Department of Respiratory and Critical Care Medicine, Xuzhou Central Hospital, Xuzhou, China.
Department of Thoracic Surgery, Xuzhou Central Hospital, 199 Jiefang South Road, Xuzhou, 221009, China.
J Cardiothorac Surg. 2020 Sep 11;15(1):252. doi: 10.1186/s13019-020-01295-9.
Neurogenic tumor arising within the pulmonary sequestration (PS) is rare.
A 42-year-old asymptomatic female was referred to our hospital for work-up of extralobar PS. The independent feeding artery from the thoracic aorta was confirmed by three-dimensional computed tomography angiography (3D-CTA). Uniportal thoracoscopic resection of the sequestrated lung with mediastinal lymph node sampling was performed successfully. Ganglioneurofibroma within the PS was diagnosed as the specimen revealed positive expression of SRY-related HMG-box 10 protein, neuron-specific enolase, S-100, chromogranin A and synuclein. Tumor recurrence was not recorded 1 year after the surgery.
Preoperative 3D-CTA is useful to identify the aberrant vessels of PS. An elaborate diagnostic work-up after a timely resection is necessary for subsequent management and follow-up plan.
肺隔离症（PS）内发生的神经源性肿瘤较为罕见。
一位 42 岁无症状女性因肺外叶隔离症的检查被转至我院。三维计算机断层血管造影（3D-CTA）证实存在发自胸主动脉的独立供养动脉。成功实施了单孔式胸腔镜下隔离肺切除术及纵隔淋巴结采样术。PS 内的神经节神经纤维瘤诊断为标本显示 SRY 相关高迁移率盒蛋白 10 阳性表达、神经元特异性烯醇化酶、S-100、嗜铬粒蛋白 A 和突触核蛋白。术后 1 年无肿瘤复发记录。
术前 3D-CTA 有助于识别 PS 的异常血管。及时切除后进行详细的诊断性检查对于后续管理和随访计划是必要的。
