Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1

Fathia Gibril, Yuan Jia Chen, David S. Schrump, Alexander Vortmeyer, Zhengping Zhuang, Irina A. Lubensky, James C. Reynolds, Adeline Louie, Laurence K. Entsuah, Kane Huang, Behnam Asgharian, Robert T. Jensen

Research output: Contribution to journalArticle

150 Scopus citations

Abstract

Little is known of the natural history of thymic carcinoids in multiple endocrine neoplasia type 1 (MEN1). This is important because in 1993 they were identified as a frequent cause of death, yet only small retrospective studies and case reports exist. We report results of a prospective study of 85 patients with MEN1 evaluated for pancreatic endocrine tumors and followed over a mean of 8 yr with serial chest computed tomography, magnetic resonance imaging (MRI), chest x-ray, and, since 1994, octreoscans [somatostatin receptor scintigraphy (SRS)]. Seven patients (8%) developed thymic carcinoids. Patients with and without carcinoids did not differ in clinical, laboratory, or MEN1 tumor features, except for male gender and the presence of a gastric carcinoid. All thymic tumors were hormonally inactive. Four thymic carcinoids lacked 11q loss of heterozygosity, although it was found in three pancreatic endocrine tumors. Computed tomography and/or MRI were more sensitive than SRS or chest x-ray in detecting tumors initially or with recurrence. All patients underwent resection of the thymic carcinoid, and in all patients followed more than 1 yr, the tumor recurred. Bone metastases developed in two patients and were detected early only on MRI, not SRS. This study provides information on early thymic carcinoids and allows modifications of existing guidelines to be recommended for their diagnosis, surveillance, and treatment.

Original languageEnglish (US)
Pages (from-to)1066-1081
Number of pages16
JournalJournal of Clinical Endocrinology and Metabolism
Volume88
Issue number3
DOIs
StatePublished - Mar 1 2003
Externally publishedYes

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Endocrinology
  • Clinical Biochemistry
  • Biochemistry, medical

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    Gibril, F., Chen, Y. J., Schrump, D. S., Vortmeyer, A., Zhuang, Z., Lubensky, I. A., Reynolds, J. C., Louie, A., Entsuah, L. K., Huang, K., Asgharian, B., & Jensen, R. T. (2003). Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1. Journal of Clinical Endocrinology and Metabolism, 88(3), 1066-1081. https://doi.org/10.1210/jc.2002-021314