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小儿鼻腔癌

Cancer of the nasal cavity in the pediatric population.

作者信息

Benoit Margo McKenna, Bhattacharyya Neil, Faquin William, Cunningham Michael

机构信息

Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114, USA.

出版信息

Pediatrics. 2008 Jan;121(1):e141-5. doi: 10.1542/peds.2007-1319. Epub 2007 Dec 17.

Abstract

OBJECTIVE

The purpose of this work was to investigate the clinical manifestations and diagnostic range of malignant entities presenting as a nasal mass in the pediatric population.

PATIENTS AND METHODS

A retrospective cohort analysis was conducted at a specialty hospital and a tertiary care university hospital. Patients aged between birth and 18 years and diagnosed with a malignancy that arose within the nasal cavity between the years 1991 and 2006 were included. This institution-specific patient group was compared with a similar cohort of patients extracted from the national Surveillance Epidemiology and End Results database. The main outcome measures were the incidence, presentation, and diagnoses of nasal cancer presenting in this population.

RESULTS

Sixteen patients with nasal malignancies presented institutionally in the defined pediatric age group. Patient age at the time of diagnosis ranged from 7 months to 17 years, with a slight male predominance. The main presenting symptoms were unilateral nasal congestion and ophthalmologic complaints. The median time from presentation to diagnosis was 7 weeks; patients who presented with nonspecific complaints, such as nasal obstruction, headache, and fatigue, were given a diagnosis, on average, later than those who presented with focal manifestations. Nationwide, 47 patients were identified from the Surveillance Epidemiology and End Results database. In both subject groups, the most common diagnoses were rhabdomyosarcoma (37.5% institutionally and 23% in the Surveillance Epidemiology and End Results group) and esthesioneuroblastoma (25% institutionally and 28% Surveillance Epidemiology and End Results). In the Surveillance Epidemiology and End Results cohort, the overall mean survival rate was 188 months.

CONCLUSIONS

Nasal cancer in the pediatric population often presents with nonspecific signs and symptoms, and a high index of suspicion is necessary for a timely diagnosis. Soft tissue sarcomas are expectedly common. The relative high frequency of esthesioneuroblastoma is particularly noteworthy.

摘要

目的

本研究旨在调查小儿群体中表现为鼻腔肿物的恶性病变的临床表现及诊断范围。

患者与方法

在一家专科医院和一所三级医疗大学医院进行了一项回顾性队列分析。纳入了出生至18岁之间、于1991年至2006年间被诊断出鼻腔内发生恶性肿瘤的患者。将该特定机构的患者群体与从国家监测、流行病学及最终结果数据库中提取的类似患者队列进行比较。主要观察指标为该人群中鼻腔癌的发病率、表现及诊断情况。

结果

在规定的小儿年龄组中,该机构有16例鼻腔恶性肿瘤患者。诊断时患者年龄从7个月至17岁不等,男性略占优势。主要表现症状为单侧鼻塞和眼部不适。从出现症状到诊断的中位时间为7周;出现鼻塞、头痛和疲劳等非特异性症状的患者,平均诊断时间晚于出现局灶性表现的患者。在全国范围内,从监测、流行病学及最终结果数据库中识别出47例患者。在两个研究组中,最常见的诊断均为横纹肌肉瘤(该机构组为37.5%,监测、流行病学及最终结果组为23%)和嗅神经母细胞瘤(该机构组为25%,监测、流行病学及最终结果组为28%)。在监测、流行病学及最终结果队列中,总体平均生存率为188个月。

结论

小儿群体中的鼻腔癌常表现为非特异性体征和症状,及时诊断需要高度的怀疑指数。软组织肉瘤较为常见在意料之中。嗅神经母细胞瘤相对较高的发病率尤其值得注意。

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