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胸腺癌合并 I 型多发性内分泌肿瘤综合征。

Thymic carcinoid associated with multiple endocrine neoplasia syndrome type I.

机构信息

Servicio de Cirugía Torácica, Hospital Universitario Miguel Servet y Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.

出版信息

Arch Bronconeumol. 2013 Mar;49(3):122-5. doi: 10.1016/j.arbres.2012.06.002. Epub 2012 Jul 23.

Abstract

Thymic carcinoids are a rare entity that may be associated with endocrine diseases like Cushing's syndrome or multiple endocrine neoplasia syndrome type I (MEN1). These tumors represent 4% of anterior mediastinal tumors and are characterized by their very aggressive behavior. We present the case of a patient with a previous MEN 1 diagnosis in whom, during the follow up of his disease, a thoracic image compatible with thymic carcinoid was detected. After an extended thymectomy that included peri-thymic fat resection, the clinical diagnosis was confirmed. A follow up examination 14 months later revealed a suspicious lesion that suggested local recurrence, therefore the patient was reoperated on. The pathology report of this surgery indicated post-radiation fibrosis. Likewise, we present a review of the current diagnostic and therapeutic management of patients with MEN1 syndrome who are diagnosed with thymic carcinoid.

摘要

胸腺癌是一种罕见的实体瘤,可能与库欣综合征或多发性内分泌腺瘤综合征 1 型(MEN1)等内分泌疾病有关。这些肿瘤占前纵隔肿瘤的 4%,其特征为侵袭性很强。我们报告了 1 例先前诊断为 MEN1 的患者,在对其疾病进行随访时,发现了与胸腺癌一致的胸部影像。在进行了包括胸腺周围脂肪切除的广泛胸腺切除术之后,临床诊断得到了确认。14 个月后的随访检查发现了一个可疑病变,提示局部复发,因此对患者进行了再次手术。该手术的病理报告显示为放射性纤维化。此外,我们还回顾了诊断为胸腺癌的 MEN1 综合征患者的当前诊断和治疗管理。

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