胰升糖素瘤14例病例总结并文献复习

A retrospective study of 14 cases of glucagonoma with literature review

目的探讨胰升糖素瘤的临床特点。方法对14例胰升糖素瘤病例进行回顾性归纳总结。结果(1)男性病例:女性病例=1:1。(2)中位起病年龄47岁(范围33~61岁);中位确诊年龄50岁(范围33~64岁)。(3)最常见的首发症状为坏死松解性游走性红斑(7/14,50%)。(4)确诊时最常见症状为高血糖(14/14,100%),其次为坏死松解性游走性红斑(13/14,92.9%)。(5)就诊我院前的误诊率85.7%(12/14),最常见的误诊诊断为湿疹。(6)从起病到确诊的中位时长4年。(7)确诊时血浆胰升糖素的中位水平为798pg/ml(范围200~2853)。(8)确诊时胰腺肿瘤最长径的中位值为4.0cm(范围2.2~8.0)。(9)57.1%(8/14)的胰腺原发灶位于胰尾部。(10)57.1%(8/14)患者确诊时已存在转移。结论(1)坏死松解性游走性红斑对胰升糖素瘤的诊断较为特异和敏感。(2)胰升糖素瘤的诊断常存在延误的情况。(3)大部分胰升糖素瘤患者在确诊时已存在转移。

Objective To improve the clinical understanding of glucagonoma. Methods A total of 14 cases of glucagonoma were admitted to our hospital, and the clinical features of these cases were retrospectively analyzed. Results (1) The female/ male case ratio was 1 : 1. (2) The median age of onset was 47 years (range 33-61), while the median age at diagnosis was 50 years (range 33-64). (3) The most common initial presentation was necrolytic migratory erythema (7/ 14, 50%). (4) Hyperglycemia was the most common presentation at the time of diagnosis (14/ 14, 100%), followed by necrolytic migratory erythema (13/ 14, 92.9%). (5) The misdiagnosis rate before admission to our hospital was as high as 85.7% (12/ 14), and the most common misdiagnosis was eczema (7/ 12, 58.3%). (6) The median time from onset of probable symptoms to diagnosis was 4 years. (7) The median plasma glucagon level at diagnosis was 798 pg/ ml (range 200-2853). (8) The median length of the longest tumor diameter of the pancreatic tumors at diagnosis was 4. 0cm ( range 2. 2-8. 0). ( 9) 57. 1% of primary pancreatic glucagonomas localized in the tail of the pancreas. (10) 57.1% of patients (8/ 14) had metastases at the time of diagnosis. Conclusions (1)Necrolytic migratory erythema is a relatively sensitive and specific symptom for diagnosis of glucagonoma. (2) The diagnosis for glucagonoma was often delayed, and misdiagnosis was common. ( 3) Most glucagonoma patients had metastases at the time of diagnosis.