Juvenile nasopharyngeal angiofibroma in prepubertal males: A diagnostic dilemma

Abstract

Objectives/Hypothesis To highlight the presentation and management of juvenile nasopharyngeal angiofibroma (JNA) in prepubertal children. Study Design Single‐institution 10‐year retrospective review. Methods 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. Results 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). Conclusions 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. Level of Evidence 4 Laryngoscope, 129:1777–1783, 2019

Juvenile Nasopharyngeal Angiofibroma: Outcomes Analysis Based Upon Hospital Volume

“…The mean patient age of 13.9 (± 3.0) years reported here corresponds with previously reported age ranges from smaller studies 8–10 . Notably there were 40 patients under 10 years of age identified in this national JNA cohort, corroborating a prior study identifying JNA in prepubertal males 9 .…”

“…The national demographic data provided here is in accordance with such previously published studies. It remains uncertain, however, if a Caucasian predilection is true internationally as there is no global registry of JNA cases, and a relatively high incidence of JNA has been reported in India 7,9 . The international demographics of JNA remain uncertain.…”

Combined endoscopic and transoral resection of a high‐staged juvenile nasopharyngeal angiofibroma: A pictorial essay

“…Given its location and propensity for local invasion and destruction, complete surgical resection is indicated, typically preceded by preoperative embolization due to the highly vascular nature of the tumor 4 . Tumor staging, location, and size factor are into the operative planning and surgical approach, where endoscopic, open approaches, or a combination of both may be implemented in certain cases 3,5‐9 …”

“…4 Tumor staging, location, and size factor are into the operative planning and surgical approach, where endoscopic, open approaches, or a combination of both may be implemented in certain cases. 3,[5][6][7][8][9] We operated on a 14-year-old male with an extensive high-stage dumbbell-shaped JNA involving the infratemporal fossa (ITF), orbit, buccal space, and intracranial extension into Meckel's cave, staged as Andrews-Fisch IIIb, Radkowski IIIa, or UPMC V. Large tumors with this pattern of extensive spread would usually involve extensive open approaches such as a facial translocation, maxillary swing approach, or an orbitozygomatic craniotomy. 10 However, we describe a single-stage, combined endoscopic and transoral approach with minimal morbidity and relative technical ease and good postoperative outcome.…”

Pterygovaginal artery as a target of embolization before endoscopic skull base surgery

“…22 Many reports on juvenile angiofibroma embolization do not mention the PtVA probably because clinicians are often unaware of its presence or indifferent to differentiate it from the SPA or VidA. [23][24][25][26] In fact, the PtVA often cannot be distinguished from these branches by conventional DSA without 3DRA or CBCT images. However, recognizing the PtVA is important because it has a unique course, territory and anastomoses.…”