Shepherd J J, Challis D R, Davies P F, McArdle J P, Teh B T, Wilkinson S
Department of Surgery, University of Tasmania, Hobart, Australia.
Arch Surg. 1993 Oct;128(10):1133-42. doi: 10.1001/archsurg.1993.01420220053007.
We reviewed the age of presentation, malignant potential, and outcome of gastrinomas and pancreatic tumors in patients with multiple endocrine neoplasm, type 1.
Screening of one very large and one smaller, possibly related family on an island, including serum gastrin estimations and, when indicated, pancreatic ultrasound.
Over 2000 family members and their family physicians were advised on screening procedures.
Data were collected and reviewed retrospectively and prospectively for all medical records, investigations, surgical procedures, and available tissue samples.
Criteria for diagnosis were established for radiological, biochemical, and histological studies.
Sixty-two patients had evidence of gastrinoma or pancreatic neoplasm. In 19 patients the diagnosis was based on demonstration of a tumor. In 21 patients the diagnosis was based on elevated serum gastrin concentration in the absence of demonstrable tumor. None of these patients required gastric surgery if they first underwent parathyroidectomy. In 18 patients the diagnosis was based on the combination of demonstrated pancreatic tumor plus elevated glucagon (two patients), gastrin (11 patients), or insulin (five patients) concentration. Peptic ulcer was difficult to control in seven of the 11 patients with elevated gastrin concentrations plus demonstrated tumor. Four patients had liver metastases that appeared to be secondary to the pancreatic gastrinoma. In patients with insulinomas, the first symptoms occurred before age 20 years. Elevated serum gastrin concentrations were not seen before age 24 years and were observed to occur for the first time in two patients after age 50 years.
我们回顾了1型多发性内分泌腺瘤患者中胃泌素瘤和胰腺肿瘤的发病年龄、恶性潜能及预后。
对一个大的和一个较小的、可能相关的岛上家族进行筛查，包括血清胃泌素测定，必要时进行胰腺超声检查。
就筛查程序向2000多名家庭成员及其家庭医生提供了建议。
对所有病历、检查、外科手术及可用组织样本的数据进行回顾性和前瞻性收集与审查。
为放射学、生物化学和组织学研究制定了诊断标准。
62例患者有胃泌素瘤或胰腺肿瘤的证据。19例患者的诊断基于肿瘤的证实。21例患者的诊断基于在未发现可证实肿瘤的情况下血清胃泌素浓度升高。如果这些患者首先接受甲状旁腺切除术，则无需进行胃手术。18例患者的诊断基于已证实的胰腺肿瘤加上胰高血糖素（2例）、胃泌素（11例）或胰岛素（5例）浓度升高。在11例胃泌素浓度升高且有肿瘤证实的患者中，7例的消化性溃疡难以控制。4例患者有肝转移，似乎继发于胰腺胃泌素瘤。胰岛素瘤患者的首发症状出现在20岁之前。血清胃泌素浓度升高在24岁之前未见，在2例50岁以后的患者中首次观察到。
