Two cases of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma and literature review.
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.
Koo
Bo Kyung
BK
Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam-city, South Korea.
An
Jee Hyun
JH
Jeon
Ki Hyun
KH
Choi
Sung Hee
SH
Cho
Young Min
YM
Jang
Hak Chul
HC
Chung
Jin-Haeng
JH
Lee
Chol Hee
CH
Lim
Soo
S
D000182:ACTH Syndrome, Ectopic / Q000000981:diagnostic imaging*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D000368:Aged; D018304:Esthesioneuroblastoma, Olfactory / Q000097:blood / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000652:urine; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000652:urine; D009296:Nasal Cavity / Q000000981:diagnostic imaging* / Q000378:metabolism; D009669:Nose Neoplasms / Q000097:blood / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000652:urine; D011859:Radiography
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone