Incidence and survival of rare cancers in the US and Europe

Botta, Laura; Gatta, Gemma; Trama, Annalisa; Bernasconi, Alice; Sharon, Elad; Capocaccia, Riccardo; Mariotto, Angela B.

doi:10.1002/cam4.3137

Cited by 29 publications

(38 citation statements)

References 16 publications

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“…Our results for NOS tumours compare favourably with other registries. There were 3.9% NOS cases for solid cancers (morphology codes: M8000–8001, M8800–8801) which was comparable to a similar study of US and EU cancer registries, which had figures of 3.2 and 9.6% of respectively [ 30 ]. Haematological cancers NOS (morphology codes: M9590–9591, M9760, M9800–9801, M9820 and M9860) were also comparable to the US and EU cancer registries, comprising 8.6% of cases compared to the US and EU proportions of 7.1 and 13.5% respectively [ 30 ].…”

Section: Methodssupporting

confidence: 78%

“…There were 3.9% NOS cases for solid cancers (morphology codes: M8000–8001, M8800–8801) which was comparable to a similar study of US and EU cancer registries, which had figures of 3.2 and 9.6% of respectively [ 30 ]. Haematological cancers NOS (morphology codes: M9590–9591, M9760, M9800–9801, M9820 and M9860) were also comparable to the US and EU cancer registries, comprising 8.6% of cases compared to the US and EU proportions of 7.1 and 13.5% respectively [ 30 ]. The 0.1% of topography NOS cases were also comparable to international studies, such as the RARECARE study, which had 0.7% cases of topography NOS cases [ 2 ].…”

Section: Methodssupporting

confidence: 78%

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Descriptive epidemiological study of rare, less common and common cancers in Western Australia

Bilkey

Trevithick

Coles³

et al. 2021

BMC Cancer

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Background There are no epidemiological studies describing rare cancers in Western Australia (WA). We aimed to fill this gap by estimating the incidence and five-year survival of rare, less common and common cancers in WA, based on definitions for rarity used by the Australian Institute of Health and Welfare and cancer groupings from the project on Surveillance of Rare Cancers in Europe (RARECARE). This research will enable policy- and decision-makers to better understand the size and nature of the public health problem presented by rare cancers in WA. It is anticipated that this study will inform improved health service design and delivery for all WA cancer patients, but particularly those with rare and less common cancers. Methods We estimated incidence and five-year survival rates of rare, less common and common cancers in WA using data sourced from the WA Cancer Registry for the 2013–2017 period. Cancers were defined as rare (< 6), less common (6–12), or common (> 12) based on their crude incidence rate per 100,000 people per year. Results Rare cancers make up 21.5% of all cancer diagnoses in WA, with a significantly poorer five-year survival of 58.2% (95% confidence interval (CI) 57.3–59.1%), compared to patients diagnosed with a common cancer, whose five-year survival was 87.8% (95% CI 87.3–88.3%). Survival for less common cancers was significantly poorer than both rare and common cancers, at 48.1% (95% CI 47.3–49.0%). Together, rare and less common cancers represent 48.4% of all cancer diagnoses in WA. Conclusions While rare cancers are individually scarce, collectively over one in five cancer patients in WA are diagnosed with a rare cancer. These patients experience significantly worse prognoses compared to patients with common cancers.

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Section: Methodssupporting

confidence: 78%

Section: Methodssupporting

confidence: 78%

Descriptive epidemiological study of rare, less common and common cancers in Western Australia

Bilkey

Trevithick

Coles³

et al. 2021

BMC Cancer

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“…Rare cancers are defined as malignancies with an incidence of <6 per 100,000 per year. [1][2][3][4][5] This definition is the result of a consensus effort within the European oncology community that took place in the context of a project funded by the European Union entitled Surveillance of Rare Cancers in Europe (RARECARE).…”

Section: Introductionmentioning

confidence: 99%

“…1,6 The incidence-based criterion for defining rare cancers has been internationally recognized and is currently used in Europe, the United States, and Eastern Asian countries. 5,7,8 In Europe, 12 families of rare cancers were identified with a wide consensus in the context of the Joint Action on Rare Cancers, launched by the European Union. 6 Sarcomas are 1 of the rare cancer families with an incidence of <6 per 100,000.…”

Section: Introductionmentioning

confidence: 99%

“…The definition of rare cancers is based on incidence, and not on prevalence, because it is in rare non‐neoplastic diseases 1,6 . The incidence‐based criterion for defining rare cancers has been internationally recognized and is currently used in Europe, the United States, and Eastern Asian countries 5,7,8 . In Europe, 12 families of rare cancers were identified with a wide consensus in the context of the Joint Action on Rare Cancers, launched by the European Union 6 .…”

Section: Introductionmentioning

confidence: 99%

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Ultra‐rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

Stacchiotti¹,

Frezza²,

Blay

et al. 2021

Cancer

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130

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BACKGROUND: Among sarcomas, which are rare cancers, many types are exceedingly rare; however, a definition of ultra-rare cancers has not been established. The problem of ultra-rare sarcomas is particularly relevant because they represent unique diseases, and their rarity poses major challenges for diagnosis, understanding disease biology, generating clinical evidence to support new drug development, and achieving formal authorization for novel therapies. METHODS: The Connective Tissue Oncology Society promoted

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Extrapleural pneumonectomy for sarcoma: Outcomes of adult patients at a specialized center

Zhang,

Wilson‐Smith,

Connolly

et al. 2024

Cancer Reports

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BackgroundExtrapleural pneumonectomy (EPP) is a complex surgical procedure involving en‐bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural‐based sarcoma of predominantly pediatric patients suggest EPP may be a life‐prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center.MethodsClinicopathologic variables, surgical details, and follow‐up information were extracted for patients undergoing EPP for pleural‐based sarcoma between August 2017 and December 2020. Primary outcomes were event‐free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease‐free interval (DFI) prior to EPP, and early and late postoperative complications.ResultsEight patients were identified, seven with soft tissue sarcoma and one with bone sarcoma. Patients had either localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. All patients underwent resection of their pleural‐based sarcoma by an experienced cardiothoracic surgeon, and some patients had pre or postoperative treatment. The perioperative morbidity was comparable with previously published reports of EPP performed in mesothelioma patients. At median follow‐up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients (75%) had disease recurrence; five (62.5%) died of progressive disease. Two patients (25%) had not recurred: one died of a radiation‐related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low‐grade histology and achieving a metabolic response to preoperative chemotherapy.ConclusionsIn adults with pleural‐based sarcoma, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes. In the absence of a confirmed response to neoadjuvant treatment, long term survival is poor and EPP should not be recommended.

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Incidence and survival of rare cancers in the US and Europe

Cited by 29 publications

References 16 publications

Descriptive epidemiological study of rare, less common and common cancers in Western Australia

Descriptive epidemiological study of rare, less common and common cancers in Western Australia

Ultra‐rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

Extrapleural pneumonectomy for sarcoma: Outcomes of adult patients at a specialized center

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