Background: The exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes. Methods: Data were collected from population-based cancer registries covering 22% of the French population. Crude and world age-standardized incidence rates (ASR) were estimated according to anatomic, histological and genetic groups, age and sex over the 2010–2013 period. Results: Time trends in incidence were calculated by the annual percent change over the 2000–2013 period. During the most recent period (2010–2013), 3942 patients with sarcoma were included. The ASR of soft-tissue and bone sarcomas, and gastro-intestinal stromal tumors (GIST) were 2.1, 1.0 and 0.6, respectively. For the four most frequent histological subtypes (unclassified, leiomyosarcoma, GIST and liposarcoma), the ASR ranged from 0.4 to 0.7. ASRs were 1.9 for complex genomic and 1.3 for recurrent translocation sarcomas. The time-trend analysis showed a significant increase of sarcoma incidence rate between 2000 and 2005, which stabilized thereafter. Incidence rates increased for four histological subtypes (GIST, chondrosarcoma, myxofibrosarcoma, solitary fibrous tumors) and decreased for three (leiomyosarcomas, Kaposi sarcoma and fibrosarcoma). Conclusion: To our knowledge, this study is the first to investigate sarcoma incidence based on a systematic pathological review of these cancers and on the updated sarcoma classifications. Due to the paucity of literature on sarcomas, future studies using data from population-based cancer registries should consider a standardized inclusion criterion presented in our study to better describe and compare data between countries.
Keywords: Sarcoma; Incidence; Trends in incidence; France; Cancer registry
Sarcomas are a heterogeneous group of rare malignant tumors derived from primitive mesenchymal cells. These tumors arise from muscle, connective tissue, supportive tissue and vascular tissue, and more than 80 histologic subtypes are included in the 2013 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone [[
From an epidemiological point of view, the lack of a unified method of reporting sarcomas has led to considerable variations in the reported incidence and time trends Sarcomas are sometimes mistaken for carcinomas of the same organ, and can involve a variety of localizations. As a consequence, 30 % of sarcomas are misclassified at initial diagnosis [[
Currently, data for sarcomas in the French population are provided by the reference networks for sarcomas that collect and manage cases of soft tissue, bone and visceral sarcomas. Reference networks propose a systematic second histologic review by expert pathologists [[
Besides reference networks, cancer surveillance information is coming from the French Network of population-based cancer registries that exhaustively collects all newly diagnosed and confirmed cancer cases within geographical areas in France [[
Cases included in this study were children and adults with sarcoma diagnosed between January 1, 2000 and December 31, 2013, and living in one of the administrative areas covered by a population-based cancer registry of the French Network (details in online supplementary material). The French sarcoma pathological reference network (RRePS) and the French reference Network for bone sarcoma and rare bone tumors (RESOS) propose a systematic second histologic review and confirmation for all diagnoses of sarcomas across France [[
The following data were collected for each case: general demographic characteristics of the patients (age, sex, and residence area), the date of diagnosis, the anatomical site, and the histology of the tumor according to the International Classification of Diseases for Oncology, third edition (ICD-O-3) (
This study included intermediate (only with a "/3" behavior) and malignant sarcomas presenting morphologic criteria described in the 2013 WHO Classification of Tumors of Soft Tissue and Bone (fourth edition), regardless of the anatomic site [[
Certain alignments could not be performed: ten morphological terms not described in this updated classification (e.g. sarcoma NOS, periosteal fibrosarcoma, fascial fibrosarcoma ...) have been maintained for analyses. Conversely, well differentiated liposarcoma and chondroblastoma have been changed from malignant to borderline diseases. In the same way, behaviors for dermatofibrosarcoma protuberans and pigmented dermatofibrosarcoma protuberans have been also changed from malignant to borderline with henceforth, only fibrosarcomatous dermatofibrosarcoma protuberans which is coded as malignant behavior. In our analyses, we have made the choice to keep all dermatofibrosarcomas. Indeed, we do not have the possibility to differentiate if this is a dermatofibrosarcoma borderline or malignant. Besides, endometrial stromal sarcoma NOS (89303), low grade endometrial stromal sarcoma (89313) and stromal sarcoma (89353) not described in the WHO 2013 have been also included. Additional details on the list and choice of classification systems are provided in the online supporting material (see Additional File 1).
This classification also provides new genetic and molecular data for each histologic entity allowing a better characterization of sarcomas. The same group of experts were consulted with the aim of proposing the optimal classification system for sarcomas based on the genetic profile. Two main distinct genetic groups were defined: (i) sarcomas defined with simple genetics based on recurrent translocations (e.g. Ewing sarcoma, myxoïd liposarcoma), activating or inactivating mutations (e.g epithelioid sarcoma, gastrointestinal stromal tumor), MDM2 amplification (e.g. dedifferentiated liposarcoma, low-grade central osteosarcoma); and (ii) sarcomas with complex genomic profiles (e.g. angiosarcoma, leiomyosarcoma). Another group was defined for miscellaneous and undefined alterations. The list of histology codes according to their genetic groups is presented in the supplementary material.
This study is based on data from cancer registries gathered in the French network of cancer registries and a representative of each registry was involved in the study and approved the use of its data All French registries received an authorization to collect patient data from the data protection authority (Commission Nationale de l'Informatique et des Libertés). Ethics approval and consent to participate were not required for this study which is an observational research without direct contact with patient.
Two datasets were used: i) the first one was used to estimate the incidence of patients diagnosed during the 2010–13 period and that included data from 19 registries; and ii) the second one was used to examine trends in the incidence from 2000 to 2013 in only 11 registries for which data were available over the entire studied period. Incidence rates were presented per 100,000 person-years.
The incidence of sarcomas was described according to 1) the anatomic group (i.e. soft-tissue, bone, gastro-intestinal, skin, female genital organs, other viscera and other sites), and to 2) histologic and 3) genetic groups based on guidelines developed by sarcoma specialists (see Additional File 1).
Age-standardized incidence rates (ASR) were estimated using direct standardization and were calculated using the population data for each age group and year supplied by the National Institute of Statistics and Economic Studies (
Time trends were calculated using Joinpoint Trend Analysis Software setting a maximum of a single Joinpoint (details in online supplementary material). The annual percent change (APC) with the 95% confidence interval (CI) was estimated according to topographic and histologic groups.
Over the 2010–13 period, sarcomas accounted for 1.3% (3942/307,862) of all malignant tumors diagnosed over the French registry area. The male/female ratio for overall sarcomas was 1.0 but ranged from 0.5 for angiosarcomas to 6.2 for Kaposi sarcomas (KS) (Table 1). The median age was 63 years (range: 0–106) with large intergroup variations. About 9% of subjects were under 24 years and 27% were older than 75 years. Almost half of the cases were soft tissue sarcomas (45%). The most frequent histological subtypes were undifferentiated or unclassified sarcomas (16%), leiomyosarcoma (14%) and GIST (13%). Sarcomas with complex genomics accounted for the most frequent molecular profile (40%).
Gender distribution of sarcoma patients according to age and topographic, genomic and histologic groups. FRANCIM network data 2010–2013 (19 registries)
0–14 81 4.1 81 4.1 162 4.1 1.0 15–24 99 5.0 79 4.0 178 4.5 1.3 25–39 197 10.0 160 8.1 357 9.1 1.2 40–64 671 34.1 738 37.4 1409 35.7 0.9 65–74 376 19.1 379 19.2 755 19.2 1.0 75 and more 546 27.7 535 27.1 1081 27.4 1.0 Soft tissue 972 49.3 812 41.2 1784 45.3 1.2 Bone 310 15.7 259 13.1 569 14.4 1.2 Skin 262 13.3 167 8.5 429 10.9 1.6 Viscera Gastro-intestinal organs 291 14.8 287 14.6 578 14.7 1.0 Female genital organs – – 282 14.3 282 7.2 – Others visceral organs 102 5.2 129 6.5 231 5.9 0.8 Other anatomic sites 33 1.7 36 1.8 69 1.8 0.9 Complex genomic alterations 723 36.6 847 43.0 1570 39.8 0.9 135 6.9 81 4.1 216 5.5 1.7 Mutations 274 13.9 276 14.0 550 14.0 1.0 Recurrent translocations 340 17.3 438 22.2 778 19.7 0.8 Undefined/Miscellaneous alterations 498 25.3 330 16.7 828 21.0 1.5 Unclassified sarcomaa 327 16.6 308 15.6 635 16.1 1.1 Leiomyosarcoma 205 10.4 346 17.5 551 14.0 0.6 GIST 246 12.5 250 12.7 496 12.6 1.0 Liposarcoma 228 11.6 130 6.6 358 9.1 1.8 Chondrosarcoma 123 6.2 118 6.0 241 6.1 1.0 Dermatofibrosarcoma 101 5.1 124 6.3 225 5.7 0.8 Kaposi sarcoma 156 7.9 25 1.3 181 4.6 6.2 Angiosarcoma 54 2.7 115 5.8 169 4.3 0.5 Osteosarcoma 84 4.3 71 3.6 155 3.9 1.2 Ewing sarcoma 72 3.7 65 3.3 137 3.5 1.1 Myxofibrosarcoma 75 3.8 49 2.5 124 3.1 1.5 Rhabdomyosarcoma 66 3.4 51 2.6 117 3.0 1.3 Nerve Sheath Tumors 38 1.9 44 2.2 82 2.1 0.9 Endometrial stromal sarcoma – – 81 4.1 81 2.1 – Synovial sarcoma 37 1.9 40 2.0 77 2.0 0.9 Chordoma 40 2.0 27 1.4 67 1.7 1.5 Solitary fibrous tumor, malignant 33 1.7 33 1.7 66 1.7 1.0 Fibrosarcoma 15 0.8 16 0.8 31 0.8 0.9 Malignant myoepithelioma 12 0.6 11 0.6 23 0.6 1.1 Epithelioid haemangioendothelioma 9 0.5 11 0.6 20 0.5 0.8 Other (with fewer than 20 cases) 49 2.5 57 2.9 106 2.7 0.7 1970 100.0 1972 100.0 3942 100.0 1.0
The crude incidence rate and ASR-W of sarcomas were 7.4 and 5.0, respectively (Table 2). The ASR-W of soft tissue, bone and gastro-intestinal sarcomas were 2.1, 1.0 and 0.6, respectively. For the five most frequent histological subtypes, the ASR-W ranged from 0.3 to 0.7 with gender variations. For the two most frequent genomic profiles (over 60% of all sarcoma cases) the ASR-W was 1.9 for complex genomic and 1.3 for recurrent translocation events.
Sarcoma crude and age-standardized incidence rate per 100,000 person-years according to topographic, genomic and histological groups by sex. FRANCIM network data 2010–2013 (19 registries)
Median Age Male Female Overall CIR ASR-W (segi) ASR-E ASR-US CIR ASR-W (segi) ASR-E ASR-US CIR ASR-W (segi) ASR-E ASR-US Soft tissue 65 3.70 2.43 3.12 3.31 2.90 1.91 2.34 2.38 3.30 2.15 2.68 2.78 Bone 47 1.20 1.09 1.16 1.17 0.90 0.85 0.87 0.88 1.10 0.96 1.01 1.01 Skin 54 1.00 0.70 0.88 0.91 0.60 0.46 0.54 0.54 0.80 0.59 0.72 0.73 Viscera Gastro-intestinal organs 69 1.10 0.65 0.91 0.95 1.00 0.57 0.77 0.79 1.10 0.58 0.81 0.84 Female genital organs 62 – – – – 1.00 0.62 0.82 0.81 1.00 0.62 0.82 0.81 Other visceral organs 65 0.40 0.26 0.33 0.33 0.50 0.28 0.36 0.37 0.40 0.26 0.34 0.35 Others anatomic sites 55 0.10 0.15 0.13 0.13 0.10 0.10 0.10 0.10 0.10 0.10 0.10 0.10 Complex genomic alterations 65 2.80 1.78 2.30 2.43 3.10 1.93 2.44 2.47 2.90 1.87 2.38 2.45 68 0.50 0.30 0.42 0.45 0.30 0.18 0.23 0.23 0.40 0.23 0.31 0.33 Mutations 68 1.10 0.66 0.87 0.90 1.00 0.58 0.76 0.77 1.00 0.62 0.80 0.83 Recurrent translocations 46 1.30 1.23 1.30 1.30 1.60 1.44 1.56 1.54 1.50 1.34 1.44 1.43 Undefined/Miscellaneous alterations 65 1.90 1.27 1.62 1.70 1.20 0.73 0.90 0.94 1.60 0.97 1.22 1.26 Unclassified sarcomaa 69 1.30 0.71 1.00 1.08 1.10 0.62 0.82 0.85 1.20 0.65 0.90 0.94 Leiomyosarcoma 66 0.80 0.43 0.62 0.68 1.30 0.74 0.99 0.99 1.00 0.58 0.79 0.81 GIST 69 0.90 0.52 0.75 0.78 0.90 0.47 0.65 0.67 0.90 0.50 0.70 0.72 Liposarcoma 67 0.90 0.48 0.68 0.72 0.50 0.28 0.37 0.37 0.70 0.38 0.51 0.53 Chondrosarcoma 55 0.50 0.32 0.41 0.40 0.40 0.30 0.36 0.36 0.50 0.34 0.41 0.41 Dermatofibrosarcoma 44 0.40 0.31 0.35 0.35 0.50 0.40 0.45 0.44 0.40 0.37 0.42 0.41 Kaposi sarcoma 63 0.60 0.40 0.52 0.54 0.10 0.04 0.06 0.06 0.30 0.20 0.27 0.28 Angiosarcoma 73 0.20 0.10 0.15 0.16 0.40 0.17 0.25 0.27 0.30 0.16 0.23 0.25 Osteosarcoma 34 0.30 0.34 0.33 0.33 0.30 0.25 0.24 0.25 0.30 0.28 0.27 0.27 Ewing sarcoma 19 0.30 0.35 0.30 0.30 0.20 0.32 0.27 0.27 0.30 0.33 0.28 0.28 Myxofibrosarcoma 66 0.30 0.16 0.22 0.22 0.20 0.10 0.14 0.14 0.20 0.14 0.19 0.19 Rhabdomyosarcoma 25 0.30 0.26 0.24 0.25 0.20 0.21 0.18 0.18 0.20 0.25 0.22 0.23 Nerve Sheath Tumors 55 0.10 0.10 0.13 0.13 0.20 0.12 0.15 0.15 0.20 0.12 0.15 0.15 Endometrial stromal sarcoma 62 – – – – 0.30 0.17 0.23 0.23 0.30 0.17 0.23 0.23 Synovial sarcoma 47 0.10 0.16 0.17 0.17 0.10 0.13 0.15 0.14 0.10 0.15 0.16 0.16 Chordoma 61 0.20 0.11 0.14 0.15 0.10 0.06 0.08 0.08 0.10 0.09 0.12 0.12 Solitary fibrous tumor. Malignant 63 0.10 0.11 0.14 0.13 0.10 0.08 0.10 0.10 0.10 0.10 0.12 0.12 Fibrosarcoma 60 0.10 0.03 0.05 0.05 0.10 0.03 0.04 0.04 0.10 0.03 0.04 0.04 Malignant myoepithelioma 56 0.00 0.03 0.04 0.04 0.00 0.03 0.04 0.04 0.00 0.03 0.04 0.04 Epithelioid haemangioendothelioma 47 0.00 0.03 0.03 0.03 0.00 0.05 0.05 0.05 0.00 0.05 0.06 0.06 Other (with fewer than 20 cases) 46 0.20 0.19 0.17 0.18 0.20 0.19 0.20 0.20 0.20 0.20 0.20 0.20 63 7.60 5.27 6.54 6.80 7.20 4.81 5.83 5.90 7.40 5.00 6.12 6.26
Abreviations: GIST Gastro-Intestinal Stromal Tumors, CIR Crude Incidence Rate per 100,000 persons-years, ASR-W, ASR-E and ASR-US Age-Standardized incidence Rate from three reference populations (W, World Segi; E, European; US, United-States)
The overall sarcoma incidence peaked at 22 in patients aged 75 or over (data not shown). Age-specific rates for soft tissue, viscera and skin sarcomas were relatively stable among patients aged between 0 and 40 years, and then increased with age (Fig. 1). This increase was less pronounced in women. In men, bone sarcomas presented a biphasic profile with a first peak in young people between 15 and 25 years of age and a second peak in adults aged between 65 and 74 years of age. With respect to histological subtypes, age-specific incidence rates had various profiles (see Additional File 2). According to the genomic profile, the incidence increased steadily with age, except for tumors harboring recurrent translocations and MDM2 amplification among women (see Additional File 3).
Graph: Fig. 1 Age-specific incidence rates of sarcomas per 100,000 person-years according to topographic groups. FRANCIM network data 2010–2013 (19 registries)
The ASR-W for all sarcomas increased between 2000 and 2005 (APC = 3.6%), and remained stable since 2005 (non-significant APC, Table 3). According to the anatomic site, the ASR-W decreased for skin sarcomas (APC = -2.0%) and female genital tumors between 2005 and 2013 (APC = -2.2%). Stratifying by major histological subtypes, the ASR-W increased for GIST (APC = 3.7%), chondrosarcoma (APC = 4.1%), myxofibrosarcoma (8.2%) and solitary fibrous tumors (12.2%) and decreased for leiomyosarcoma (APC = -2.6%), Kaposi sarcoma (− 4.1%) and fibrosarcoma (APC = -9.2%). All trend figures are provided in the online supplementary material (see Additional Files 4 and 5).
Annual percentage change of world age-standardized incidence rate by topographic groups, histologic types. FRANCIM network data 2000–2013 (11 registries)
n Joinpoint APC 95% CI Soft Tissue 3766 0.8 (−0.4; 2.0) Bone 1193 1.2 (−0.4; 2.9) Skin 1062 (−3.5; −0.4) Viscera tumors organs Gastro-intestinal organs 1053 1.5 (−0.3; 3.3) Female genital organs 297 2000–2005 4.2 (−4.2; 13.2) 376 2005–2013 (−10.4; −2.7) Other visceral organs 540 −1.7 (−5.1; 1.8) Other anatomic sites 171 1.0 (−3.4; 5.6) Unclassified sarcoma 1513 −1.6 (−3.6; 0.3) Leiomyosarcoma 1281 (−4.6; −0.6) GIST 822 (0.8; 6.8) Liposarcoma 713 1.3 (−1.1; 3.7) Dermatofibrosarcoma 496 0.6 (−1.4; 2.7) Chondrosarcoma 454 (1.6; 6.6) Kaposi sarcoma 419 (−6.8; −1.4) Osteosarcoma 359 −0.6 (−3.7; 2.6) Angiosarcoma 335 2.2 (−1.2; 5.7) Ewing sarcoma 330 −0.2 (−4.1; 3.8) Rhabdomyosarcoma 286 −1.1 (−6.2; 4.4) Others (with fewer than 20 cases) 226 4.1 (−1.3; 9.8) Synovial sarcoma 219 1.2 (−4.2; 6.9) Nerve Sheath Tumors 191 −0.1 (−4.3; 4.4) Myxofibrosarcoma 183 (0.4; 16.6) Endometrial stromal sarcoma 173 −3.7 (−7.4; 0.1) Fibrosarcoma 151 (−15.7; −2.3) Chordoma 126 0.8 (−4.8; 6.6) Solitary fibrous tumor. Malignant 102 (6.2; 18.5) Epithelioid haemangioendothelioma 55 – – Myoepithelial carcinoma 24 – – 3359 2000–2005 (0.2; 7.1) 5099 2005–2013 −1.4 (−2.9; 0.1)
Note. Joinpoint = years when statistically significant changes in incidence trend occurred APC Annual Percent Change, CI Confidence Interval
In this study, we precisely described the incidence of sarcomas according to different classifications (anatomic, histologic and genetic) using data from population-based cancer registries. To our knowledge, this is one of the first reports on sarcomas based on a systematic pathological review of these cancers while taking into account the updated sarcoma classifications.
In this study, sarcomas accounted for 1.3% of all malignant tumors (1.1% for soft tissue -including skin and viscera- and 0.2% for bone) and had an ASR-E of 6.1 per 100,000 person-years over the 2010–2013 period (European population standard). The ASR-E was slightly higher than that reported in Europe [[
With respect to the anatomic site, ASR-E for STS (2.7) in our study was below most published international incidence rates. This may be explained by the exclusion of visceral sarcomas of soft tissue and the different description of well-differentiated liposarcoma compared to the WHO 2013 classification. In the current study, ASR-Ws for bone sarcomas among males and females (1.1 and 0.9 respectively) were close to those recently reported in five continents (2010–13 period, ASR-W 0.8–1.2 in males and 0.5–1.0 in females) [[
The comparison of ASRs for main histologic groups between studies with a shorter inclusion period showed that the ASR-E for leiomyosarcoma (0.8; 0.6 for males and 1.0 for females) was greater than that reported in France (0.6) and was similar to that reported in three European regions (0.5 for males and 1.0 for females) [[
Molecular biology of sarcomas, available for diagnosis in France since 2010 is a complementary approach and has led to a molecular classification for sarcomas [[
This study provides the first time trend analysis of sarcomas in France and shows that ASR-W for sarcomas increased between 2000 and 2005 (APC = 3.6%) and stabilized from 2005. The current study has not shown an increase in ASR-W for soft-tissue sarcomas. This is in contrast to reports in others countries covering different periods: in the United States APC was 1.2% for males and 0.8% for females between 1978 and 2001, in Japan APC was 0.6% between 1978 and 2007 and in Serbia APC was 0.77% between 1985 and 2009 [[
The different incidence trends for sarcomas reported over the world may partly be explained by variations in diagnosis practices and the classification used. The impact of environmental factors in the etiology of these cancers may also be a point at issue. However, the large heterogeneity of histological subtypes and the rarity of sarcomas prevent examining this association and drawing conclusions from existing environmental epidemiological studies. A national French study on the etiology of sarcomas (Etiosarc) has been launched to study the possible effect of environmental factors [[
A major strength in this study is that the incidence of sarcomas was estimated using the 2013 WHO classification [[
Moreover, this study is the first to describe sarcomas in a geographic area where an expert sarcoma pathologist reviews the pathologic diagnosis. Contrary to imperfectly estimated sarcoma incidence rates, this review allows to provide a consistent incidence of sarcomas. A French study, confirmed these results and indicated that 45% of sarcomas are misclassified at initial diagnosis and that 19% have complete discordance [[
This study provided the opportunity to precisely describe the incidence of sarcomas according to three different groups (anatomic, histologic and genetic) defined by sarcoma specialists using data from population-based cancer registries. To our knowledge, this study is the first to report sarcoma incidence based on a systematic pathological review of these cancers and taking into account the updated sarcoma classifications. Due to literature paucity on sarcomas, future studies using data from population-based cancer registries will have to consider a strict inclusion criterion presented in our study to better describe and compare data between countries. The molecular classification will be useful for etiological studies as incidence studies.
This work was supported by the French National Cancer Institute (in the framework of INCa-BCB 2012 grant for constitution of multicentre clinical and biological databases nationwide in cancer. Funding bodies had no role in the design of the study, collection, analysis, and interpretation of data and in writing the manuscript.
We thank Vianney Jouhet for advice about classification alignements and Marie Poiseuil for datamanagement. Thanks to Jone Iriondo-Alberdi for proofreading and comments.
We thank the Francim Network for their collaboration in the study: J Jégu, M Velten (Bas-Rhin General Cancer Registry); E Cornet, X Troussard (Registre Régional des Hémopathies Malignes de Basse Normandie); A M Bouvier (Registre Bourguignon des Cancers Digestifs); A V Guizard (Registre Général des Tumeurs du Calvados); V Bouvier, G Launoy (Registre des Tumeurs Digestives du Calvados); P Arveux (Breast cancers registry of Côte-d'Or France); M Maynadié, M Mounier (Hémopathies Malignes de Côte d'Or); A S Woronoff (Doubs and Belfort Territory General Cancer Registry); M Daoulas, M Robaszkiewicz (Finistère Cancer Registry); J Clavel, S Goujon (French National Registry of Childhood Hematopoietic Malignancies); B Lacour (National Registry of Childhood Solid Tumors); I Baldi, C Pouchieu (Gironde Registry of Primary Central Nervous System Tumors); B Amadeo, G Coureau (General Cancer Registry of Gironde Department); S Leguyader, A Monnereau, S Orazio (Registre des Hémopathies Malignes de la Gironde); P M Preux, F Rharbaoui (Registre Général des Cancers de Haute-Vienne); E Marrer (Haut-Rhin Cancer Registry); B Trétarre (Registre des Tumeurs de l'Hérault); M Colonna, P Delafosse (Registre du Cancer du Département de l'Isère); K Ligier, S Plouvier (Registre Général des Cancers de Lille et de sa Region); A Cowppli-Bony, F Molinié (Loire-Atlantique-Vendée Cancer Registry); S Bara (Manche Cancer Registry); O Ganry, B Lapôtre-Ledoux (Registre du Cancer de la Somme); P Grosclaude (Tarn Cancer Registry); N Bossard, Z Uhry (Hospices Civils de Lyon). We thank all pathologists, clinicians, and clinical research assistants of French sarcoma networks (RRePS, NetSarc and ReSos).
BA performed the statistical analyses and wrote the original draft. ED, SMP and NP conceived of the study and contributed to revising the manuscript for intellectual content. JMC, IRC, NP (sarcoma specialists) validated ICD–O3 codes to include in the study. Francim network participated in the data acquisition. JG contributed to manuscript preparation and writing review. KL, PD, AMB, SP, AL, GC and AM contributed to manuscript validation and writing-review. All authors read and approval the final manuscript.
The datasets analyzed during the current study are not publicly available due to national regulations. Permission to use French cancer registry data was provided by the National Cancer Institute after consultation with the data protection authority.
This study is based on data from cancer registries gathered in the French network of cancer registries and a representative of each registry was involved in the study and approved the use of its data. All French registries received an authorization to collect patient data from the data protection authority (Commission Nationale de l'Informatique et des Libertés). Ethics approval and consent to participate were not required for this study which is an observational research without direct contact with patient.
Not applicable.
The authors declare that they have no competing interests.
Graph: Additional file 1. Complementary information on data collection and statistical analyses.
Graph: Additional file 2: Figure S1. Age-specific incidence rates of sarcomas per 100,000 person-years according to histologic groups. FRANCIM network data 2010–2013 (19 registries).
Graph: Additional file 3: Figure S2. Age-specific incidence rates of sarcomas per 100,000 person-years according to genomic groups. FRANCIM network data 2010–2013 (19 registries).
Graph: Additional file 4: Figure S3. Sarcoma trends and annual percentage change (APC) of world age-standardized incidence rate according to topographic group. FRANCIM network data 2000–2013 (11 registries).
Graph: Additional file 5: Figure S4. Sarcoma trends and annual percentage change (APC) of world age-standardized incidence rate according to histologic group. FRANCIM network data 2000–2013 (11 registries).
• APC
- Annual percentage change
• ASR
- Age-standardized incidence rates
• CI
- Confident interval
• GIST
- Gastro-intestinal stromal tumors
• ICD-O-3
- International Classification of Diseases for Oncology, third edition
• KS
- Kaposi sarcoma
Supplementary information accompanies this paper at 10.1186/s12885-020-6683-0.
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By Brice Amadeo; Nicolas Penel; Jean-Michel Coindre; Isabelle Ray-Coquard; Karine Ligier; Patricia Delafosse; Anne-Marie Bouvier; Sandrine Plouvier; Justine Gallet; Aude Lacourt; Gaëlle Coureau; Alain Monnereau; Simone Mathoulin-Pélissier and Emmanuel Desandes
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