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肺炎性肌纤维母细胞瘤的临床特征与预后:一项超过10年的回顾性分析

Clinical characteristics and prognosis of pulmonary inflammatory myofibroblastic tumor: An over 10-year retrospective analysis.

作者信息

Zhang Na, Zeng Qi, Chen Chenghao, Yu Jie, Yan Dong, Xu Changqi, Liu Dingyi, Zhang Qian, Zhang Xu

机构信息

Department of Thoracic Surgery Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China.

出版信息

Pediatr Investig. 2020 Sep 27;4(3):192-197. doi: 10.1002/ped4.12218. eCollection 2020 Sep.

Abstract

IMPORTANCE

Pulmonary inflammatory myofibroblastic tumors (PIMTs) are primary lung tumors in children. Misdiagnosis easily occurs because of the nonspecific clinical manifestations, laboratory examination results, and imaging findings in affected patients.

OBJECTIVE

To summarize the clinical characteristics, diagnosis, and prognosis of children with PIMTs.

METHODS

This retrospective analysis included 23 children with PIMTs who received treatment in our hospital from January 2008 to January 2019. The clinical manifestations, laboratory examination results, and computed tomography findings were retrospectively analyzed.

RESULTS

The population included 13 boys and 10 girls, with a median age at onset of 78 months (range, 10-126 months). Fourteen patients had PIMT lesions in the right lung and nine patients had PIMT lesions in the left lung. The surgical procedures included pulmonary wedge resection, pulmonary lobectomy, and total pneumonectomy. The median operation time was 115 min (range, 45-235 min); the median intraoperative blood loss volume was 30 mL (range, 3-500 mL). During the operation, one patient each had pulmonary hemorrhage, vena cava hemorrhage, and thoracic duct injury. Postoperative complications included pulmonary embolism in one patient and tumor recurrence in two patients; neither of these complications recurred after reoperation. The median follow-up period was 49 months (range, 2-127 months).

INTERPRETATION

Children with PIMTs exhibited good prognoses and primarily underwent surgical resection as treatment. Complete preoperative evaluation should be performed in affected patients.

摘要

重要性

肺炎症性肌纤维母细胞瘤(PIMTs)是儿童原发性肺部肿瘤。由于患病患者临床表现、实验室检查结果及影像学表现缺乏特异性,故易发生误诊。

目的

总结儿童PIMTs的临床特征、诊断及预后情况。

方法

本回顾性分析纳入了2008年1月至2019年1月在我院接受治疗的23例儿童PIMTs患者。对其临床表现、实验室检查结果及计算机断层扫描结果进行回顾性分析。

结果

研究对象包括13例男孩和10例女孩,发病年龄中位数为78个月(范围10 - 126个月)。14例患者右肺有PIMT病变,9例患者左肺有PIMT病变。手术方式包括肺楔形切除术、肺叶切除术及全肺切除术。手术时间中位数为115分钟(范围45 - 235分钟);术中失血量中位数为30毫升(范围3 - 500毫升)。手术过程中,各有1例患者发生肺出血、腔静脉出血及胸导管损伤。术后并发症包括1例肺栓塞和2例肿瘤复发;再次手术后这些并发症均未复发。中位随访期为49个月(范围2 - 127个月)。

解读

儿童PIMTs预后良好,主要治疗方式为手术切除。应对患病患者进行全面的术前评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f6c/7520109/20a30265028b/PED4-4-192-g001.jpg

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