ArticlePloS one2021
Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network.
Article in PloS one, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 128 papers, 1 of them a synthesis that pooled it.
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Who cites it
128 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Prognostic factors in patients with relapsed high-grade osteosarcoma: a systematic review.British journal of cancer · 2025Pooled it
- A Single-Arm Phase 2 Trial of Trametinib in Patients with Locally Advanced or Metastatic Epithelioid Hemangioendothelioma.Clinical cancer research : an official journal of the American Association for Cancer Research · 2024Trial
- Survival of patients with ruptured gastrointestinal stromal tumour treated with adjuvant imatinib in a randomised trial.British journal of cancer · 2024Trial
- Afamitresgene autoleucel for advanced synovial sarcoma and myxoid round cell liposarcoma (SPEARHEAD-1): an international, open-label, phase 2 trial.Lancet (London, England) · 2024Trial
- Trial
- Paediatric therapeutic development workshop on desmoplastic small round cell tumours.British journal of cancer · 2026Review
- Precision therapeutic strategies for advanced gastrointestinal stromal tumors.Cancer metastasis reviews · 2026Review
- MiT fusions, TSC1-TSC2 divergence, and stem-like programs reveal distinct origins and vulnerabilities in PEComa.Nature communications · 2026Article
- Overall survival of patients with localized soft tissue sarcoma treated with or without adjuvant chemotherapy (DEEPSARC): a real world nationwide retrospective cohort study in France.EClinicalMedicine · 2026Article
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- Systemic Therapy for Solitary Fibrous Tumor.Current treatment options in oncology · 2026Review
- Pleomorphic rhabdomyosarcoma, outcomes of patients with advanced disease treated with systemic agents: Retrospective study from the global pushing ultra-rare sarcomas towards hope (PUSH) consortium.European journal of cancer (Oxford, England : 1990) · 2026Article
- Multi-layered molecular profiling informs the diagnosis and targeted therapy of desmoplastic small round cell tumor.Nature communications · 2026Article
- Molecular epidemiology of the expression of urokinase plasminogen activator receptor-associated protein (uPARAP) in mesenchymal malignancies.Translational oncology · 2026Article
- Hippo signalling pathway mediates oncogenic properties of NAB2::STAT6 in solitary fibrous tumour.Cellular oncology (Dordrecht, Netherlands) · 2026Article
- Article
- The effect of adjuvant imatinib treatment for patients with high-risk gastrointestinal stromal tumor.Frontiers in oncology · 2026Article
- Chromatin Remodeling Subunit ARID1A Negatively Regulates the Malignant Progression of Gastrointestinal Stromal Tumors by Targeting the MEMO1 Promoter.International journal of medical sciences · 2026Article
- Epithelioid Hemangioendothelioma With a Novel Chromosomal Deletion and an Aggressive Clinical Course: A Case Report and Literature Review.Case reports in oncological medicine · 2026Article
- First case report of hypouricemia associated with adjuvant imatinib therapy in a patient with small intestinal gastrointestinal stromal tumor.Frontiers in oncology · 2026Article
68 more citing papers are in PubMed but not listed here.
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Authors and funding
55 authors.
Funding
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Abstract
backgroundSince 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France.
methodsThe nationwide incidence of sarcoma or TIM (2013-2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. Simple clinical characteristics, yearly variations and correlation of incidence with published clinical trials are presented and analyzed.
resultsOver 150 different histological subtypes are reported from the 25172 patients with sarcomas (n = 18712, 74,3%) or TIM (n = 6460, 25.7%), with n = 5838, n = 6153, n = 6654, and n = 6527 yearly cases from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/106/year, higher than 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 10/106/year. There were respectively 30, 64 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1-0.1/106, or < 0.1/106/year respectively. The 2 latter incidence groups represented 21% of the patients with 130 histotypes. Published phase III and phase II clinical trials (p<10-6) are significantly higher with sarcomas subtypes with an incidence above 1/106 per.
conclusionsThis nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (1<106/year) are less likely to be included in clinical trials.
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