Liu Yuanshun, Jiang Hua, Xu Qiuran, Zhou Hongbin, Li Yaqing
Department of Respiratory Medicine, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou, Zhejiang 310014, P.R. China.
The Second Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310053, P.R. China.
Oncol Lett. 2018 Jul;16(1):843-848. doi: 10.3892/ol.2018.8737. Epub 2018 May 18.
Liposarcoma is the most common type of soft-tissue sarcoma, and predominantly originates from the extremities and retroperitoneal cavity. However, primary endobronchial liposarcoma is extremely rare. The present study reports on the case of a 54-year-old man, a smoker, diagnosed with primary endobronchial atypical lipomatous tumor/well-differentiated liposarcoma (ALT-WDLS), which was successfully resected via bronchoscopy. Chest computed tomography (CT) revealed a neoplasm in the left main bronchus measuring 12.8×7.8 mm. Bronchoscopy demonstrated multiple roundish, pedunculated, polypoid masses in the left main bronchus. Bioptic specimens were obtained from the roundish masses, and pathological examination revealed an adipose tissue-derived tumor. The masses were completely resected during the second bronchoscopy under general anesthesia. Histopathological examination of the radical resection specimen revealed that it was an ALT-WDLS. The patient showed good quality of life at the 6-month postoperative follow-up without evidence of recurrence. Immunohistochemistry was completed with the human homologue of murine double-minute type 2 (MDM2) (+), cyclin-dependent kinase 4 (CDK4) (+), p16 (+), S-100 (+), Ki-67 (+), cluster of differentiation 34 (CD34) (+) and retinoblastoma protein (+), confirming ALT-WDLS. However, the fluorescence hybridization assay revealed no amplification of MDM2 and CDK4 in the ALT-WDLS. To the best of our knowledge, the present case report is the first to describe the clinicopathologic features and genetic analysis of endobronchial liposarcoma. Although rare, this case is a reminder that clinicians should consider the possibility of this rare endobronchial tumor in patients with nonspecific symptoms including chronic cough, chest pain and recurrent pneumonia. As in this case, endoscopic treatment provided an excellent clinical outcome in patients with primary endobronchial ALT-WDLS.
脂肪肉瘤是最常见的软组织肉瘤类型,主要起源于四肢和腹膜后腔。然而,原发性支气管内脂肪肉瘤极为罕见。本研究报告了一例54岁男性吸烟者,被诊断为原发性支气管内非典型脂肪瘤性肿瘤/高分化脂肪肉瘤(ALT-WDLS),通过支气管镜检查成功切除。胸部计算机断层扫描(CT)显示左主支气管内有一个大小为12.8×7.8 mm的肿瘤。支气管镜检查发现左主支气管内有多个圆形、有蒂、息肉样肿物。从圆形肿物获取活检标本,病理检查显示为脂肪组织来源的肿瘤。在全身麻醉下第二次支气管镜检查时将肿物完全切除。根治性切除标本的组织病理学检查显示为ALT-WDLS。术后6个月随访时患者生活质量良好,无复发迹象。免疫组织化学检测结果显示鼠双微体2型(MDM2)的人类同源物(+)、细胞周期蛋白依赖性激酶4(CDK4)(+)、p16(+)、S-100(+)、Ki-67(+)、分化簇34(CD34)(+)和成视网膜细胞瘤蛋白(+),确诊为ALT-WDLS。然而,荧光杂交检测显示ALT-WDLS中MDM2和CDK4无扩增。据我们所知,本病例报告首次描述了支气管内脂肪肉瘤的临床病理特征和基因分析。尽管罕见,但该病例提醒临床医生,对于出现慢性咳嗽、胸痛和反复肺炎等非特异性症状的患者,应考虑这种罕见的支气管内肿瘤的可能性。如本病例所示,内镜治疗为原发性支气管内ALT-WDLS患者提供了良好的临床疗效。