Ruszniewski P, Podevin P, Cadiot G, Marmuse J P, Mignon M, Vissuzaine C, Bonfils S, Lehy T
Department of Gastroenterology, CHU Bichat-Claude Bernard, Paris, France.
Pancreas. 1993 May;8(3):295-304. doi: 10.1097/00006676-199305000-00003.
The clinical evolution of type I multiple endocrine neoplasia (MEN I) was studied in 45 patients among a consecutive series of 172 with Zollinger-Ellison syndrome (ZES). These 172 patients were seen in our hospital between 1959 and 1989. Diarrhea was half as frequent in ZES-MEN I as in sporadic ZES cases. At diagnosis, mean basal acid output and serum gastrin levels in MEN I patients (28.8 +/- 6.6 mmol/h and 587 +/- 487 pg/ml, respectively) were not different from those observed in the others with sporadic ZES. Laparotomy was performed in all 36 patients with no diffuse liver involvement to attempt the removal of gastrinomas. Twenty-nine patients had adenomas, located in the pancreas in 21, in the duodenal wall in 3, and in both in 5. Adenomas were multiple in 23 cases (78%). No tumor was found in seven patients. Twenty-nine of the 36 operated patients were tumor-free after surgery; 7 died in the postoperative period between 1959 and 1970. Median follow-up of the 38 other patients was 95 months (range 17-278 months). Among the 24 patients without residual tumor at discharge (group I), biological and/or morphological evidence of a persistent or recurrent source of gastrin was obtained in 22. Among the 14 patients with residual tumor (group II), an increase in tumor size was seen in 5 after a median of 27 months (range 9-36 months), while no change occurred in 9 after 54 months (3-100 months). Actuarial survival curves were not different, either in group I versus group II patients (67 and 72% at 5-year follow-up, respectively) or in MEN I versus sporadic ZES patients. Apparently, complete resection of primary tumor did not reduce the incidence of subsequent liver metastases. In all, 21 of the 45 patients had malignant gastrinomas (47%), consisting of liver metastases in 14 (31%), metastatic lymph nodes in 11 (24%), and lung metastases in 2 (4%). Monitoring of fundic argyrophil cells disclosed hyperplasia in 13 of the 14 MEN I patients (92%), and 5 had invasive carcinoid tumors. Taken together, these results prompt us to recommend that in ZES-MEN I patients, surgery should be avoided and oxyntic mucosa regularly monitored.
在172例连续的卓-艾综合征（ZES）患者中，对45例I型多发性内分泌腺瘤病（MEN I）患者的临床病程进行了研究。这172例患者于1959年至1989年在我院就诊。ZES-MEN I患者腹泻的发生率是散发性ZES病例的一半。在诊断时，MEN I患者的平均基础酸排量和血清胃泌素水平（分别为28.8±6.6 mmol/h和587±487 pg/ml）与其他散发性ZES患者观察到的水平无差异。对所有36例无弥漫性肝脏受累的患者进行了剖腹手术，试图切除胃泌素瘤。29例患者有腺瘤，21例位于胰腺，3例位于十二指肠壁，5例两者均有。23例（78%）腺瘤为多发性。7例患者未发现肿瘤。36例接受手术的患者中有29例术后无肿瘤；7例在1959年至1970年的术后期间死亡。其他38例患者的中位随访时间为95个月（范围17 - 278个月）。在出院时无残留肿瘤的24例患者（I组）中，22例获得了胃泌素持续或复发来源的生物学和/或形态学证据。在14例有残留肿瘤的患者（II组）中，5例在中位27个月（范围9 - 36个月）后肿瘤大小增加，而9例在54个月（3 - 100个月）后无变化。精算生存曲线在I组与II组患者之间（5年随访时分别为67%和72%）或MEN I与散发性ZES患者之间无差异。显然，原发性肿瘤的完全切除并未降低随后肝转移的发生率。45例患者中共有21例有恶性胃泌素瘤（47%），其中14例（31%）有肝转移，11例（24%）有转移性淋巴结，2例（4%）有肺转移。对胃底嗜银细胞的监测发现，14例MEN I患者中有13例（92%）有增生，5例有侵袭性类癌肿瘤。综上所述，这些结果促使我们建议，对于ZES-MEN I患者，应避免手术并定期监测泌酸黏膜。
