Two Cases of Ectopic Adrenocorticotropic Hormone Syndrome with Olfactory Neuroblastoma and Literature Review

DOI Web Site 被引用文献1件 参考文献61件
  • KOO Bo Kyung
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital
  • AN Jee Hyun
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital
  • JEON Ki Hyun
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital
  • CHOI Sung Hee
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital
  • CHO Young Min
    Department of Internal Medicine, Seoul National University College of Medicine
  • JANG Hak Chul
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital
  • CHUNG Jin-Haeng
    Department of Pathology, Seoul National University College of Medicine
  • LEE Chol Hee
    Department of Otorhinolanryngology-Head and Neck Surgery, Seoul National University College of Medicine
  • LIM Soo
    Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital

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抄録

Olfactory neuroblastomas are rare, slow-growing malignant tumors, usually diagnosed at advanced stages. Ectopic adrenocorticotropic hormone (ACTH) syndrome caused by an olfactory neuroblastoma is extremely rare. We reported two Korean women who suffered from ectopic ACTH syndrome (EAS) caused by olfactory neuroblastomas. The first patient was a 66-year-old woman who had been diagnosed as olfactory neuroblastoma and refused the management two years before and the second patient was a 37-year-old woman on chemotherapy for olfactory neuroblastoma. In the first case, she presented the Cushingoid appearance with systemic edema and her tumor was removed surgically. ACTH secretion by the tissue was confirmed by immunohistochemistry. By contrast, the second patient presented as severe pneumonia caused by cytomegalovirus and was treated with anti-viral agent followed by chemotherapy and radiotherapy, and her residual mass remained. However, after treatment, both patients' plasma ACTH and cortisol levels returned to normal without any adrenolytic therapy. Considering the causative tumors of EAS can be rarely cured and EAS increases the susceptibility to infections, it is prudent to suppress any hypercortisolemia initially, apart from treating the causal malignancy.<br>

収録刊行物

  • Endocrine Journal

    Endocrine Journal 55 (3), 469-475, 2008

    一般社団法人 日本内分泌学会

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