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胸腺瘤。9 例报告,重点探讨其与重症肌无力的关系。

Thymolipoma. A report of nine cases, with emphasis on its association with myasthenia gravis.

机构信息

Department of Pathology, University Hospital, Heidelberg, Germany.

出版信息

Surg Today. 2010;40(2):132-6. doi: 10.1007/s00595-009-4042-5. Epub 2010 Jan 28.

Abstract

PURPOSE

Thymolipomas are rare tumors of the anterior mediastinum accounting for up to 9% of all thymic tumors. These tumors are associated with autoimmune diseases in up to 50% of the patients, including myasthenia gravis, aplastic anemia, hypogammaglobulinemia, lichen planus, and Graves' disease. These tumors with a fatty appearance also can arise in older patients with autoimmune disease.

METHODS

This retrospective study evaluated the thymolipomas from nine patients at a single institution, which were resected between 2002 and 2007. The clinical data as well as radiologic findings were evaluated, together with the follow-up.

RESULTS

Seven patients initially presented with myasthenia gravis, and therefore they underwent a resection of the thymus, even though imaging techniques did not reveal a tumor in any of the cases. Another patient showed no symptoms of autoimmune disease for 20 years, and though cardiomegaly was suspected, further investigation revealed a thymolipoma. The symptoms of myasthenia gravis improved following the surgery in one patient. During follow-up, one patient died due to esophageal cancer, and the remaining patients are alive without recurrence.

CONCLUSIONS

Thymolipomas are benign tumors that show an excellent outcome. Patients with autoimmune disease symptoms occasionally show an improvement of the symptoms after a resection of the tumors.

摘要

目的

胸腺瘤是前纵隔罕见的肿瘤,占所有胸腺肿瘤的 9%。这些肿瘤与多达 50%的患者的自身免疫性疾病有关,包括重症肌无力、再生障碍性贫血、低丙种球蛋白血症、扁平苔藓和格雷夫斯病。这些具有脂肪外观的肿瘤也可能发生在患有自身免疫性疾病的老年患者中。

方法

本回顾性研究评估了 2002 年至 2007 年间在一家机构接受手术切除的 9 例胸腺瘤患者的资料。评估了临床资料和影像学表现以及随访情况。

结果

7 例患者最初表现为重症肌无力,因此即使影像学检查在任何病例中均未发现肿瘤,他们仍接受了胸腺切除术。另一位患者 20 年来无自身免疫性疾病症状,但怀疑存在心肌肥大,进一步检查显示为胸腺瘤。一例重症肌无力患者手术后症状改善。在随访期间,一名患者因食管癌死亡,其余患者无复发且存活。

结论

胸腺瘤是良性肿瘤,预后良好。有自身免疫性疾病症状的患者偶尔会在肿瘤切除后症状改善。

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