OA Journal of Otorhinolaryngology

A Unique Case of Juvenile Nasopharyngeal Angiofibroma (JNA) Involving the Skull Base, Intracranial Cavity, and Para-Pharyngeal Space

*Dr.Randa Barazi
Department Of Otolaryngology, American University Of Beirut, Lebanon

*Corresponding Author:
Dr.Randa Barazi
Department Of Otolaryngology, American University Of Beirut, Lebanon
Email:ra110@aub.edu.lb

Published on: 2019-08-13

Abstract

Juvenile nasopharyngeal angiofibroma (JNA) constitutes 0.05 to 0.5% of all head and neck tumors. This tumor has the propensity to spread from the sphenopalatine fossa to nearby structures such as the intracranial cavity, infratemporal fossa, or much more rarely the para-pharyngeal space. In this case report, we present a 15-year-old male with JNA involving the intracranial cavity, parapharyngeal space, and skull base altogether. Unusually, the patient has had a cranioplasty with a floating anterior skull bone after a repair which made this clinical scenario more challenging. As far as our knowledge, it is the first case of JNA with such extreme involvement to be reported in the literature. A craniofacial approach to the tumor was performed with subsequent peri-cranial flap skull base reconstruction. Since JNA is a highly vascularized tumor with risk for life-threatening hemorrhage, it is necessary to perform a preoperative angiography and embolization in order to reduce operative morbidity. JNA is usually treated with complete surgical excision. Advanced stage JNAs have a tendency to recur. Recurrence is also treated with complete resection and previously other adjacent measures whenever possible.

Keywords

Juvenile Nasopharyngeal Angiofibroma, Otolaryngology, Nasopharyngeal neoplasm, biopsy,head and neck tumors

Introduction

Juvenile nasopharyngeal angiofibroma (JNA) is a rare nasopharyngeal neoplasm that is vascular in origin, and constitutes 0.05 to 0.5% of all head and neck tumors [1]. It primarily consists of vasogenic elements, and is almost always found in adolescent males due to its androgen dependence [2]. It is speculated that JNAs originate in the superior margin of the sphenopalatine foramen [3, 4]. Despite their classification as benign neoplasms, JNAs are not usually encapsulated, can be locally invasive and aggressive, and have been found to spread to nearby structure, including the infratemporal fossa and middle cranial fossa [3]. Anterior cranial fossa involvement is rare but has been reported [4]. JNAs can cause bony erosion by expanding and compressing the bones of the face and skull, what is usually described as a pushing growth pattern [4, 5]. A total of 20 to 30% of JNAs are found to grow and invade to adjacent structures and spaces [4]. Intracranial extension of JNA used to be considered inoperable, and was solely treated with radiotherapy in the past [4, 6]. This article reports the case of a 15 year old male presenting with a very large intracranial JNA involving the para-pharyngeal space as well as the skull base/intracranial involvement of the middle and anterior cranial fossae with no epistaxis. The pathogenesis and approaches to the treatment of intracranial JNAs will be reviewed with reference to the literature.