Annals of Surgical Oncology

, Volume 23, Issue 2, pp 424–433 | Cite as

The Incidence and Survival of Rare Cancers of the Thyroid, Parathyroid, Adrenal, and Pancreas

  • Benjamin C. James
  • Briseis Aschebrook-Kilfoy
  • Nicole Cipriani
  • Edwin L. Kaplan
  • Peter Angelos
  • Raymon H. Grogan
Endocrine Tumors



With the exception of papillary and follicular thyroid cancer, malignant cancers of the thyroid, parathyroid, adrenal, and endocrine pancreas are uncommon. These rare malignancies present a challenge to both the clinician and patient, because few data exist on their incidence or survival. We analyzed the incidence and survival of these rare endocrine cancers (RECs), as well as the trends in incidence over time.


We used the NCI’s SEER 18 database (2000–2012) to investigate incidence and survival of rare cancers of the thyroid, parathyroid, adrenal, and endocrine pancreas. Cancers were categorized using the WHO classification systems. We collected data on incidence, gender, stage, size, and survival. Time trends were evaluated from 2000–2002 to 2010–2012.


We identified 36 types of rare cancers in the endocrine organs captured in the SEER database. RECs of the thyroid had the highest combined incidence rate (IR 8.26), followed by pancreas (IR 3.24), adrenal (IR 2.71), and parathyroid (IR 0.41). The incidence rate for all rare endocrine organs combined increased 32.4 % during the study period. The majority of the increase was attributable to rare cancers of thyroid, which increased in not only microcarcinomas, but in all sizes. The mean 5-year survival for RECs is 59.56 % (range 2.49–100 %).


This study is a comprehensive analysis of the incidence and survival for rare malignant endocrine cancers. There has been an increase in incidence rate of almost all RECs and their survival is low. We hope that our data will serve as a source of information for clinicians as well as bring awareness regarding these uncommon cancers.


Incidence Rate Adrenal Gland Parathyroid Gland Papillary Thyroid Cancer Parathyroid Carcinoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



This project was supported by the University of Chicago Bucksbaum Institute for Clinical Excellence. RHG was supported by Award Number K12CA139160 from the National Cancer Institute. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Cancer Institute or The National Institutes of Health.


Funding was provided by internal sources from the Department of Surgery at the University of Chicago.

Financial Disclosures

There are no relevant conflicts of interests by any of the authors.


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Copyright information

© Society of Surgical Oncology 2015

Authors and Affiliations

  • Benjamin C. James
    • 1
  • Briseis Aschebrook-Kilfoy
    • 2
  • Nicole Cipriani
    • 3
  • Edwin L. Kaplan
    • 4
  • Peter Angelos
    • 4
  • Raymon H. Grogan
    • 4
  1. 1.Section of Endocrine Surgery, Department of SurgeryIndiana UniversityIndianapolisUSA
  2. 2.Department of Health Studies, Pritzker School of MedicineUniversity of ChicagoChicagoUSA
  3. 3.Department of Pathology, Pritzker School of MedicineUniversity of ChicagoChicagoUSA
  4. 4.Endocrine Surgery Research Program, Department of Surgery, Pritzker School of MedicineUniversity of ChicagoChicagoUSA

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