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Abstract：
BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns.
OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented.
METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages.
RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively).
CONCLUSIONS: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.
OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented.
METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages.
RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively).
CONCLUSIONS: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.
摘要：
背景：青少年鼻咽血管纤维瘤（JNA）是鼻咽的纤维血管肿瘤，典型地出现在青少年男性中。报告的平均发病年龄在13至22岁之间[1-6]。假设显着的雄激素刺激可以解释JNA在年轻的青少年男性中存在的强烈倾向。然而,诊断时存在相当大的年龄差异，很少有非常年轻的患者参与与典型的男性青春期生长模式不一致。
目的：本系统综述的目的是确定早发性JNA（EOJNA）的病例，（定义为年龄<10岁），并检查该患者组的疾病特征和治疗方法。还描述并介绍了在我们机构中患有EOJNA的7岁男孩的病例。
方法：我们搜索了Embase，Cochrane数据库和MEDLINE从1996年到2021年2月，用于报道EOJNA病例的研究。相关临床人口统计数据，记录疾病严重程度和治疗结果,并使用描述性统计进行分析.我们将我们的发现与所有年龄段的JNA报告的平均值进行了比较。
结果：我们确定了29项包含34例EOJNA的研究。绝大多数（31/34）患者为男性，诊断平均年龄为8.15岁。最常见的症状是鼻塞（65.2％）和鼻出血（60.9％）。患者最常见的是RadkowskiII期（39.4%）和III期（39.4%）。主要治疗方式包括开放手术（66.7％），内镜手术(24.2%),和放疗（9.1%）。30％的复发明显。在EOJNA组中，Radkowski分期和治疗类型没有显着差异（分别为p=0.440和p=0.659）。
结论：本系统综述提示EOJNA的罕见病例具有明显的疾病特征。与报告的平均值相比，该队列中的患者似乎患有更晚期的疾病和更高的复发率。我们希望这篇综述能提高临床对这种潜在更具侵袭性的JNA亚型的认识。随着EOJNA病例的报道越来越多，对该队列进行更有效的统计分析是可行的.
目的：本系统综述的目的是确定早发性JNA（EOJNA）的病例，（定义为年龄<10岁），并检查该患者组的疾病特征和治疗方法。还描述并介绍了在我们机构中患有EOJNA的7岁男孩的病例。
方法：我们搜索了Embase，Cochrane数据库和MEDLINE从1996年到2021年2月，用于报道EOJNA病例的研究。相关临床人口统计数据，记录疾病严重程度和治疗结果,并使用描述性统计进行分析.我们将我们的发现与所有年龄段的JNA报告的平均值进行了比较。
结果：我们确定了29项包含34例EOJNA的研究。绝大多数（31/34）患者为男性，诊断平均年龄为8.15岁。最常见的症状是鼻塞（65.2％）和鼻出血（60.9％）。患者最常见的是RadkowskiII期（39.4%）和III期（39.4%）。主要治疗方式包括开放手术（66.7％），内镜手术(24.2%),和放疗（9.1%）。30％的复发明显。在EOJNA组中，Radkowski分期和治疗类型没有显着差异（分别为p=0.440和p=0.659）。
结论：本系统综述提示EOJNA的罕见病例具有明显的疾病特征。与报告的平均值相比，该队列中的患者似乎患有更晚期的疾病和更高的复发率。我们希望这篇综述能提高临床对这种潜在更具侵袭性的JNA亚型的认识。随着EOJNA病例的报道越来越多，对该队列进行更有效的统计分析是可行的.
