Nasopharyngeal angiofibroma is an angiofibroma also known as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma of the nasal cavity.[3] It is a histologically benign but locally aggressive vascular tumor of the nasopharynx that arises from the superior margin of the sphenopalatine foramen and grows in the back of the nasal cavity. It most commonly affects adolescent males (because it is a hormone-sensitive tumor).
Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit (frog face deformity), or brain.[4] Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction with profuse epistaxis.
If nasopharyngeal angiofibroma is suspected based on physical examination (a smooth vascular submucosal mass in the posterior nasal cavity of an adolescent male), imaging studies such as CTorMRI should be performed. Biopsy should be avoided as to avoid extensive bleeding since the tumor is composed of blood vessels without a muscular coat.[citation needed]
Antral sign or Holman-Miller sign (forward bowing of posterior wall of maxilla) is pathognomic of angiofibroma.[citation needed]
DSA (digital subtraction angiography) of carotid artery to see the extension of tumors and feeding vessels[citation needed]
There are many different staging- systems published.[6] One of the most used is that of Radkowsky:[7]
Stage
Tumor Extension
IA
Limited to the nose or the nasopharynx
IB
Extension into one or more paranasal sinus
IIA
Minimal extension through sphenopalatine foramen into and including a minimal part of the medial-most part of pterygomaxillary fossa
IIB
Full involvement of the pterygomaxillary fossa, displacing posterior wall of maxillary antrum forward. Lateral or anterior displacement of the branches of maxillary artery. Superior extension may occur, eroding the orbital bones
IIC
Extension through the pterygomaxillary fossa into the cheek and temporal fossa or posterior to the pterygoid plates
IIIA
Erosion of the skull base with minimal intracranial extension
IIIB
Erosion of skull base with extensive intracranial involvement with or without cavernous sinus involvement
Treatment for Nasopharyngeal angiofibroma (JNA) is primarily surgical. The tumor is primarily excised by external or endoscopic approach. Medical treatment and radiation therapy are only of historical interest.[citation needed]
Endoscopic approach is an excellent tool in primary and recurrent JNA, it allows visualisation and precise removal of the lesion. Preoperative embolisation of tumour may be of some use in reducing intraoperative bleeding.[citation needed]
If the tumor is limited to nasopharynx and nose, for endoscopic approach or Wilson's transpalatal approach is used. It can be extended into Sardana's approach if the tumor extends laterally.
For tumors of infratemporal fossa, Maxillary Swing approach is used.
Transmaxillary Le Fort 1 approach is used for tumors extending into maxillary and ethmoid sinuses and pterygopalatine fossa.
If the tumor extend up to the cheek, for Weber–Ferguson approach should be used.
Prognosis for nasopharyngeal angiofibroma is favorable. Because these tumors are benign, metastasis to distal sites does not occur. However, these tumors are highly vascularized and grow rapidly. Removal is important in preventing nasal obstruction and recurrent epistaxis. Mortality is not associated with nasopharyngeal angiofibroma.[8]
^Li W, Ni Y, Lu H, Hu L, Wang D (June 2019). "Current perspectives on the origin theory of juvenile nasopharyngeal angiofibroma". Discovery Medicine. 27 (150): 245–254. PMID31421693.
^Tos M, Larsen PL, Larsen K, Cayé-Thomasen P (2000). "Nasal Polyps". In Stamm AC, Draf W (eds.). Micro-endoscopic Surgery of the Paranasal Sinuses and the Skull Base. Springer Berlin Heidelberg. pp. 103–125. doi:10.1007/978-3-642-57153-4_8. ISBN9783642571534.
