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

Endocrine Journal

Bo Kyung KOO¹  Jin-Haeng CHUNG⁴  Jee Hyun AN¹  Young Min CHO²  Ki Hyun JEON¹  Soo LIM¹  Chol Hee LEE³  Sung Hee CHOI¹  Hak Chul JANG¹ 

[1] Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital;Department of Internal Medicine, Seoul National University College of Medicine;Department of Otorhinolanryngology-Head and Neck Surgery, Seoul National University College of Medicine;Department of Pathology, Seoul National University College of Medicine

关键词: Olfactory neuroblastoma;    Ectopic ACTH syndrome;   

DOI  :  10.1507/endocrj.K07E-005

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(36)Cited-By(11)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.

【 授权许可】

Unknown