Endocrine pancreatic tumors with glucagon hypersecretion:: a retrospective study of 23 cases during 20 years
Endocrine pancreatic tumors with glucagon hypersecretion:: a retrospective study of 23 cases during 20 years
复制标题
DOI：
10.1007/s12032-007-0011-2
复制
发表时间：
2007-01-01
期刊：
影响因子：
3.4
通讯作者：
Eriksson, Barbro
中科院分区：
文献类型：
--
作者：
Kindmark, Henrik;Sundin, Anders;Eriksson, Barbro
背景胰高血糖素分泌性内分泌胰腺肿瘤是一种罕见的疾病，因此缺乏临床治疗的对照研究。为了评估胰高血糖素瘤患者的诊断和治疗措施的有效性，回顾性分析了340例胰腺内分泌肿瘤患者的病历资料，其中23例为胰腺恶性内分泌肿瘤伴胰高血糖素水平升高患者。的组织学证实的肿瘤患者符合我们的胰高血糖素瘤标准。这些患者中只有22%在诊断为胰高血糖素瘤之前患有糖尿病。78%的患者在诊断时有肝脏转移性疾病。52%的患者被诊断为或临床疑似坏死性溶解性游走性红斑。生长抑素受体显像阳性率为95%。19例患者接受了化疗，其中18例为链脲佐菌素和5 FU。通过这种治疗，在50%的可评价患者中观察到客观放射学反应。其他治疗方法包括干扰素、生长抑素类似物、肝动脉栓塞、肝转移瘤射频消融和放射性标记生长抑素类似物。在研究期间，11名患者在诊断后中位数80个月死亡，而11名患者在中位数52个月的随访后仍然活着。结论胰高血糖素瘤占胰腺内分泌肿瘤的7%。坏死溶解性游走性红斑是一种常见的发现（52%），糖尿病的发生率低于以前的报告。肿瘤生长抑素受体显像阳性，化疗客观反应。
Background Glucagon-secreting endocrine pancreatic tumor is a rare disease, hence controlled studies on clinical management are lacking. In an attempt to assess the efficacy of diagnostic and therapeutic measures in patients with glucagonoma, a retrospective study was performed using the archives of a tertiary care center.Patients and methods Records from 340 patients with endocrine pancreatic tumors were reassessed and 23 patients with malignant endocrine pancreatic tumor and elevated plasma glucagon levels were identified.Results About 7% of patients with histologically verified tumors fullfilled our criteria for glucagonoma. Only 22% of these patients had developed diabetes prior to the diagnosis of glucagonoma. Seventy eight percent had metastatic disease to the liver at diagnosis. Necrolytic migratory erythema was diagnosed or clinically suspected in 52%. Somatostatin receptor scintigraphy was positive in 95%. Nineteen patients received chemotherapy at some point, in 18 cases streptozotocin and 5 FU. With this treatment, objective radiological responses were seen in 50% of evaluable patients. Other treatment modalities used were interferon, somatostatin analogs, hepatic artery embolization, radio-frequency ablation of liver metastases, and radiolabeled somatostatin analogs. During the study period, 11 patients died at a median of 80 months from diagnosis whereas 11 patients are still alive after a median follow up of 52 months. One patient was lost to follow-up.Conclusions Glucagonomas represent 7% of our comprehensive referal material of endocrine pancreatic tumors. Necrolytic migratory erythema was a common finding (52%) and diabetes less frequent at presentation than previously reported. Tumors were positive on somatostatin receptor scintigraphy and objective responses were seen to chemotherapy.