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Thymic Neuroendocrine Carcinoma (Carcinoid) in Multiple Endocrine Neoplasia Type 1 Syndrome: The Italian Series

Department of Internal Medicine and Endocrine Sciences, Thoracic Surgery, Institute of Pathology University of Perugia (P.Fe., N.A., G.D., F.P., R.R., F.S., G.A.), 06100 Perugia, Italy; Department of Internal Medicine, University of Florence, and Regional Center for Hereditary Endocrine Tumors (A.F., M.L.B.), Florence 50100, Italy; Department of Thoracic Surgery, University of Torino, San Giovanni Battista Hospital (P.Fi.), Torino 10100, Italy; Department of Internal Medicine and Gastroenterology, University of Bologna (P.T.), Bologna 40100, Italy; and Department of Clinical Science, Endocrine Section, University of Rome La Sapienza (G.T.), Rome 00100, Italy

Address all correspondence and requests for reprints to: Dr. P. Ferolla, Department of Internal Medicine and Endocrine Sciences, University of Perugia, Via E. Dal Pozzo, 06100 Perugia, Italy. E-mail: pferolla{at}tin.it.

Neuroendocrine tumors may occur in the setting of multiple endocrine neoplasia type 1 (MEN1) syndrome. Among these, a probably underestimated prevalence of well differentiated neuroendocrine thymic carcinoma (carcinoid), a neoplasm characterized by very aggressive behavior, has been described. We report characterization of the seven Italian cases in which this association occurred among a series of 221 MEN1 patients (41 sporadic and 180 familial cases; prevalence, 3.1%). All of the patients were male, and six of seven (85%) were heavy smokers. No associated hormonal hypersecretion was detected. The first diagnosis was between the second and fifth decades. Familial clusters were present in three of seven (42.8%). No genotype-phenotype correlation was found. All seven cases were associated with hyperparathyroidism. In one patient, prophylactic thymectomy revealed a small nodular lesion suggestive of a thymic carcinoid, providing evidence that preventive thymectomy might prevent additional growth of an occult thymic carcinoid. These findings confirm that thymic carcinoids are associated with a very high lethality, with a near-total prevalence in smoker males. Therefore, prophylactic thymectomy should be considered at neck surgery for primary hyperparathyroidism in MEN1 male patients, especially for smokers, and, due to the frequent familial clusters distribution of this pathology, in subjects with affected relatives presenting this feature. Thus, we recommend screening every patient affected with a neuroendocrine thymic neoplasm for MEN1 syndrome.

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