Current Management of Juvenile Nasopharyngeal Angiofibroma
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Pediatric Dermatology and Allergy (J Lee, Section Editor)
Current Management of Juvenile Nasopharyngeal Angiofibroma Spencer Uetz, MD Dana L. Crosby, MD, MPH* Address * Department of Otolaryngology Head and Neck Surgery, Southern Illinois University School of Medicine, 720 N Bond St, Springfield, IL, 62702, USA Email: [email protected]
Published online: 28 August 2020 * Springer Nature Switzerland AG 2020
This article is part of the Topical Collection on Pediatric Dermatology and Allergy Keywords Juvenile nasopharyngeal angiofibroma I JNA I Sinonasal tumor I Pterygopalatine I Anterior skull base I Endoscopic endonasal
Abstract Purpose of review The purpose of this article is to provide a current review of the most important management principles for evaluation and treatment of juvenile nasopharyngeal angiofibroma. Recent findings Numerical staging systems have been adapted many times over the years, but a new nominal staging system based on specific anatomical site involvement and residual internal carotid artery vascularization helps to provide a more comprehensive alternative for evaluation. An expanding body of evidence supports the use of an endoscopic surgical approach to reduce risk of tumor recurrence and reduce intraoperative blood loss. With minimally invasive, endoscopic techniques available, the primary treatment modality for JNA remains surgery. However, gamma knife radiosurgery can be useful in a multimodality approach to more advanced cases of JNA and to address recurrence in the immediate postoperative period. Summary This review focuses on the evaluation and surgical management of juvenile nasopharyngeal angiofibroma with emphasis placed on the most current staging systems, the necessity for preoperative embolization, the endoscopic approach to resection, and the adjuvant therapies that are available.
Introduction Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular, benign tumor of the anterior skull
base that occurs almost exclusively in adolescent males. Although histologically benign, these tumors
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Pediatric Dermatology and Allergy (J Lee, Section Editor)
often demonstrate locally aggressive features with invasion into the nasal cavity, nasopharynx,
pterygopalatine fossa, paranasal sinuses, orbit, and cranium.
Epidemiology JNA is the most common benign sinonasal neoplasm, comprising up to 0.5% of all head and neck tumors [1]. There is a reported incidence ranging from 1 in 150,000 to 1 in 15,000,000 [2]. Due to the reporting discrepancy in third-world countries caused by underreporting and underdiagnosis, it is challenging to make geographical comparisons. However, there appears to be a higher incidence in India and other Middle East countries as compared to people of European descent. One hypothesis is that this is related to environmental and social interactions in this region that are known to cause a disruption in hormone regulation [3]. These complex interactions include air and water pollution, obesity, nutritional deficiencies, socioeconomic differences, and psy
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