| Nasopharyngeal angiofibroma | |
| Field: | ENT surgery |
| Synonyms: | Juvenile nasopharyngeal angiofibroma |
Nasopharyngeal angiofibroma is an angiofibroma also known as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma of the nasal cavity.[1] 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.[2] 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 CT or MRI should be performed. Biopsy should be avoided as to avoid extensive bleeding since the tumor is composed of blood vessels without a muscular coat.
Antral sign or Holman-Miller sign (forward bowing of posterior wall of maxilla) is pathognomic of angiofibroma.
DSA (digital subtraction angiography) of carotid artery to see the extension of tumors and feeding vessels
There are many different staging- systems published.[4] One of the most used is that of Radkowsky:[5]
| 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.
External approaches:
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.
Direct visualization is not common-
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.[6]