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儿童和青少年气管支气管黏液表皮样癌:病例报告及文献复习

Tracheobronchial mucoepidermoid carcinoma in childhood and adolescence: case report and review of the literature.

作者信息

Welsh J H, Maxson T, Jaksic T, Shahab I, Hicks J

机构信息

Department of Pediatric Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston 77030-2399, USA.

出版信息

Int J Pediatr Otorhinolaryngol. 1998 Oct 15;45(3):265-73. doi: 10.1016/s0165-5876(98)00120-7.

Abstract

In children and adolescents, primary neoplasms of the tracheobronchial tree and lungs are rare, with most tumors involving the respiratory system being metastatic, small, blue cell tumors of childhood. Of the primary pulmonary neoplasms, most are malignant with mucoepidermoid carcinoma representing about 10% of these malignant tumors. We present an 8-year-old Hispanic male with hemoptysis and several episodes of pneumonia which initially was thought to be infectious upon biopsy during bronchoscopy, but proved to be mucoepidermoid carcinoma of the tracheobronchial tree by microscopic examination during an open lung biopsy. This rare tumor is more common in adults than in children, and infrequently presents with hemoptysis. Mucoepidermoid tumors of the tracheobronchial tree carry a more favorable prognosis in children than adults. In the adult population, the overall mortality is slightly less than 30%. In contrast, of the 31 reported cases of tracheobronchial mucoepidermoid carcinoma in pediatrics, all children are free of tumor involvement with a mean follow-up period of 5.8 years (range, 0.7-21 years). Based upon the available clinical outcome and survival data, it would appear that tracheobronchial mucoepidermoid carcinoma may be successfully managed by surgical intervention alone in children and adolescents.

摘要

在儿童和青少年中,气管支气管树和肺部的原发性肿瘤很少见,大多数累及呼吸系统的肿瘤是转移性的儿童小蓝细胞肿瘤。在原发性肺肿瘤中,大多数是恶性的,黏液表皮样癌约占这些恶性肿瘤的10%。我们报告一名8岁西班牙裔男性,有咯血和几次肺炎发作,支气管镜检查活检时最初认为是感染性的,但在开胸肺活检时经显微镜检查证实为气管支气管树的黏液表皮样癌。这种罕见肿瘤在成人中比在儿童中更常见,很少表现为咯血。气管支气管树的黏液表皮样肿瘤在儿童中的预后比成人更有利。在成人中,总体死亡率略低于30%。相比之下,在儿科报告的31例气管支气管黏液表皮样癌病例中,所有儿童均无肿瘤累及,平均随访期为5.8年(范围0.7 - 21年)。根据现有的临床结果和生存数据,似乎气管支气管黏液表皮样癌在儿童和青少年中仅通过手术干预就可以成功治疗。

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