Wilson S D
Surgery. 1982 Oct;92(4):682-92.
Long-term follow-up of children with the Zollinger-Ellison syndrome (gastrinomas) suggests that surgical management is still advantageous. Twenty-eight children with the Zollinger-Ellison syndrome have been followed up to 21 years after their initial surgical procedure. Six of seven children with less than total gastrectomy, all of whom underwent operation before the introduction of histamine H2-receptor antagonists, are known dead from complications of continued gastric hypersecretion and tumor growth. Sixteen children had a total gastrectomy, with no operative deaths, and only one died of progressive tumor growth, even though 14 had evidence of metastatic islet-cell carcinoma. Follow-up serum gastrin measurements have been obtained for 13 patients with total gastrectomy, and 5 patients now have a normal serum gastrin levels. Malignant gastrinomas in children have been slow growing, indolent, and compatible with long life. The biologic behavior of malignant gastrinomas appears to be more favorable in the young patient. Total gastrectomy can be done safely in children with the Zollinger-Ellison syndrome and effectively controls gastric hypersecretion when all gastrin-producing tumor cannot be excised. Surgical exploration and an attempt at "curative" tumor excision, even when tumor is extrapancreatic and in lymph nodes, appear worthwhile in selected patients.
对佐林格-埃利森综合征（胃泌素瘤）患儿的长期随访表明，手术治疗仍然具有优势。28例佐林格-埃利森综合征患儿在首次手术后接受了长达21年的随访。7例未接受全胃切除术的患儿中有6例，他们均在组胺H2受体拮抗剂问世之前接受了手术，已知死于持续胃酸分泌过多和肿瘤生长的并发症。16例患儿接受了全胃切除术，无手术死亡病例，尽管14例有转移性胰岛细胞癌的证据，但只有1例死于肿瘤进展。对13例接受全胃切除术的患者进行了随访血清胃泌素测量，5例患者目前血清胃泌素水平正常。儿童恶性胃泌素瘤生长缓慢、病程惰性，与长寿相符。恶性胃泌素瘤的生物学行为在年轻患者中似乎更有利。对于佐林格-埃利森综合征患儿，全胃切除术可以安全进行，当无法切除所有产生胃泌素的肿瘤时，可有效控制胃酸分泌过多。即使肿瘤位于胰腺外和淋巴结，手术探查并尝试进行“根治性”肿瘤切除，在选定的患者中似乎也是值得的。
