Camilon P Ryan, Rahbar Reza, Cunningham Michael J, Adil Eelam A
Department of Otolaryngology, Boston Medical Center, Boston, Massachusetts.
Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts.
Laryngoscope. 2019 Aug;129(8):1777-1783. doi: 10.1002/lary.27633. Epub 2018 Dec 19.
OBJECTIVES/HYPOTHESIS: To highlight the presentation and management of juvenile nasopharyngeal angiofibroma (JNA) in prepubertal children.
Single-institution 10-year retrospective review.
All identified cases of pathologically confirmed JNA in children <10 years of age were assessed from a gender, imaging and embolization findings, tumor stage, surgical approach, and clinical outcomes standpoint, and compared to a group of stage-matched older patients from the same time period.
Of 45 patients over the 10-year study period, four male children between 8 to 9.8 years of age were identified. One patient had University of Pittsburgh Medical Center stage 1 disease, and the other three had stage 3 disease at presentation. A malignant process other than JNA was of concern preoperatively in two of the four children due to a combination of aggressive imaging characteristics and an absence of pterygopalatine fossa involvement. Such pterygopalatine fossa involvement was comparatively uniformly present in a group of stage-matched JNA patients aged 15 to 21 years. All four prepubescent children underwent surgical resection via transnasal endoscopic approach following ipsilateral sphenopalatine artery embolization without the need for blood transfusion. There were no recurrences in three of the four cases at a median follow-up duration of 2.3 years (range, 0.8-6.4 years).
JNA may pose a diagnostic challenge in prepubertal males due to the atypical age at presentation and absence of classic imaging characteristics. Successful endoscopic transnasal resection is possible despite anatomic constrictions.
4 Laryngoscope, 129:1777-1783, 2019.
目的/假设：强调青春期前儿童青少年鼻咽血管纤维瘤（JNA）的临床表现及治疗。
单机构10年回顾性研究。
从性别、影像学及栓塞结果、肿瘤分期、手术方式及临床结局等方面，对所有病理确诊的10岁以下儿童JNA病例进行评估，并与同期年龄匹配的年长患者组进行比较。
在10年研究期间的45例患者中，确定了4例8至9.8岁的男性儿童。1例患者为匹兹堡大学医学中心1期疾病，另外3例初诊时为3期疾病。由于影像学表现侵袭性及翼腭窝未受累，4名儿童中有2名术前怀疑除JNA外存在恶性病变。在一组年龄匹配的15至21岁JNA患者中，翼腭窝受累情况相对较为一致。所有4例青春期前儿童在同侧蝶腭动脉栓塞后均通过鼻内镜手术切除，无需输血。4例患者中有3例在中位随访2.3年（范围0.8 - 6.4年）时无复发。
由于发病年龄不典型及缺乏典型影像学特征，JNA在青春期前男性中可能构成诊断挑战。尽管存在解剖限制，鼻内镜经鼻切除仍可成功实施。
4 喉镜，129:1777 - 1783，2019年。
