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Bronchopulmonary carcinoid in multiple endocrine neoplasia type 1†

  1. Nirupa Sachithanandan M.B.B.S.1,
  2. Robin A. Harle M.B.B.S.2,3,
  3. John R. Burgess M.D.1,4,‡,*

Article first published online: 20 DEC 2004

DOI: 10.1002/cncr.20825

Issue

Cancer

Cancer

Volume 103, Issue 3, pages 509–515, 1 February 2005

Additional Information

How to Cite

Sachithanandan, N., Harle, R. A. and Burgess, J. R. (2005), Bronchopulmonary carcinoid in multiple endocrine neoplasia type 1. Cancer, 103: 509–515. doi: 10.1002/cncr.20825

Author Information

  1. 1

    Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, Tasmania, Australia

  2. 2

    Department of Radiology, Royal Hobart Hospital, Hobart, Tasmania, Australia

  3. 3

    Discipline of Surgery, University of Tasmania, Hobart, Tasmania, Australia

  4. 4

    Discipline of Medicine, University of Tasmania, Hobart, Tasmania, Australia

  1. ‡

    Fax: (011) 613 62310154

*Department of Diabetes and Endocrinology, Royal Hobart Hospital/University of Tasmania, G.P.O. Box 1061L Hobart, Tasmania 7001, AustraliaE-mail

  1. †

    Preliminary results in relation to this work were presented at the Annual Scientific Meeting of the Endocrine Society of Australia, Melbourne, Australia, September 14–17, 2003.

Publication History

  1. Issue published online: 20 JAN 2005
  2. Article first published online: 20 DEC 2004
  3. Manuscript Accepted: 21 OCT 2004
  4. Manuscript Revised: 18 OCT 2004
  5. Manuscript Received: 24 AUG 2004

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Keywords:

  • multiple endocrine neoplasia type1;
  • bronchopulmonary;
  • carcinoid;
  • prevalence;
  • screening

Abstract

BACKGROUND

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal-dominant syndrome associated with neoplasia of pituitary, pancreas, parathyroid, and foregut lineage neuroendocrine tissue. Although enteropancreatic carcinoid has been well described in patients with MEN 1, it was believed that bronchopulmonary carcinoid was relatively uncommon, occurring in approximately 5% of patients. It is unclear whether the increased screening of asymptomatic patients with MEN 1 will facilitate early diagnosis of this tumor and improve patient prognosis.

METHODS

The authors reviewed the patient records and, when available, thoracic computed tomographic (CT) images of 129 MEN 1-affected adult members of a single family to determine the prevalence and prognosis of bronchopulmonary nodules and carcinoid.

RESULTS

Among 129 patients, a diagnosis of bronchopulmonary carcinoid was noted in the records for 6 individuals (1 male and 5 females; 5%). Thoracic CT scans also were available for review from 32 of those patients. Twelve patients (38%) had pulmonary nodules evident on CT scans. Only hypergastrinemia was significantly more common in patients with pulmonary nodules; otherwise, the spectrum of neoplasia was similar between individuals with and without pulmonary lesions. Histologic diagnoses were available in four patients (three female) with abnormal CT images, and carcinoid was confirmed in each patient. No deaths or distant metastases occurred among the patients despite long-term follow-up (mean, 127 months).

CONCLUSIONS

The findings suggested that bronchopulmonary carcinoid is more prevalent in patients with MEN 1 than was recognized previously. Furthermore, the diagnosis did not appear to portend a poor prognosis in the majority of affected patients. Cancer 2005. © 2004 American Cancer Society.

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