Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome
Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome
复制标题
DOI：
10.1200/jco.1999.17.2.615
复制
发表时间：
1999-02-01
影响因子：
45.3
通讯作者：
Jensen, RT
中科院分区：
文献类型：
--
作者：
Yu, F;Venzon, DJ;Jensen, RT
目的：未选择的胃泌素瘤及其他功能性胰腺内分泌肿瘤（pet）患者的长期临床病程在很大程度上是未知的。为了解决这个问题，我们对胃泌素瘤患者进行了评估。患者与方法：对212例Zollinger-Ellison综合征（ZES）患者进行前瞻性研究。所有患者胃酸分泌均得到控制，每年进行一次评估，平均随访时间为13.8 +/- 0.6年（范围0.1至31年）。每年对可能影响预后或治疗方法的因素进行评估，如肿瘤大小和位置；转移的存在、位置和程度；以及异位库欣综合征或其他PET综合征的发生。死亡被分类为与es相关或非es相关，并按不同原因分类。结果：31%的患者死亡，均为非酸相关原因。一半的人死于与热休克有关的原因；他们与那些死于非zes的人不同，因为他们有很大的原发肿瘤，更常见的是胰腺肿瘤；淋巴结、肝脏或骨转移；异位库欣综合征；或者更高的胃泌素水平。肝转移程度与生存率相关。单独存在肝转移只会适度减少生存时间；然而，骨转移或异位库欣综合征的额外发展显着降低了生存率。结论：在ZES中，胃泌素瘤的生长现在是长期生存的主要单一决定因素，一半的患者死于胃泌素瘤相关死亡，而没有患者死于酸相关死亡。大的原发肿瘤在胰腺的位置，肝转移的发展，（特别是如果伴有骨转移或库欣综合征），以及肝转移的程度都是重要的预后因素。这些因素的识别允许识别亚群，可用于定制抗肿瘤治疗方法。(C) 1999年由美国临床肿瘤学会出版。
Purpose: The long-term clinical course of unselected patients with gastrinomas as well as other functional pancreatic endocrine tumors (PETs) in whom the excess-hormone stare is controlled is largely unknown. To address this issue, patients with gastrinomas were assessed.Patients and Methods: Two hundred twelve patients with Zollinger-Ellison syndrome (ZES) were prospectively studied. All had controlled acid hypersecretion and were assessed yearly, with a mean follow-up period of 13.8 +/- 0.6 years (range, 0.1 to 31 years). Annual assessments of possible factors that might affect prognosis or treatment approaches were performed, such as those for tumor size and location; the presence, location, and extent of metastases; and the occurrence of ectopic Cushing's syndrome or another PET syndrome. Deaths were categorized as ZES-related or non-ZES-related and classified into different causes.Results: Thirty-one percent of patients died, all of non-acid-related causes. One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing's syndrome; or higher gastrin levels. The extent of liver metastases correlated with survival rate. The presence of liver metastases alone only moderately decreased survival time; however, the additional development of bone metastases or ectopic Cushing's syndrome markedly decreased survival rate.Conclusions: In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing's syndrome), and the extent of liver metastases are all important prognostic factors. The identification of these factors allows the recognition of subgroups that can be used to tailor antitumor treatment approaches. (C) 1999 by American Society of Clinical Oncology.