Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome

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.

Tale of Two Endocrine Organs: Medullary Thyroid Carcinoma (MTC) With Ectopic Cushing Syndrome

“…Various benign and malignant tumors are associated with ectopic ACTH syndrome (EAS) includes small cell carcinoma of lung, carcinoid tumors (lungs, thymus, gastrointestinal tract), islet cell tumors, pheochromocytoma, medullary carcinoma thyroid (MTC), and various miscellaneous tumors like paragangliomas etc [4]. Amongst patients with ectopic Cushing syndrome (ECS), MTC represents 2.2-7.5% of the total cases; however MTC with clinical manifestation of ECS is rare and seen in 0.6% of patients, and has been described in sporadic and familial cases6.…”

“…Various tumours can cause ectopic ACTH production, especially those of neuroendocrine cell origin, which are part of amine precursor uptake and decarboxylation (APUD) system [3]. The most common diseases that cause ectopic ACTH syndrome are Endobronchi¬al carcinoid tumors and Small cell lung cancer and rarely, Medullary carcinoma thyroid (MTC) [4]. MTC is a rare disease with incidence of 3% amongst all thyroid cancer and out of these approximately 0.6% of patients suffer from ectopic ACTH syndrome [5,6].…”

Mucosal Neuroma Cues for Endocrine Emergency Treatment

“…Gyu Gang Choi 1 , Hwan Jin Lee 1 , Hyo Jin Han 1 , Young Beom Jeong 2 , Heung Bum Lee 1 , Ji Hyun Park 1 Departments of 1 Internal Medicine, 2 Urology, Jeonbuk National University Medical School, Jeonju, Korea A 34-year-old man presented with poor oral intake and abdominal pain with bilateral adrenal masses on abdominal computed tomography (CT). He had marfanoid features and mild intellectual disability.…”

Articles in 'Endocrinology and Metabolism' in 2014

“…Other excellent articles on basic endocrine research have also been published [ 60 , 61 , 62 ]. Finally, interesting case reports on patients with various endocrine diseases appeared in 2014 in ' Endocrinology and Metabolism ' [ 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 ].…”