背景:异位分泌促肾上腺皮质激素(ACTH)的神经内分泌肿瘤是罕见的疾病。分泌ACTH的胰腺神经内分泌癌患者预后不良。据报道,感染和凝血病是死亡原因。然而,分泌ACTH的神经内分泌癌的病态并发症的详细临床描述尚未报道.
方法:一名78岁的日本妇女因全身水肿和上腹部不适而咨询医疗中心。实验室分析显示,皮质醇血症伴有ACTH分泌增加,血清皮质醇水平无昼夜变化。增强的计算机断层扫描(CT)扫描显示胰头有3厘米的肿瘤。内窥镜超声引导下细针抽吸的细胞学材料与分泌ACTH的胰腺神经内分泌癌兼容。Ki-67指数为40%。她被转移到Mie大学医院接受手术治疗。病人被诊断为尿路感染,巨细胞病毒肝炎,食管念珠菌病,肺浸润怀疑卡氏肺孢子虫肺炎,外周深静脉血栓形成,肺栓塞,和弥散性血管内凝血。多器官感染和血栓形成对抗菌和抗凝治疗反应良好。放射性同位素研究揭示了胰腺肿瘤和肝脏转移灶,而生长抑素受体闪烁显像显示阴性结果,提示原发性和转移性肿瘤分化差。入院前的CT扫描显示无转移性肝脏病变,提示胰腺肿瘤进展迅速。而不是手术,抗肿瘤化疗。患者被转移到另一家医院开始化疗。然而,4个月后她死于肿瘤进展迅速.
结论:分泌ACTH的胰腺神经内分泌肿瘤是一种罕见的疾病,预后极差。肿瘤的临床过程和急性并发症仍未报道。在这里,我们报告了一个快速进展的ACTH分泌胰腺神经内分泌肿瘤的临床过程,该肿瘤由于多器官中的许多类型的病原体而出现感染性并发症。广泛的血栓形成,肺栓塞,和弥散性血管内凝血。
BACKGROUND: Ectopic adrenocorticotropic hormone (ACTH)-secreting neuroendocrine tumors are rare diseases. Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis. Infections and coagulopathies have been reported as the cause of death. However, detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.
METHODS: A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort. Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level. An enhanced computed tomography (CT) scan revealed a 3-cm tumor in the pancreatic head. The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTH-secreting pancreatic neuroendocrine carcinoma. The Ki-67 index was 40%. She was transferred to Mie University Hospital for surgical treatment. The patient was diagnosed with urinary tract infection, cytomegalovirus hepatitis, esophageal candidiasis, pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia, peripheral deep vein thrombosis, pulmonary embolism, and disseminated intravascular coagulation. The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy. Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver, whereas somatostatin receptor scintigraphy showed negative findings, suggesting the primary and metastatic tumors were poorly differentiated. A CT scan before admission showed no metastatic liver lesion, suggesting that the pancreatic tumor was rapidly progressing. Instead of surgery, antitumor chemotherapy was indicated. The patient was transferred to another hospital to initiate chemotherapy. However, she died four months later due to the rapidly progressive tumor.
CONCLUSIONS: ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis. The clinical course and acute complications of the tumor remain unreported. Here we report the clinical course of a rapidly progressive
case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs, widespread thromboses, pulmonary embolism, and disseminated intravascular coagulation.