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

• Published in: Endocrine Journal Vol. 55; no. 3; pp. 469 - 475
• Main Authors: KOO, Bo Kyung, AN, Jee Hyun, JEON, Ki Hyun, CHOI, Sung Hee, CHO, Young Min, JANG, Hak Chul, CHUNG, Jin-Haeng, LEE, Chol Hee, LIM, Soo
• Format: Journal Article
• Language: English
• Published: Japan The Japan Endocrine Society 2008
• Subjects: ACTH Syndrome, Ectopic - diagnostic imaging; Adrenocorticotropic Hormone - blood; Adult; Aged; Cytomegalovirus; Ectopic ACTH syndrome; Esthesioneuroblastoma, Olfactory - blood; Esthesioneuroblastoma, Olfactory - diagnostic imaging; Esthesioneuroblastoma, Olfactory - metabolism; Esthesioneuroblastoma, Olfactory - urine; Female; Humans; Hydrocortisone - urine; Nasal Cavity - diagnostic imaging; Nasal Cavity - metabolism; Nose Neoplasms - blood; Nose Neoplasms - diagnostic imaging; Nose Neoplasms - metabolism; Nose Neoplasms - urine; Olfactory neuroblastoma; Radiography
• ISSN: 0918-8959; 1348-4540
• DOI: 10.1507/endocrj.K07E-005

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.