Ectopic Cushing's syndrome (CS) occurs rarely in patients with pancreatic neuroendocrine neoplasms. Early recognition of symptoms is critical given the high morbidity and mortality associated with CS. A database of pancreatic neuroendocrine neoplasms (NENs) seen at the Moffitt Cancer Center between 1/2008 and 4/2022 was reviewed and cases of ectopic CS were identified. Information was extracted on tumor characteristics, clinical signs and symptoms, therapies, and outcomes. Thirteen cases were identified, ranging in age from 16 to 65 years at the initial time of diagnosis (median 42). Twelve of 13 patients had metastatic tumors at presentation. All were well-differentiated at diagnosis although two were described as transformed to poorly differentiated on rebiopsy. A total of 4 patients also experienced Zollinger-Ellison syndrome. Three patients underwent bilateral adrenalectomy to manage uncontrolled CS. Median overall survival of was 56 months from the time of initial cancer diagnosis but only 18 months from diagnosis of CS. Our study showed that ectopic CS is a highly morbid condition when occurring in pancreatic NENs and is associated with aggressive disease. Bilateral adrenalectomy can be considered for syndrome control. To our knowledge, this is the largest institutional case-series of ACTH-secreting metastatic pancreatic NEN.

中文翻译：

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分泌 ACTH 的胰腺神经内分泌肿瘤：病例系列

异位库欣综合征（CS）很少发生在胰腺神经内分泌肿瘤患者中。鉴于 CS 相关的高发病率和死亡率，早期识别症状至关重要。对 2008 年 1 月至 2022 年 4 月间在莫菲特癌症中心发现的胰腺神经内分泌肿瘤 (NEN) 数据库进行了审查，并确定了异位 CS 病例。提取有关肿瘤特征、临床体征和症状、治疗和结果的信息。已确定 13 例病例，初次诊断时年龄从 16 岁到 65 岁不等（中位数 42 岁）。13 名患者中有 12 名就诊时患有转移性肿瘤。尽管有两个在重新活检时被描述为分化不良，但所有患者在诊断时均分化良好。共有 4 名患者同时出现佐林格-埃里森综合征。三名患者接受了双侧肾上腺切除术以治疗不受控制的 CS。从初次癌症诊断起，中位总生存期为 56 个月，但从诊断出 CS 起，中位总生存期仅为 18 个月。我们的研究表明，异位 CS 发生在胰腺 NEN 中时是一种高度发病的病症，并且与侵袭性疾病相关。可考虑进行双侧肾上腺切除术来控制综合征。据我们所知，这是分泌 ACTH 的转移性胰腺 NEN 的最大机构病例系列。