Head and Neck

Insular thyroid carcinoma in a patient with Cowden syndrome†‡

  1. Henry R. Diggelmann MD1,
  2. Douglas J. Van Daele MD1,
  3. Thomas M. O'Dorisio MD2,
  4. Henry T. Hoffman MD, MS, FACS1,*

Article first published online: 18 DEC 2009

DOI: 10.1002/lary.20507

Issue

The Laryngoscope

The Laryngoscope

Volume 120, Issue 3, pages 454–457, March 2010

Additional Information

How to Cite

Diggelmann, H. R., Van Daele, D. J., O'Dorisio, T. M. and Hoffman, H. T. (2010), Insular thyroid carcinoma in a patient with Cowden syndrome. The Laryngoscope, 120: 454–457. doi: 10.1002/lary.20507

Author Information

  1. 1

    Department of Otolaryngology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, U.S.A.

  2. 2

    Department of Endocrinology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, U.S.A.

*The University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52242

  1. †

    Presented at the Triological Society Combined Middle and Southern Sections Meeting, Bonita Springs, Florida, U.S.A., January 8–11, 2009.

  2. ‡

    All of the work was done at this institution. There was no financial support.

Publication History

  1. Issue published online: 16 FEB 2010
  2. Article first published online: 18 DEC 2009
  3. Manuscript Accepted: 26 MAR 2009

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Keywords:

  • Cowden syndrome;
  • hamartomas;
  • insular thyroid cancer;
  • phosphatase and tensin homolog (PTEN);
  • mutation

Abstract

Cowden syndrome is an autosomal dominant disorder characterized by benign and malignant hamartomatous lesions that can develop from all three germ cell derivatives. This disorder predisposes patients to develop malignant tumors of the breast, endometrium, and thyroid. We present a patient with clinically relevant manifestations of Cowden syndrome, with genetic verification, impacting by way of airway compromise due to hamartomas, urinary tract abnormalities, and insular thyroid cancer. This case illustrates the value of recognizing Cowden syndrome at an earlier stage when the patient could have received appropriate management to decrease the morbidity of untreated hamartomatous growths, and an elective thyroidectomy would have been a viable option to manage his malignancy. Through this case report, we provide further insight into management of this disorder. Laryngoscope, 2010

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