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
Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam-city, South Korea.
Endocr J. 2008 Jul;55(3):469-75. doi: 10.1507/endocrj.k07e-005. Epub 2008 May 9.
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.
嗅神经母细胞瘤是一种罕见的、生长缓慢的恶性肿瘤，通常在晚期才被诊断出来。由嗅神经母细胞瘤引起的异位促肾上腺皮质激素（ACTH）综合征极为罕见。我们报告了两名患有嗅神经母细胞瘤所致异位ACTH综合征（EAS）的韩国女性。首例患者是一名66岁女性，两年前被诊断为嗅神经母细胞瘤但拒绝接受治疗；第二例患者是一名37岁女性，正在接受嗅神经母细胞瘤化疗。在第一例病例中，她出现了库欣样面容并伴有全身性水肿，其肿瘤通过手术切除。组织的ACTH分泌通过免疫组织化学得以证实。相比之下，第二例患者表现为巨细胞病毒引起的重症肺炎，接受抗病毒药物治疗后接着进行化疗和放疗，其残留肿块依然存在。然而，治疗后，两名患者的血浆ACTH和皮质醇水平均恢复正常，且未进行任何肾上腺溶解疗法。鉴于EAS的致病肿瘤很少能被治愈，且EAS会增加感染易感性，除了治疗病因性恶性肿瘤外，最初谨慎地抑制任何高皮质醇血症是明智的。
