嗅神经母细胞瘤很少分泌促肾上腺皮质激素，导致异位促肾上腺皮质激素综合征。然而，嗅神经母细胞瘤患者异位促肾上腺皮质激素综合征的患病率、发生时间和触发因素仍不清楚。本研究旨在调查这些因素并进行文献综述。包括 15 名在我们机构接受手术的嗅神经母细胞瘤患者。通过使用免疫组织化学评估促肾上腺皮质激素表达来评估异位促肾上腺皮质激素综合征发生的患病率。此外，还回顾了之前报道中出现异位促肾上腺皮质激素综合征的 26 名嗅神经母细胞瘤患者。在这 15 名患者中，三名患者 (20%) 在初次手术时显示促肾上腺皮质激素阳性肿瘤细胞，两名 (13%) 出现异位促肾上腺皮质激素综合征。发生异位促肾上腺皮质激素综合征的时间为嗅神经母细胞瘤初次治疗后 2.5 年和 10 年。根据文献综述，9例复发转移性嗅神经母细胞瘤患者在初次手术后出现异位促肾上腺皮质激素综合征，其中3例在发生异位促肾上腺皮质激素综合征后确诊，3例在疾病进展过程中发生，2例在接受化疗后发生，其中一种是在活检后出现的。异位促肾上腺皮质激素综合征的发生时间为初次治疗后2.5-15年。我们的研究表明，承认嗅神经母细胞瘤可以表现为异位促肾上腺皮质激素综合征，且患病率一定较低，这一点至关重要。此外，我们的研究推测肿瘤刺激，如活检或化疗，以及疾病进展，可能引发异位促肾上腺皮质激素综合征的发作。因此，即使在初始治疗后很长时间，嗅神经母细胞瘤也可能发展为异位促肾上腺皮质激素综合征。

Olfactory neuroblastomas rarely secrete adrenocorticotropic hormone, leading to ectopic adrenocorticotropic hormone syndrome. However, the prevalence, timing, and triggers of ectopic adrenocorticotropic hormone syndrome in patients with olfactory neuroblastomas remain unclear. This study aimed to investigate these factors and conduct a literature review. Fifteen patients with olfactory neuroblastomas who underwent surgery at our institution were included. The prevalence of ectopic adrenocorticotropic hormone syndrome development was assessed by evaluating adrenocorticotropic hormone expression using immunohistochemistry. Furthermore, 26 patients with olfactory neuroblastomas who developed ectopic adrenocorticotropic hormone syndrome from previous reports were reviewed. Among the 15 patients, three (20%) showed adrenocorticotropic hormone-positive tumor cells at the time of initial surgery, and two (13%) developed ectopic adrenocorticotropic hormone syndrome. The timing of developing ectopic adrenocorticotropic hormone syndrome was 2.5 and 10 years following initial treatment of olfactory neuroblastoma. Based on the literature review, nine patients with recurrent and metastatic olfactory neuroblastoma developed ectopic adrenocorticotropic hormone syndrome after the initial surgery, of whom, three had confirmed disease after developing ectopic adrenocorticotropic hormone syndrome, three developed during disease progression, two developed after receiving chemotherapy, and one developed after undergoing a biopsy. The timing of ectopic adrenocorticotropic hormone syndrome was 2.5-15 years after initial treatment. Our study revealed that acknowledging olfactory neuroblastomas can manifest as ectopic adrenocorticotropic hormone syndrome with a certain low prevalence is crucial. Moreover, our study speculated that tumor stimulation, such as biopsy or chemotherapy, as well as disease progression, could trigger ectopic adrenocorticotropic hormone syndrome onset. Thus, olfactory neuroblastomas can develop into ectopic adrenocorticotropic hormone syndrome, even long after the initial treatment.