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Prospective Study of Thymic Carcinoids in Patients with Multiple Endocrine Neoplasia Type 1

Digestive Diseases Branch (F.G., Y-J.C., K.H., L.K.E., B.A., R.T.J.), National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health (NIH); Thoracic Oncology Section (D.S.S.), Surgery Branch, National Cancer Institute, NIH; Molecular Pathogenesis Unit, Surgical Neurology Branch (A.V., Z.Z., I.A.L.) National Institute of Neurological Disorders and Strokes, NIH; Nuclear Medicine Department (J.C.R.) and Diagnostic Radiology Department (A.L.), Warren Grant Magnuson Clinical Center, NIH, Bethesda, Maryland 20892

Address all correspondence and requests for reprints to: Dr. Robert T. Jensen, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Digestive Diseases Branch, Building 10, Room 9C-103, 10 Center Drive, MSC 1804, Bethesda, Maryland 20892-1804. E-mail: robertj{at}bdg10.niddk.nih.gov.

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.

Abbreviations: BAO, Basal acid output; CT, computed tomography; 5-HIAA, 5-hydroxyindolacetic acid; LOH, loss of heterozygosity; MEN1, multiple endocrine neoplasia type 1; MRI, magnetic resonance imaging; PET, pancreatic endocrine tumor; SPECT, single photon emission CT; SRS, somatostatin receptor scintigraphy; ZES, Zollinger-Ellison syndrome.

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