Abstract：
Pancreatic neuroendocrine tumors (PanNETs) are rare malignant tumors that occur in the pancreas. They are divided into functioning and non-functioning tumors based on the presence or absence of their specific hormonal hyper-expression symptoms. Adrenocorticotropic hormone (ACTH)-producing PanNETs are rare, functional tumors, and their clinical characteristics and outcomes have not been well reported.Here, we report the cases of two patients with PanNETs who presented with ectopic ACTH syndrome (EAS) during the course of their disease. Case 1 involved a non-functioning PanNET at the time of surgery. During treatment for recurrent liver metastases, the patient presented with EAS and tumor-associated hypercalcemia, probably due to ACTH and parathyroid hormone-related peptide (PTHrP) production from the liver tumor. Case 2 was a gastrinoma, and similar to Case 1, this patient presented with EAS during the treatment of recurrent liver metastases.It is not uncommon for patients with PanNETs to have multiple hormones and develop secondary hormone secretion during their disease course, although tumor phenotypes differ between primary and metastatic sites. In patients with functioning PanNETs, symptom control with anti-hormonal therapy is essential, in addition to anti-tumor therapy, especially for EAS, which is an endocrine emergency disease that requires prompt diagnosis and treatment.
摘要：
胰腺神经内分泌肿瘤（PanNETs）是胰腺中罕见的恶性肿瘤。根据其特定激素高表达症状的存在或不存在，将其分为功能性和非功能性肿瘤。产生促肾上腺皮质激素（ACTH）的PanNETs很少见，功能性肿瘤，他们的临床特征和结局没有得到很好的报道.这里,我们报道了2例PanNETs患者在病程中出现异位ACTH综合征(EAS)的病例.病例1涉及手术时无功能的PanNET。在治疗复发性肝转移期间，患者出现EAS和肿瘤相关高钙血症，可能是由于肝肿瘤产生的ACTH和甲状旁腺激素相关肽（PTHrP）。病例2是胃泌素瘤,与病例1相似，该患者在治疗复发性肝转移期间出现EAS。PanNETs患者在病程中出现多种激素并产生次级激素分泌的情况并不少见，尽管肿瘤表型在原发部位和转移部位之间不同。在功能正常的PanNETs患者中，抗激素治疗的症状控制至关重要，除了抗肿瘤治疗，尤其是对EAS来说,这是一种需要及时诊断和治疗的内分泌急诊疾病。
