Showing papers by "Dominique Ranchère-Vince published in 2020"
TL;DR: This nationwide registry of Sarcoma patients with histology reviewed by sarcoma experts shows that the incidence of sarcomas and TIM is higher than reported, and that tumors with an incidence<106/year have a much lower access to clinical trials.
Abstract: Background Since 2010, NETSARC and RREPS collected and reviewed prospectively all cases of sarcomas and tumors of intermediate malignancy (TIM) nationwide Methods The nationwide incidence of sarcoma or TIM (2013-2016), confirmed by expert pathologists using WHO classification are presented Yearly variations and correlation with published clinical trials was analyzed Results 139 histological subtypes are reported among the 25172 patients with sarcomas (n=18710, 64%) or TIM (n=6460, 36%), respectively n=5838, n=6153, n=6654, and n=6527 yearly from 2013 to 2016 Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 797, 249 and 951/106/year, above that previously reported GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of tumors Only GIST, as a single entity had a yearly incidence above 10/million/year There were respectively 30, 63 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1-01/106, or Conclusions This nationwide registry of sarcoma patients with histology reviewed by sarcoma experts shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with an incidence
71 citations
TL;DR: T in combination with RT was feasible and well tolerated in the preoperative setting and could potentially become an alternative to anthracycline+ifosfamide concurrent to RT in high risk localized ML.
Abstract: 11514Background: Myxoid liposarcoma (ML) exhibits especial sensitivity to trabectedin (T). In prospective series, T showed responses in 44% of patients (pts) with ML. ML is also sensitive to radiat...
8 citations
TL;DR: Leiomyosarcomas (LMS) are currently treated as a single entity but are now identified as a transcriptionally, genomically and clinically homogeneous subgroup of LMS, whose oncogenesis is driven by the acquisition of high differentiation through MYOCD over-expression, which confers them sensitivity toMYOCD/SRF inhibitors, which could thus become a potential therapeutic target.
Abstract: Leiomyosarcoma is a very aggressive tumor with poor prognosis since half of the patients will develop metastasis. Today, neither chemotherapy nor targeted or immune therapies have demonstrated efficacy. These tumors show highly rearranged genomes and appear to form a heterogenous group. Understanding their complex oncogenesis either as a single disease or by subtypes has already been challenged but the underlying mechanisms driving leiomyosarcoma development which could lead to a patient care improvement remain to be deciphered. We identified a group of tightly clustered leiomyosarcomas due to their gene expression homogeneity, named "hLMS". We derived a transcriptional signature able to consistently stratify patients from two independent cohorts. In all cohorts, hLMS were preferentially carried by women, located in the internal trunk, highly differentiated, and similarly altered at the genomic level. Through the multi-omics integrative bioinformatic analysis, we show that hLMS originate from vascular smooth muscle cells presenting both contractile and synthetic characteristics, while the other group could derive from fibroblasts. We identified the MYOCD overexpression as a hLMS-specific driver candidate and functionally showed that the MYOCD/SRF axis is only essential for hLMS survival. Identification of hLMS could become standard clinical practice, leading to the development of specific effective treatments with MYOCD/SRF inhibitors.
4 citations