Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

Gatta, Gemma; Capocaccia, Riccardo; Botta, Laura; Mallone, Sandra; Angelis, Roberta De; Ardanáz, Eva; Comber, Harry; Dimitrova, Nadya; Leinonen, Maarit K.; Siesling, Sabine; Zwan, Jan Maarten van der; Eycken, Liesbet Van; Visser, Otto; Žakelj, Maja Primic; Anderson, Lesley A.; Bella, Francesca; Innos, Kaire; Otter, Renée; Stiller, Charles; Trama, Annalisa; Hackl, Monika; Henau, Kris; Damme, Nancy Van; Valerianova, Z.; Šekerija, Mario; Dušek, Ladislav; Mägi, Margit; Paapsi, Keiu; Malila, Nea; Velten, Michel; Troussard, Xavier; Bouvier, Véronique; Guizard, A.V.; Bouvier, Anne; Arveux, Patrick; Maynadié, Marc; Woronoff, Anne Sophie; Robaszkiewicz, Michel; Baldi, Isabelle; Monnereau, Alain; Trétarre, Brigitte; Colonna, Marc; Molinié, F.; Bara, Simona; Schvartz, Claire; Lapôtre-Ledoux, Bénédicte; Grosclaude, Pascale; Stabenow, Roland; Luttmann, Sabine; Nennecke, Alice; Engel, Jutta; Schubert-Fritschle, Gabriele; Heidrich, Jan; Holleczek, Bernd; Jónasson, Jón Gunnlaugur; Clough‐Gorr, Kerri M.; Mazzoleni, Guido; Giacomin, Adriano; Sardo, Antonella Sutera; Barchielli, Alessandro; Serraino, Diego; Collarile, Paolo; Pannozzo, Fabio; Ricci, Paolo; Autelitano, M.; Spagnoli, Gianbattista; Fusco, Mario; Usala, M.; Vitale, Francesco; Michiara, Maria; Tumino, Rosario; Mangone, Lucìa; Falcini, Fabio; Ferretti, Stefano; Filiberti, Rosa Angela; Marani, Enza; Caiazzo, Anna Luisa; Iannelli, A.; Sensi, Flavio; Piffer, Silvano; Gentilini, Maria; Madeddu, A; Colanino, Antonino Ziino; Maspero, S.; Candela, P.; Stracci, Fabrizio; Tagliabue, Giovanna; Rugge, Massimo; Baili, Paolo; Minicozzi, Pamela; Sant, Milena; Ţereanu, Carmen; Francisci, Silvia; Tavilla, Andrea; Pierannunzio, Daniela; Rossi, Silvia; Santaquilani, Mariano; Knijn, Arnold; Pildava, Santa; Smailytė, Giedrė; Calleja, Neville; Agius, Dominic; Johannesen, Tom Børge; Rachtan, Jadwiga; Góźdż, Stanisław; Błaszczyk, Jerzy; Kępska, Kamila; Lacerda, G. Forjaz de; Bento, Maria José; Miranda, Ana; Diba, C. Safaei; Žagar, Tina; Almar, Enrique; Larrañaga, Nerea; Munain, Arantza López de; Torrella-Ramos, A.; García, J.M. Díaz; Marcos‐Gragera, Rafael; Yuste-Sánchez, María José; Navarro, Carmen; Salmerón, Diego; Moreno‐Iribas, Conchi; Galcerán, Jaume; Carulla, Marià; Mousavi, Seyed Mohsen; Bouchardy, Christine; Ess, Silvia; Bordoni, Andrea; Konzelmann, Isabelle; Rashbass, Jem; Gavin, Anna; Brewster, David H.; Huws, Dyfed Wyn; Ho, Vincent K.Y.; Benhamou, E

doi:10.1016/s1470-2045(17)30445-x

Cited by 305 publications

(234 citation statements)

References 19 publications

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“…Limited treatment options, high costs of existing treatments, and frequent misdiagnosis of rare cancers burden patients, physicians, and health systems [4]. The lack of epidemiological knowledge about the risk factors associated with many rare cancers precludes the development of viable prevention, making it one of the most dramatic unresolved public health problems [1,3,5]. In comparison, well-studied cancers such as colorectal cancer, which constitutes a smaller portion of the overall cancer cases (13%), has had significant progress in cancer control and prevention [5,6].…”

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confidence: 99%

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001–2016

Bock

Hoffmann

Jiang

et al. 2020

IJERPH

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Rare cancers, affecting 1 in 5 cancer patients, disproportionally contribute to cancer mortality. This research focuses on liposarcoma, an understudied rare cancer with unknown risk factors and limited treatment options. Liposarcoma incident cases were identified from the U.S. Surveillance, Epidemiology, and End Result (SEER) program and the combined SEER-National Program of Cancer Registries (CNPCR) between 2001-2016. Incidence rates (age-adjusted and age-specific), 5-year survival, and the time trends were determined using SEER*stat software. Three-dimensional visualization of age-time curves was conducted for males and females. SEER liposarcoma cases represented~30% (n = 11,162) of the nationwide pool (N = 37,499). Both sources of data showed males accounting for~60% of the cases; 82%-86% cases were identified among whites. Age-adjusted incidence was greater among males vs. females and whites vs. blacks, whereas survival did not differ by sex and race. The dedifferentiated (57.2%), pleomorphic (64.1%), and retroperitoneal (63.9%) tumors had the worse survival. Nationwide, liposarcoma rates increased by 19%, with the annual percent increase (APC) of 1.43% (95% confidence interval (CI): 1.12-1.74). The APC was greater for males vs. females (1.67% vs. 0.89%) and retroperitoneal vs. extremity tumors (1.94% vs. 0.58%). Thus, incidence increased faster in the high-risk subgroup (males), and for retroperitoneal tumors, the low-survival subtype. The SEER generally over-estimated the rates and time trends compared to nationwide data but under-estimated time trends for retroperitoneal tumors. The time trends suggest an interaction between genetic and non-genetic modifiable risk factors may play a role in the etiology of this malignancy. Differences between SEER and CNCPR findings emphasize the need for nationwide cancer surveillance.have a considerable impact on the overall cancer outcomes. Limited treatment options, high costs of existing treatments, and frequent misdiagnosis of rare cancers burden patients, physicians, and health systems [4]. The lack of epidemiological knowledge about the risk factors associated with many rare cancers precludes the development of viable prevention, making it one of the most dramatic unresolved public health problems [1,3,5]. In comparison, well-studied cancers such as colorectal cancer, which constitutes a smaller portion of the overall cancer cases (13%), has had significant progress in cancer control and prevention [5,6]. The existing gaps in research and treatment are reflected in rare cancer survival rates. The 5-year survival for rare cancers is worse compared to common cancers: 49% vs. 63% in Europe [5]; and the U.S., 55% vs. 75% among males and 60% vs. 74% among females [4]. In light of these facts, population-based surveillance of rare cancers and their descriptive epidemiology provide the foundation for advancing etiological and clinical research with the ultimate goal of reducing the public health burden of rare cancers.This research focuses on the under-studied rare cancer...

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confidence: 99%

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001–2016

Bock

Hoffmann

Jiang

et al. 2020

IJERPH

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“…European data were provided by European population‐based cancer registries (CRs) contributing to the Information network on rare cancers (RARECAREnet) database . The RARECAREnet database was derived from the European Cancer Registry‐Based Study on Survival and Care of Cancer Patients (EUROCARE‐5) adult and childhood databases that included 195,263 children and AYAs diagnosed with cancer during 1978 to 2006 and compiled from 14 CRs in 11 countries, with follow‐up available until at least December 31, 2008 (Table ).…”

Section: Methodsmentioning

confidence: 99%

Is the cancer survival improvement in European and American adolescent and young adults still lagging behind that in children?

Trama

Bernasconi

McCabe

et al. 2018

Pediatric Blood & Cancer

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Improvements during 1978 to 2006 in the 5-year survival rate of adolescents and young adults (AYAs, age 15-39) and children with cancers common to both age groups were evaluated for 1978 to 2006 in Europe and the USA. AYAs had absolute survival increases of 25% and 15% in Europe and the USA, respectively, but in both cases, AYA 5-year survival was, as of 2006, 4% lower than those in children. Acute lymphoblastic leukemia (ALL) explained most of the survival difference between AYAs and children on both the continents. In the USA, 20- to 39-year-olds with ALL have had less survival improvement than those in Europe.

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“…Pheochromocytomas and paragangliomas (PCPGs) originate from catecholamine‐producing neuroendocrine cells of the adrenal medulla and extra‐adrenal paraganglia, respectively. Ten to twenty percent of PCPG are metastatic, for those patients the 5‐year survival rate is ~55–70% . Identifying PCPGs with a high risk of metastasis, as well as improved therapy of disseminated disease, are two important unmet needs.…”

Section: Introductionmentioning

confidence: 99%

“…Ten to twenty percent of PCPG are metastatic, for those patients the 5-year survival rate is~55-70%. 1,2 Identifying PCPGs with a high risk of metastasis, as well as improved therapy of disseminated disease, are two important unmet needs. Recent comprehensive analysis by The Cancer Genome Atlas (TCGA) refined our understanding of the biology of PCPGs, that can be divided into three main molecular subtypes: pseudohypoxia (SDHA-D, SDHAF2, FH, MDH2, VHL, EPAS1, EGLN1, and EGLN2), Wntaltered (CSDE1 or MAML3), and kinase-signaling (RET, NF1, TMEM127, MAX, HRAS, FGFR1, MET).…”

Section: Introductionmentioning

confidence: 99%

Long intergenic noncoding RNA profiles of pheochromocytoma and paraganglioma: A novel prognostic biomarker

Ghosal

Das

Pang

et al. 2019

Intl Journal of Cancer

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Many long intergenic noncoding RNAs (lincRNAs) serve as cancer biomarkers for diagnosis or prognostication. To understand the role of lincRNAs in the rare neuroendocrine tumors pheochromocytoma and paraganglioma (PCPG), we performed first time in‐depth characterization of lincRNA expression profiles and correlated findings to clinical outcomes of the disease. RNA‐Seq data from patients with PCPGs and 17 other tumor types from The Cancer Genome Atlas and other published sources were obtained. Differential expression analysis and a machine‐learning model were used to identify transcripts specific to PCPGs, as well as established PCPG molecular subtypes. Similarly, lincRNAs specific to aggressive PCPGs were identified, and univariate and multivariate analysis was performed for metastasis‐free survival. The results were validated in independent samples using RT‐PCR. From a pan‐cancer context, PCPGs had a specific and unique lincRNA profile. Among PCPGs, five different molecular subtypes were identified corresponding to the established molecular classification. Upregulation of 13 lincRNAs was found to be associated with aggressive/metastatic PCPGs. RT‐PCR validation confirmed the overexpression of four lincRNAs in metastatic compared to non‐metastatic PCPGs. Kaplan–Meier analysis identified five lincRNAs as prognostic markers for metastasis‐free survival of patients in three subtypes of PCPGs. Stratification of PCPG patients with a risk‐score formulated using multivariate analysis of lincRNA expression profiles, presence of key driver mutations, tumor location, and hormone secretion profiles showed significant differences in metastasis‐free survival. PCPGs thus exhibit a specific lincRNA expression profile that also corresponds to the established molecular subgroups and can be potential marker for the aggressive/metastatic PCPGs.

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Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study

Cited by 305 publications

References 19 publications

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001–2016

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001–2016

Is the cancer survival improvement in European and American adolescent and young adults still lagging behind that in children?

Long intergenic noncoding RNA profiles of pheochromocytoma and paraganglioma: A novel prognostic biomarker

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