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Hormones and Cancer

, Volume 7, Issue 4, pp 279–287 | Cite as

Clinical Features, Treatments, and Outcomes of Patients with Thymic Carcinoids and Multiple Endocrine Neoplasia Type 1 Syndrome at MD Anderson Cancer Center

  • Ioannis Christakis
  • Wei Qiu
  • Angelica M. Silva Figueroa
  • Samuel Hyde
  • Gilbert J. Cote
  • Naifa L. Busaidy
  • Michelle Williams
  • Elizabeth Grubbs
  • Jeffrey E. Lee
  • Nancy D. PerrierEmail author
Original Paper

Abstract

Thymic carcinoids are rare neuroendocrine tumors that occur in 1–5 % of patients with multiple endocrine neoplasia type 1 (MEN1) and are a major cause of morbidity and mortality. The few published reports associate these tumors with male sex and smoking. Our objective was to describe cases of these tumors treated at our institution. We performed a retrospective chart review of all patients diagnosed with MEN1 at our tertiary referral center from 1980 to 2014. Patients with a histopathologic, fine-needle aspiration, or clinical diagnosis of a thymic carcinoid were included. Two hundred ninety-one patients fulfilled the criteria for a diagnosis of MEN1. Clinicopathologic characteristics, MEN1 genetic testing results, treatments, and survival rates were analyzed. Nine patients had a thymic carcinoid, six men (67 %) and three women (33 %). Six patients were non-smokers (67 %). Two patients had synchronous (22 %) and eight patients (89 %) had metachronous distant metastasis. The 10-year overall survival rate was 45 % (lower 95 % upper 95 % CI 20–100 %). The 10-year disease-free survival rate was 42 % (lower 95 % upper 95 % CI 15–100 %). Five patients had MEN1 genetic testing, and the genotypes of affected individuals were p.W341X, c.275_286delGCTTCACCGCCC, p.R98X, c.1350+(1_11)del11, and partial duplication of exons 9 and 10. A higher percentage of MEN1-related thymic carcinoids can occur in women and in non-smokers than previously reported. Both novel and known mutations were present in our cohort. Eighty nine percent of patients developed a metachronous metastasis from the thymic carcinoid. Patients with MEN1 and thymic carcinoids should be followed closely.

Keywords

Synchronous Metastasis Metachronous Metastasis Large Cell Neuroendocrine Carcinoma Thymic Carcinoid Ominous Prognosis 
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.

Notes

Acknowledgments

This work was supported by the National Institutes of Health/National Cancer Institute under award number P30CA016672 (and using the Clinical Trials Support Resource) and by the Cancer Center Support Grant (National Cancer Institute Grant P30CA016672).

Author Contributions

Ioannis A. Christakis: collection, management, analysis, and interpretation of the data and preparation, review, or approval of the manuscript

Wei Qiu: collection, management, interpretation of the data and preparation, review, or approval of the manuscript

Angelica M. Silva Figueroa: collection, management, interpretation of the data and preparation, review, or approval of the manuscript

Samuel Hyde: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Gilbert J. Cote: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Naifa L. Busaidy: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Michelle Williams: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Jeffrey E. Lee: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Nancy D. Perrier: analysis and interpretation of the data and preparation, review, or approval of the manuscript

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflicts of interest.

Supplementary material

12672_2016_269_MOESM1_ESM.docx (34 kb)
Supplementary Table 1 (DOCX 33 kb)

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Ioannis Christakis
    • 1
  • Wei Qiu
    • 1
    • 2
  • Angelica M. Silva Figueroa
    • 1
  • Samuel Hyde
    • 3
  • Gilbert J. Cote
    • 3
  • Naifa L. Busaidy
    • 3
  • Michelle Williams
    • 4
  • Elizabeth Grubbs
    • 1
  • Jeffrey E. Lee
    • 1
  • Nancy D. Perrier
    • 1
    Email author
  1. 1.Department of Surgical OncologyThe University of Texas MD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Hepatobiliary Pancreatic SurgeryThe First Hospital of Jilin UniversityChangchunChina
  3. 3.Department of Endocrine Neoplasia and Hormonal DisordersThe University of Texas MD Anderson Cancer CenterHoustonUSA
  4. 4.Department of PathologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

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