Keller U, Fankhauser S, Schumacher A, Trimble E R, Heitz P U
Schweiz Med Wochenschr. 1982 Feb 27;112(9):297-304.
A 60-year-old patient developed signs and symptoms of glucagonoma syndrome (dermatitis, weight loss, anemia and hypoaminoacidemia). However, diabetes mellitus was absent. Glucagonoma was suspected because of markedly elevated plasma glucagon levels and the tumor was subsequently removed by surgery. Acidethanol extraction of the tumor and immunohistochemistry provided evidence of the presence of all four islet hormones, particularly that of glucagon and pancreatic polypeptide and to a lesser extent of somatostatin and insulin. Immunohistochemistry of the tumor (but not plasma) also showed the presence of alpha-HCG. Plasma glucagon immunoreactivity consisted to a large extent (approx. 90%) of a high molecular form of glucagon, probably proglucagon. In spite of the presence of alpha-HCG - which is assumed to be a marker of malignancy - the patient has been free of recurrence for the 2 1/2 years since surgery. The increasing number of cases reported during the past few years demonstrates that the syndrome is more common than previously suspected. Glucagon secretion and its typical clinical picture may be a valuable marker of a multihormonal pancreatic tumor. In a case of suspected glucagonoma, diagnosis can be established simply by obtaining a plasma glucagon level measurement.
一名60岁患者出现了胰高血糖素瘤综合征的体征和症状（皮炎、体重减轻、贫血和低氨基酸血症）。然而，患者并无糖尿病。由于血浆胰高血糖素水平显著升高，怀疑患有胰高血糖素瘤，随后通过手术切除了肿瘤。对肿瘤进行酸乙醇提取和免疫组织化学检测，结果表明所有四种胰岛激素均存在，尤其是胰高血糖素和胰多肽，生长抑素和胰岛素的含量较少。肿瘤（而非血浆）的免疫组织化学检测还显示存在α-HCG。血浆胰高血糖素免疫反应性在很大程度上（约90%）由一种高分子形式的胰高血糖素组成，可能是前胰高血糖素。尽管存在α-HCG（一般认为它是恶性肿瘤的标志物），但该患者自手术以来的2年半时间里未出现复发。过去几年报告的病例数量不断增加，表明该综合征比之前怀疑的更为常见。胰高血糖素分泌及其典型的临床表现可能是多激素胰腺肿瘤的一个重要标志物。对于疑似胰高血糖素瘤的病例，仅通过检测血浆胰高血糖素水平即可确诊。
