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儿童类癌和黏液表皮样支气管肿瘤

Carcinoid and mucoepidermoid bronchial tumours in children.

作者信息

Fauroux Brigitte, Aynie Valérie, Larroquet Michèle, Boccon-Gibod Liliane, Ducou le Pointe Hubert, Tamalet Aline, Clément Annick

机构信息

Paediatric Pulmonary Department, Research Unit INSERM U 719, Armand Trousseau Hospital, Paris, France.

出版信息

Eur J Pediatr. 2005 Dec;164(12):748-52. doi: 10.1007/s00431-005-1740-x. Epub 2005 Aug 25.

Abstract

UNLABELLED

The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour and 6 cases of mucoepidermoid tumour. The mean age of the patients was 10.5+/-3.0 years, with a range of 5 to 15 years. Twelve and 6 patients presented with evidence of bronchial obstruction and haemoptysis, respectively. Fibre optic bronchoscopy confirmed the presence of a bronchial tumour in all cases and endobronchial biopsies were diagnostic in 11 of 12 cases. A chest CT scan revealed the presence of a hypervascular tumour in 8 of 12 patients. The distribution of the location of the tumours was equal between the right and the left lung, and, in 9 cases, the airways were totally occluded by the tumour. Complete surgical resection (lobectomy in 15 patients and pneumonectomy in 2 patients) was performed in all cases without pre-operative chemotherapy or radiotherapy. The mean duration of follow-up was 4.0+/-3.0 years. In 2 patients, auscultation assymetry and an episode of haemoptysis revealed the recurrence of a mucoepidermoid tumour, successfully cured by removal of the tumour and chemotherapy and radiotherapy in one child. No death was observed.

CONCLUSION

Pulmonary carcinoid and mucoepidermoid tumours are rare in children. Bronchoscopic removal should not be performed. With aggressive surgical therapy, the prognosis is excellent. Fibre optic bronchoscopy confirms the presence of an endobronchial mass. A biopsy is needed for diagnosis and complete surgical removal is the treatment of choice. Long-term results are excellent but a clinical follow-up is recommended.

摘要

未标注

本研究的目的是确定儿童类癌或黏液表皮样癌的特征及预后。对1984年至2001年间在法国接受类癌或黏液表皮样癌治疗的所有患者进行了回顾性分析。有11例类癌和6例黏液表皮样癌。患者的平均年龄为10.5±3.0岁,范围为5至15岁。分别有12例和6例患者出现支气管阻塞和咯血的证据。纤维支气管镜检查在所有病例中均证实存在支气管肿瘤,12例中有11例经支气管内活检确诊。胸部CT扫描显示12例患者中有8例存在高血管肿瘤。肿瘤在左右肺的分布位置相等,9例患者气道被肿瘤完全阻塞。所有病例均进行了完整的手术切除(15例患者行肺叶切除术,2例患者行全肺切除术),未进行术前化疗或放疗。平均随访时间为4.0±3.0年。2例患者听诊不对称和咯血一次,提示黏液表皮样癌复发,其中一名儿童通过切除肿瘤及化疗和放疗成功治愈。未观察到死亡病例。

结论

儿童肺类癌和黏液表皮样癌罕见。不应进行支气管镜切除。积极的手术治疗预后良好。纤维支气管镜可证实支气管内肿物的存在。诊断需要活检,完整的手术切除是首选治疗方法。长期结果良好,但建议进行临床随访。

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