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Review
. 2021 Jul;30(7):1305-1311.
doi: 10.1158/1055-9965.EPI-20-1796. Epub 2021 Apr 1.

Epidemiologic Research of Rare Cancers: Trends, Resources, and Challenges

Lisa Gallicchio  1 , Danielle L Daee  2 , Melissa Rotunno  2 , Rolando Barajas  2 , Sarah Fagan  2 , Danielle M Carrick  2 , Rao L Divi  2 , Kelly K Filipski  2 , Andrew N Freedman  2 , Elizabeth M Gillanders  2 , Tram K Lam  2 , Damali N Martin  2 , Scott Rogers  2 , Mukesh Verma  2 , Stefanie A Nelson  2
Affiliations
Review

Epidemiologic Research of Rare Cancers: Trends, Resources, and Challenges

Lisa Gallicchio et al. Cancer Epidemiol Biomarkers Prev. 2021 Jul.

Abstract

Background: The goals of this project were to assess the status of NCI's rare cancer-focused population science research managed by the Division of Cancer Control and Population Sciences (DCCPS), to develop a framework for evaluation of rare cancer research activities, and to review available resources to study rare cancers.

Methods: Cancer types with an overall age-adjusted incidence rate of less than 20 cases per 100,000 individuals were identified using NCI Surveillance, Epidemiology and End Results (SEER) Program data. SEER data were utilized to develop a framework based on statistical commonalities. A portfolio analysis of DCCPS-supported active grants and a review of three genomic databases were conducted.

Results: For the 45 rare cancer types included in the analysis, 123 active DCCPS-supported rare cancer-focused grants were identified, of which the highest percentage (18.7%) focused on ovarian cancer. The developed framework revealed five clusters of rare cancer types. The cluster with the highest number of grants (n = 43) and grants per cancer type (10.8) was the cluster that included cancer types of higher incidence, average to better survival, and high prevalence (in comparison with other rare cancers). Resource review revealed rare cancers are represented in available genomic resources, but to a lesser extent compared with more common cancers.

Conclusions: This article provides an overview of the rare cancer-focused population sciences research landscape as well as information on gaps and opportunities.

Impact: The findings of this article can be used to develop efficient and comprehensive strategies to accelerate rare cancer research.See related commentary by James V. Lacey Jr, p. 1300.

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Conflict of interest statement

Conflict of interest statement: The authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
Clusters of rare cancer types based on statistical commonalities. Heat map was generated utilizing the R statistical package. Data were coded so that the data points indicating ‘worse’ individual or public health cancer burden had a higher absolute value and are colored in red on the heatmap. Data points indicating ‘better’ individual or public health cancer burden are colored in blue. Boundaries of the clusters were identified using visualization of the heatmap.
Figure 2.
Figure 2.
Number of active rare cancer-focused grants managed by the National Cancer Institute’s Division of Cancer Control and Population Sciences, by cancer type. A grant may include several rare cancer types. CNS or other NS = Central Nervous System or other Nervous System; NOS = not otherwise specified; NHL= Non-Hodgkin Lymphoma
Figure 3.
Figure 3.
Common Scientific Outline broad scientific area code fractions for active rare cancer-focused grants, by cancer type. CNS or other NS = Central Nervous System or other Nervous System; NOS = not otherwise specified; NHL= Non-Hodgkin Lymphoma
See this image and copyright information in PMC

Comment in

  • A Rare Cancer Opportunity.
    Lacey JV Jr. Lacey JV Jr. Cancer Epidemiol Biomarkers Prev. 2021 Jul;30(7):1300-1301. doi: 10.1158/1055-9965.EPI-21-0427. Cancer Epidemiol Biomarkers Prev. 2021. PMID: 34210679

References

    1. Greenlee RT, Goodman MT, Lynch CF, Platz CE, Havener LA, Howe HL. The occurrence of rare cancers in U.S. adults, 1995–2004. Public Health Rep 2010;125:28–43. - PMC - PubMed
    1. DeSantis CE, Kramer JL, Jemal A. The burden of rare cancers in the United States. CA Cancer J Clin 2017;67:261–72. - PubMed
    1. Howlader N, Noone AM, Krapcho M et al. (eds). SEER Cancer Statistics Review, 1975–2016. National Cancer Insitute. Bethesda, MD, https://seer.cancer.gov/csr/1975_2016/, based on November 2018 SEER data submission, posted to the SEER website, April 2019.

MeSH terms