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14岁女孩,患有支气管内类癌瘤,表现为哮喘和大叶性肺气肿。

Fourteen-year-old girl with endobronchial carcinoid tumour presenting with asthma and lobar emphysema.

作者信息

Andersen Julie Bjerglund, Mortensen Jann, Damgaard Karen, Skov Marianne, Sparup Jørgen, Petersen Bodil Laub, Rechnitzer Catherine, Borgwardt Lise

机构信息

Department of Clinical Physiology, Nuclear Medicine & PET, Rigshospitalet, Copenhagen University Hospital, København Ø, Denmark.

出版信息

Clin Respir J. 2010 Apr;4(2):120-4. doi: 10.1111/j.1752-699X.2009.00149.x.

DOI:10.1111/j.1752-699X.2009.00149.x
PMID:20565486
Abstract

INTRODUCTION

Bronchial carcinoid tumours seldom occur in children, and represent a rare cause of pulmonary obstruction. Because of low clinical suspicion and the variable ways of presentation, diagnosis may be delayed.

OBJECTIVES

We report on a patient with this tumour. It is hoped that increased awareness of the tumour can lead to earlier diagnosis.

METHODS

Report of a case.

RESULTS

This case describes a 14-year-old previously healthy girl, presenting with asthma-like symptoms throughout 2 years, decreased lung function and emphysema in left lower lobe on chest x-ray. Computerized tomography (CT) showed an intraluminal process in the left main bronchus and emphysema in both the upper and lower left lobe and showed no signs of metastasis or spread to lung tissue. Bronchoscopy showed an inflammatory polyp. Surgical resection demonstrated a typical carcinoid tumour. Later control biopsy revealed no persisting malignant tissue. The asthma symptoms returned and a new bronchoscopy showed scarring and narrowing of the left bronchus. Treatment comprised of dilatation by bronchoscopy plus daily combination corticosteroids and beta-2-agonist inhalation and the symptoms improved. No signs of relapse 16 months postdiagnosis.

CONCLUSIONS

The case clearly shows the delay, which is common in the diagnosis of children with bronchial carcinoid tumours. Symptoms of the obstructive nature of the tumour are variable and might present as emphysema seen on x-ray and CT. Carcinoid tumour should be considered in children with longstanding pulmonary symptoms with no response to conventional treatment. Prognosis is good but long-term follow up is needed.

摘要

引言

支气管类癌瘤在儿童中很少见,是导致肺阻塞的罕见原因。由于临床怀疑度低且表现方式多样,诊断可能会延迟。

目的

我们报告一例患有这种肿瘤的患者。希望提高对该肿瘤的认识能导致更早的诊断。

方法

病例报告。

结果

该病例描述了一名14岁、此前健康的女孩,两年来一直出现哮喘样症状,肺功能下降,胸部X光显示左下叶肺气肿。计算机断层扫描(CT)显示左主支气管腔内有病变,左上叶和左下叶均有肺气肿,且未显示转移或扩散至肺组织的迹象。支气管镜检查显示为炎性息肉。手术切除显示为典型的类癌瘤。后来的对照活检未发现残留的恶性组织。哮喘症状复发,并进行了新的支气管镜检查,显示左支气管有瘢痕形成和狭窄。治疗包括支气管镜扩张术,以及每日联合使用皮质类固醇和吸入β-2激动剂,症状有所改善。诊断后16个月无复发迹象。

结论

该病例清楚地表明,支气管类癌瘤患儿的诊断常见延迟情况。肿瘤阻塞性的症状多样,可能表现为X光和CT上所见的肺气肿。对于有长期肺部症状且对常规治疗无反应的儿童,应考虑类癌瘤。预后良好,但需要长期随访。

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引用本文的文献

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Bronchoscopic management of endobronchial carcinoid presenting as asthma Mimic.表现为哮喘样症状的支气管内类癌的支气管镜管理
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Endobronchial tumor in children: Unusual finding in recurrent pneumonia, report of three cases.儿童支气管内肿瘤:复发性肺炎中的罕见发现,三例报告
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Overdiagnosis of a typical carcinoid tumor as an adenocarcinoma of the lung: a case report and review of the literature.
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