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胸腺类癌与多发性内分泌腺瘤综合征相关。

Thymic carcinoid in association with MEN syndromes.

作者信息

Zeiger M A, Swartz S E, MacGillivray D C, Linnoila I, Shakir M

机构信息

Surgery Branch, National Cancer Institute, Navy, National Institutes of Health, Bethesda, Maryland 20892.

出版信息

Am Surg. 1992 Jul;58(7):430-4.

PMID:1352092
Abstract

Although carcinoid tumors in association with multiple endocrine neoplasia syndrome (MEN) has been well described, thymic carcinoid in association with MEN is extremely rare (only 23 cases in the world literature). A patient with thymic carcinoid and MEN-I was treated with surgical resection and postoperative radiation therapy, which was later followed by subtotal parathyroidectomy for hyperparathyroidism. Four years later, a symptomatic recurrence of his thymic carcinoid was resected from below his right clavicle. Six years after his original operation, the patient came to the hospital with pancreatitis, and a 5 cm, distal, pancreatic metastasis was resected. He now has symptomatic paraspinal and pleural metastases and is receiving somatostatin. Review of the world's literature shows that the majority of patients with thymic carcinoid and MEN-I are men with an average age of 37 years. Their clinical course is indolent, and surgery represents the only means of cure. Adjuvant chemotherapy and radiation therapy confer no survival advantage. The surgical decision making involved in treating a patient with thymic carcinoid and hyperparathyroidism associated with MEN is also discussed.

摘要

虽然类癌瘤与多发性内分泌肿瘤综合征(MEN)相关的情况已有详尽描述,但胸腺类癌与MEN相关极为罕见(世界文献中仅有23例)。一名患有胸腺类癌和MEN-I的患者接受了手术切除及术后放射治疗,随后因甲状旁腺功能亢进接受了甲状旁腺次全切除术。四年后,从其右锁骨下方切除了有症状复发的胸腺类癌。初次手术后六年,该患者因胰腺炎入院,切除了一个5厘米的胰腺远端转移灶。他现在有症状性的椎旁和胸膜转移,正在接受生长抑素治疗。回顾世界文献表明,大多数患有胸腺类癌和MEN-I的患者为男性,平均年龄37岁。他们的临床病程进展缓慢,手术是唯一的治愈方法。辅助化疗和放射治疗并不能带来生存优势。文中还讨论了治疗患有胸腺类癌和与MEN相关的甲状旁腺功能亢进患者时的手术决策。

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