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儿童原发性气管神经鞘瘤:病例报告及文献复习

Primary intratracheal neurilemmoma in children: case report and literature review.

作者信息

Tang Lan Fang, Chen Zhi Min, Zou Chao Chun

机构信息

Department of Respiratory Medicine, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.

出版信息

Pediatr Pulmonol. 2005 Dec;40(6):550-3. doi: 10.1002/ppul.20279.

Abstract

A 9-year-old girl was admitted because of recurrent cough, dyspnea, and tachypnea for over 3 months, which had become worse for the preceding week. A chest X-ray revealed a mass in the lumen of the trachea. Fiberoptic bronchoscopy confirmed a polypoid mass in the upper trachea 3 cm under the vocal cords, with a 1.5-cm diameter that occluded approximately 70% of the lumen. Endoscopic excision of the neoplasm was performed with rigid bronchoscopy. Results of immunolabeling, which was positive for S-100 protein and vimentin, demonstrated a benign neurilemmoma. Clinical symptoms and signs improved after the tumor was removed. Intratracheal neurilemmoma is an extremely rare neoplasm, and only 34 cases were previously reported worldwide. The most common presentation is airway obstruction. Preoperative diagnosis is difficult, and radiographic techniques are helpful for diagnosis. Bronchoscopy is recommended for diagnosis, biopsy, and resection of the tumor. Long-term follow-up after simple enucleation is required.

摘要

一名9岁女孩因反复咳嗽、呼吸困难和呼吸急促3个多月入院,前一周病情加重。胸部X线检查显示气管腔内有一肿块。纤维支气管镜检查证实声带下3厘米处的气管上段有一息肉样肿块,直径1.5厘米,约阻塞管腔的70%。用硬支气管镜对肿瘤进行了内镜切除。免疫标记结果显示S-100蛋白和波形蛋白呈阳性,证实为良性神经鞘瘤。肿瘤切除后临床症状和体征有所改善。气管内神经鞘瘤是一种极其罕见的肿瘤,此前全球仅报道过34例。最常见的表现是气道阻塞。术前诊断困难,影像学技术有助于诊断。建议采用支气管镜进行肿瘤的诊断、活检和切除。单纯摘除术后需要长期随访。

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