma field, especially those working on soft tissue sarcoma. Topics discussed on the conferences included, among others, sarcoma genetics, clinical and molecular subclassification, targeted therapy, clinical prognostication, and new experimental sarcoma models. A large ongoing international study on germinal sarcoma genetics was presented, the interim results of which revealed the extremely complex nature of genetic disposition to sarcoma, and, surprisingly, a rather prominent place among predisposing genes for those coding for structural telomere constituents. Fusion oncogenes dominate somatic sarcoma genetics, especially because of their origin and impact on sarcoma clinical behaviour, and are especially relevant for karyotypically simple paediatric sarcomas. A crucial issue in karyotypically complex sarcomas are the efforts being made to obtain a subclassification of sarcoma, other than those based on pathology, using either the clinical characteristics of sarcomas (uterine leiomyosarcoma vs. soft tissue leiomyosarcoma) or specific gene expression profiles (molecular subtypes in undifferentiated pleiomorphic sarcoma), which showed that molecular characterization can open the way for subtype specific therapies. Other examples of where this type of strategy can be applied include gastrointestinal stromal tumours, infantile fibrosarcoma, and inflammatory myofibroblastic tumours, where targeted therapy could be conceived based on the actionable mutations identified. Attempts in this direction have been made also for clear cell sarcoma and dedifferentiated liposarcoma, albeit the effectiveness of molecular-targeted treatments for these sarcomas is still poor, and progress in the treatment of osteosarcoma is still rather slow. Actually, the platelet-derived growth factor signalling system holds a prominent position in searches for targeted therapies, not only against rare sarcoma types, where are activated by mutations (some gastrointestinal stromal tumours, infantile hereditary myofibromatosis, and dermatofibrosarcoma protuberans), but also against other more usual sarcoma types, where the blocking anti-PDGFRα-antibody olaratumab has been successfully integrated into combinatorial chemotherapeutic regimens. In the field of clinical prognostication, remarkable progress in sarcoma nomograms was reported. Interesting results were also presented in the area of new experimental sarcoma models. Participation on both scientifi c conferences and all the experimental work leading to the presented sarcoma models were supported by the Czech Science Foundation project No. 17-17636S. The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 20. 9. 2019 Accepted: 6. 11. 2019.
- Klíčová slova
- chondrosarcoma, experimental sarcoma models, genetic predisposition, molecular subtypes, osteosarcoma, prognostic nomograms, soft tissue sarcomas, targeted therapy,
- MeSH
- cílená molekulární terapie MeSH
- lidé MeSH
- nádory měkkých tkání * klasifikace genetika terapie MeSH
- sarkom * klasifikace genetika terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- kongresy MeSH
In early 2013, the new classification of tumours of soft tissue and bones was released. This edition belongs to the fourth series of so-called Blue Books published under the auspices of the World Health Organisation (WHO). The current classification follows the previous third edition, from which it differs in several aspects. The vast majority of changes are related to the soft tissue tumour section, which was enriched with three new chapters, some entities or terms were removed, new diagnoses were introduced, and several tumours were reallocated to other categories. Albeit to a lesser extent, similar changes have occurred also in the classification of bone tumours. Compared to the previous edition, more detailed molecular and cytogenetic data were incorporated in the current issue. The rapidly increasing knowledge of the genetics of mesenchymal tumours allows us to make more accurate diagnoses as well as to better understand of the pathogenesis of these lesions. However, abundant molecular and cytogenetic data highlight an increasing problem of growing numbers of genetic overlaps even among quite different tumours. The coexistence of several grading systems of soft tissue tumours is another controversial issue mentioned in the recent WHO classification. The main advantages and limitations of the two most widely used grading systems are also discussed.
- MeSH
- lidé MeSH
- nádory kostí klasifikace genetika MeSH
- nádory měkkých tkání klasifikace genetika MeSH
- Světová zdravotnická organizace MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
We report two cases of hyalinizing spindle cell tumors with giant rosettes arising in the pararectal space and soft tissues of the wrist in a 46-year-old man and 22-year-old-woman, respectively. Microscopically, the tumors exhibited a varied morphology, including hyalinizing hypocellular and cellular fibromatosis-like areas. The most striking morphologic feature was the formation of giant rosette like structures with collagen cores scattered throughout the tumors. Most of the tumor spindle cells were diffusely immunoreactive for lysozyme, CD-68, factor XIII and vimentin. Reactivity for smooth muscle actin, desmin and S-100 protein was not found. Ultrastructural examination of the rosettes in one case only showed normal native collagen.
- MeSH
- dospělí MeSH
- elektronová mikroskopie MeSH
- faktor XIII analýza MeSH
- fibrosarkom patologie MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- muramidasa analýza MeSH
- nádory měkkých tkání klasifikace patologie MeSH
- vimentin analýza MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- faktor XIII MeSH
- muramidasa MeSH
- vimentin MeSH
