Prospective Study of Thymic Carcinoids in Patients with Multiple Endocrine Neoplasia Type 1
Open Access
- 1 March 2003
- journal article
- other
- Published by The Endocrine Society in Journal of Clinical Endocrinology & Metabolism
- Vol. 88 (3), 1066-1081
- https://doi.org/10.1210/jc.2002-021314
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.Keywords
This publication has 59 references indexed in Scilit:
- CONSENSUS: Guidelines for Diagnosis and Therapy of MEN Type 1 and Type 2Journal of Clinical Endocrinology & Metabolism, 2001
- Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive FormJournal of Clinical Endocrinology & Metabolism, 2001
- Multiple Endocrine Neoplasia Type 1: Clinical and Genetic TopicsAnnals of Internal Medicine, 1998
- Thymic Carcinoids in Multiple Endocrine Neoplasia Type 1Annals of Surgery, 1998
- Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1Journal of Internal Medicine, 1998
- Clinicopathologic Studies of Thymic Carcinoids in Multiple Endocrine Neoplasia Type 1Medicine, 1997
- Zollinger-Ellison syndrome can be the initial endocrine manifestation in patients with multiple endocrine neoplasia-type IAmerican Journal Of Medicine, 1994
- Cause of Death in Multiple Endocrine Neoplasia Type 1Archives of Surgery, 1993
- The Natural History of Multiple Endocrine Neoplasia Type 1Archives of Surgery, 1991
- Mediastinal endocrine neoplasm in patients with multiple endocrine adenomatosis.A previously unrecognized associationCancer, 1972