The Journal of the Japanese Association for Chest Surgery
Online ISSN : 1881-4158
Print ISSN : 0919-0945
ISSN-L : 0919-0945
Atypical thymic carcinoid in type I multiple endocrine neoplasia syndrome: Case report
Katsuhito UenoYoshihiro MurotaHirokazu UrushiyamaYoshihito YamadaTetsuo YamaguchiYasuhiro Takeuchi
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2010 Volume 24 Issue 2 Pages 181-186

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Abstract

We report the rare case of a 40-year-old man with an anterior mediastinal tumor diagnosed postoperatively as atypical thymic carcinoid in sporadic multiple endocrine neoplasm-type 1. He had a history of diabetes mellitus, and an asymptomatic anterior mediastinal tumor was demonstrated on routine radiography. Chest computed tomography revealed an anterior mediastinal mass of 5.5 cm in diameter. Diagnostic tumor resection was performed employing video-assisted thoracoscopic surgery. The tumor was located in the right lobe of the thymus. The tumor was completely encapsulated, and no sign of local invasion was found. The preliminary pathological diagnosis during the operation was thymic carcinoid or type A thymoma. The diagnosis was confirmed as atypical thymic carcinoid based on the permanent preparation, and there was microscopic invasion into the capsule. Therefore, an additional operation was scheduled and extended total thymectomy was performed through a median sternotomy. The mediastinal regional lymph nodes and fibroadipose tissue were resected. No lymph node metastasis was identified in the permanent specimen. The patient received adjuvant mediastinal irradiation. A parathyroid tumor and pancreatic tumor were pointed out on examination for increases in calcium, intact-PTH, growth hormone, gastrin, and prolactin. The diagnosis of multiple endocrine neoplasm type 1 (MEN-1) was confirmed. Total parathyroidectomy with autotransplantation of the parathyroid gland was performed, and pathological examination revealed hyperplasia. The pancreatic tumor was small, and has been under observation, being suspected to be a gastrinoma. He is currently alive and no sign of recurrence has occurred to date at 19 months after the initial detection and treatment. His glucose tolerance improved after thymectomy and parathyroidectomy. Extended total thymectomy at an early stage in combination with radiotherapy can be effective for patients with MEN-1-related thymic carcinoid.

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© 2010 The Japanese Association for Chest Surgery
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