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多发性内分泌腺瘤病中卓-艾综合征的长期预后

Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia.

作者信息

Melvin W S, Johnson J A, Sparks J, Innes J T, Ellison E C

机构信息

Ohio Digestive Disease Institute, Ohio State University, Columbus 43210-1228.

出版信息

Surgery. 1993 Dec;114(6):1183-8.

PMID:7903006
Abstract

BACKGROUND

The long-term prognosis of Zollinger-Ellison Syndrome (ZES) is not well defined. The findings of other endocrinopathies, the need for long-term surveillance, and the role of surgical treatment are controversial.

METHODS

To help provide more information about these topics the records of 76 patients with ZES were reviewed.

RESULTS

Nineteen patients with gastrinoma had multiple endocrine neoplasia (MEN). Fifteen had hyperparathyroidism from 14 years before to 38 years after the diagnosis of ZES. Three patients had pituitary adenomas. The unusual findings of pheochromocytoma were also seen in three patients. Sixteen patients were followed at least 10 years and 12 were followed for more than 20 years. Surgical cure was achieved in only one patient after a 12-year follow-up. The actual 5-, 10-, 15-, and 20-year survival rates of 94%, 75%, 61%, and 58%, respectively, were compared with patients with sporadic ZES with 5-, 10-, 15-, and 20-year survival rates of 62%, 50%, 37%, and 31%, respectively.

CONCLUSIONS

Multiple endocrinopathies are common but are rarely diagnosed synchronously, mandating life-long surveillance for patients with ZES. Long-term prognosis is good. Survival is longer for patients with ZES and MEN compared with patients with sporadic ZES. Surgical cure is rare. Surgical excision without a single localized lesion does not seem justified.

摘要

背景

卓-艾综合征(ZES)的长期预后尚不明确。其他内分泌疾病的发现、长期监测的必要性以及手术治疗的作用存在争议。

方法

为了帮助提供有关这些主题的更多信息,回顾了76例ZES患者的记录。

结果

19例胃泌素瘤患者患有多发性内分泌肿瘤(MEN)。15例在ZES诊断前14年至诊断后38年出现甲状旁腺功能亢进。3例患者患有垂体腺瘤。3例患者还出现了罕见的嗜铬细胞瘤。16例患者随访至少10年,12例患者随访超过20年。1例患者在12年随访后实现手术治愈。将实际5年、10年、15年和20年生存率分别为94%、75%、61%和58%,与散发性ZES患者的5年、10年、15年和20年生存率分别为62%、50%、37%和31%进行比较。

结论

多发性内分泌疾病很常见,但很少同时被诊断出来,因此需要对ZES患者进行终身监测。长期预后良好。与散发性ZES患者相比,ZES和MEN患者的生存期更长。手术治愈很少见。没有单个局限性病变的手术切除似乎不合理。

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Medicine (Baltimore). 2013 May;92(3):135-181. doi: 10.1097/MD.0b013e3182954af1.
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Primary hepatic gastrinoma: an unusual case of zollinger-ellison syndrome.原发性肝胃泌素瘤:卓-艾综合征的一例罕见病例。
Gastroenterol Hepatol (N Y). 2010 Jan;6(1):53-6.
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Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies.
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Cancer. 2008 Oct 1;113(7 Suppl):1807-43. doi: 10.1002/cncr.23648.
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Medicine (Baltimore). 2006 Nov;85(6):295-330. doi: 10.1097/01.md.0000236956.74128.76.
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