Endocrinol Metab.  2014 Mar;29(1):96-100. 10.3803/EnM.2014.29.1.96.

Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome

• Choi HS
• Kim MJ
• Moon CH
• Yoon JH
• Ku HR
• Kang GW
• Na II
• Lee SS
• Lee BC
• Park YJ
• Kim HI
• Ku YH

• KMID: 2169358
• DOI: http://doi.org/10.3803/EnM.2014.29.1.96

Abstract

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.

Keyword

Cushing syndrome; ACTH syndrome, ectopic; Medullary thyroid carcinoma

MeSH Terms

ACTH Syndrome, Ectopic
Adrenalectomy
Adrenocorticotropic Hormone*
Carcinoid Tumor
Cushing Syndrome
Humans
Hypertension
Korea
Lymph Node Excision
Lymph Nodes
Male
Metastasectomy
Middle Aged
Obesity, Abdominal
Small Cell Lung Carcinoma
Thyroid Gland*
Thyroid Neoplasms*
Thyroidectomy
Adrenocorticotropic Hormone

Figure

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  Fig. 1 Computed tomography of chest and abdomen showed (A) multiple pulmonary metastatic nodules (arrows) and (B) multiple hepatic lesions (arrows).

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  Fig. 2 No adrenal mass was observed on computed tomography of the abdomen.

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  Fig. 3 No pituitary lesion was observed on sellar magnetic resonance imaging.

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  Fig. 4 Immunohistochemistry showed medullary carcinoma, staining positively for (A) calcitonin and (B) chromogranin. (C) Additionally, the carcinoma was negative on adrenocorticotropic hormone stain (×200).

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