Miller Barbra S, Rusinko Rachel Y, Fowler Larry
Division of Endocrine Surgery, Department of General Surgery, University of Michigan, Ann Arbor, MI 48109-0331, USA.
Endocr Pract. 2008 Sep;14(6):713-6. doi: 10.4158/EP.14.6.713.
To report a rare case of multiple endocrine neoplasia type 1 (MEN 1) in conjunction with concomitant thymoma and thymic carcinoid.
We describe a never before reported case involving a 63-year-old female patient with MEN 1 who had synchronous thymoma and thymic carcinoid tumors. A review of the pertinent literature is also undertaken.
Although prognosis is stage dependent for patients with thymoma, patients with thymic carcinoids and MEN 1 have been reported to have an extremely poor prognosis, with many patients dying of complications from thymic carcinoid rather than dying of other manifestations of MEN 1. Our patient underwent successful surgical treatment and remains under surveillance for all aspects of the MEN 1 syndrome.
Thymic tumors are rare, and thymic carcinoids, while very rare in occurrence overall, have a definite association with MEN 1. Thus, it is important for practitioners to screen for thymic tumors routinely in patients with MEN 1 and to treat such tumors aggressively when found because they can be a major cause of mortality. Many thymic carcinoids are far advanced before diagnosis, and optimal screening for and treatment of thymic carcinoid are still being developed.
报告一例罕见的1型多发性内分泌肿瘤（MEN 1）合并胸腺瘤和胸腺类癌的病例。
我们描述了一例此前未报道过的病例，该63岁女性患者患有MEN 1，同时患有同步性胸腺瘤和胸腺类癌肿瘤。我们还对相关文献进行了综述。
虽然胸腺瘤患者的预后取决于分期，但据报道，胸腺类癌和MEN 1患者的预后极差，许多患者死于胸腺类癌的并发症，而非死于MEN 1的其他表现。我们的患者接受了成功的手术治疗，目前仍在对MEN 1综合征的各个方面进行监测。
胸腺肿瘤罕见，胸腺类癌虽然总体发生率极低，但与MEN 1有明确关联。因此，对于MEN 1患者，从业者常规筛查胸腺肿瘤并在发现此类肿瘤时积极治疗非常重要，因为它们可能是主要的死亡原因。许多胸腺类癌在诊断前已处于晚期，胸腺类癌的最佳筛查和治疗方法仍在不断发展中。
