A Case of Medullary Thyroid Carcinoma with Ectopic ACTH Syndrome

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  • TAKAI Shin-ichiro
    The Second Department of Surgery, Osaka University Medical School
  • OGIHARA Toshio
    Department of Medicine and Geriatrics, Osaka University Medical School
  • MIYAUCHI Akira
    The Second Department of Surgery, Osaka University Medical School
  • HIGASHI Hiromu
    The Second Department of Surgery, Osaka University Medical School
  • NISHIMURA Tsuyoshi
    Department of Psychiatry, Osaka University Medical School
  • KAKUDO Kennichi
    the First Department of Pathology, Osaka University Medical School
  • KOSAKI Goro
    The Second Department of Surgery, Osaka University Medical School
  • ABE Kaoru
    Division of Endocrinology, National Cancer Center
  • KAMEYA Toru
    Division of Pathology, National Cancer Center
  • IMURA Hiroo
    the Third Department of Medicine, Kobe University School of Medicine
  • KUMAHARA Yuichi
    Department of Medicine and Geriatrics, Osaka University Medical School

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Other Title
  • 異所性ACTH症候群を呈した甲状腺髄様癌の1例
  • イショセイ ACTH ショウコウグン オ テイシタ コウジョウセン ズイヨウ

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Abstract

A 53-year-old male with Cushing's syndrome due to ectopic ACTH production from medullary carcinoma of the thyroid was reported. The clinical course and results of detailed endocrinological studies and immunohistochemical findings about the cancer tissue were described.<BR>An abnormally high concentration of calcitonin, ACTH and β-MSH in both plasma and cancer tissues (thyroid, lymph nodes and liver) were documented by radioimmunoassay. Urinary 17-OHCS was as high as 38.4 mg/day to 56.0 mg/day and showed no suppression following dexamethasone 8 mg/day administration. Oral metyrapone (3 g/day) caused no response in urinary 17-OHCS. Parallel increments in plasma calcitonin, ACTH and β-MSH were observed following calcium and gastrin loading.<BR>Total thyroidectomy with modified radical neck dissection caused minimal changes of plasma levels of calcitonin, ACTH and β-MSH and no improvement in the clinical manifestations of Cushing's syndrome. An aortogram revealed metastatic tumors in the liver. A second operation, total adrenalectomy, resulted in an improvement of the clinical and laboratory findings such as hypokalemia, high blood pressure, muscle atrophy and moon face.<BR>Immunofluorescent study showed different distribution patterns in calcitonin-and ACTH-positive cells in the primary focus but similar patterns in the liver metastasis.

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