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Abstract
Multiple endocrine neoplasia type 1 (MEN 1) is characterized by the combined occurrence of primary hyperparathyroidism, enteropancreatic tumors and anterior pituitary adenoma. Yet carcinoid tumors, adrenal adenoma and lipoma might exist simultaneously. Thymic carcinoid tumors, which are recognized as one of the causes of death for patients with MEN 1, are uncommon and their natural history has barely been investigated.
A 51-year-old man, who had undergone surgery and radiation therapy for thymic carcinoid tumor 15 years ago, was admitted to our hospital due to hypercalcemia that was detected on a routine health checkup. We found that he had hyperparathyroidism, a nonfunctioning pancreatic tumor and thyroid papillary microcarcinoma. We had studied the genetic alteration of the MEN 1 gene (menin) by using leukocyte genomic DNA. This genetic analysis clearly showed the missense mutation of the MEN 1 gene (V215M on exon 3). The thymic carcinoid tumor in this patient showed a relatively indolent course, in contrast to that for the ordinary MEN 1 patient, and this patient's thyroid papillary microcarcinoma was discovered incidentally. We present here this atypical case along with a review of the relevant literature.
Figures and Tables
Fig. 1
Thyroid sonography findings. A. Thyroid sonography showed about 1.2 × 0.7 cm sized low echogenic mass in the low portion of the right thyroid. B. About 1.1 × 1.2 cm sized low echogenic mass was found in the upper portion of the left thyroid.
Fig. 2
Parathyroid gland scintigraphy using 99mTc-sestamibi. A. The early image (10 min) showed a nodular hot uptake in the middle portion of the right thyroid, upper and lower poles of the left thyroid gland. B. In delayed image (2 hours), there was a hot uptake in the lower pole of the right thyroid gland.
Fig. 3
Abdominal dynamic computed tomography. A. A large mass (6.8 cm) was noted in left paraaortic and subphrenic space on pre-enhance phase image. B. It was well-circumscribed, large hypervascular mass with heterogeneous high enhancement on arterial phase image.
Fig. 4
Histologic feature of thyroid papillary carcinoma and parathyroid gland. A. Low power view showed focus of micropapillary thyroid carcinoma having the classic papillary growth pattern (H & E stain, ×100). B. The parathyroid adenoma was composed mainly of chief cells. They had uniformly round nuclei and a moderate amount of cytoplasm (H & E stain, ×100).
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