J Korean Soc Endocrinol.  2006 Feb;21(1):79-84. 10.3803/jkes.2006.21.1.79.

A Case of Multiple Endocrine Neoplasia Type 1 with Papillary Thyroid Carcinoma

• Kim HJ
• Kim CS
• Je HC
• Park J
• Park JS
• Kong JH
• Kang ES
• Ahn CW
• Cha BS
• Lim SK
• Kim KR
• Lee HC
• Jang HS
• Hong SW

• KMID: 2200673
• DOI: http://doi.org/10.3803/jkes.2006.21.1.79

Abstract

This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN 1). It is an hereditary syndrome characterized by neoplastic disorders such as pituitary adenoma, parathyroid adenoma or hyperplasia and pancreatic neuroendocrine tumor, such as gastrinoma just like in our case. But sometimes pheochromocytoma, mucosal ganglioneuromas, lipoma, forgut carcinoid and thyroid disease could be accompany the disease, but coincidental papillary thyroid carcinoma was never reported before in Korea. Herein we represent a 39-year-old woman who manifested typical features of MEN 1 with coincidental papillary thyroid carcinoma. Despite with definite family history of MEN 1, her genetic analysis of DNA had not found any germline mutation in MEN 1 gene. Unidentified culprit gene unable further genetic study of finding LOH (loss of heterogeneity) in 11q13, the possible explanation of papillary thyroid carcinoma as a new component of MEN 1. As we have experienced a case of MEN 1 combined with papillary thyroid carcinoma, we report it with the review of literature.

MeSH Terms

Adult
Carcinoid Tumor
DNA
Female
Ganglioneuroma
Gastrinoma
Germ-Line Mutation
Humans
Hyperplasia
Korea
Lipoma
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Neuroendocrine Tumors
Parathyroid Neoplasms
Pheochromocytoma
Pituitary Neoplasms
Prolactinoma
Thyroid Diseases
Thyroid Gland*
Thyroid Neoplasms*
DNA

Figure

• 

  Fig. 1 Thyroid US shows about 1.5 cm irregular mass at upper pole of right thyroid gland.

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  Fig. 2 Sellar MRI shows 9 × 6 mm sized microadenoma in left portion of pituitary gland.

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  Fig. 3 Abdominal dynamic CT shows 6.8 mm sized arterial enhancing mass at pancreatic body.

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  Fig. 4 Histologic feature of thyroid papillary carcinoma showing inclusion body (H & E stain, × 400).

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  Fig. 5 Histologic feature of parathyroid hyperplasia (H & E stain, × 40).

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