Nasopharyngeal angiofibroma
| Nasopharyngeal angiofibroma |
|---|
Nasopharyngeal angiofibroma (also called juvenile nasopharyngeal angiofibroma)[1][2] is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity. It most commonly affects adolescent males and may grow into fissures of the skull and may spread to adjacent structures.[3] Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction and recurrent bleeding.
Signs and symptoms
- Frequent chronic epistaxis or blood-tinged nasal discharge
- Nasal obstruction and rhinorrhea
- Facial dysmorphism (when locally invasive)
- Conductive hearing loss from eustachian-tube obstruction
- Diplopia, which occurs secondary to erosion into superior orbital fissure and due to third and sixth nerve palsy.
- Proptosis when having intraorbital extension.
- Rarely anosmia, recurrent otitis media, and eye pain.
Diagnosis
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
Differential diagnosis
- Antro-choanal polyp (benign neoplasm)
- Rhinosporidiosis (as bleeding point is here too)
- Malignancy—nasopharyngeal carcinoma, lymphoma, plasmacytoma, rhabdomyosarcoma
- Chordoma
- Nasopharyngeal cyst
- Pyogenic granuloma
Treatment
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:
- transpalatine approach
- transpalatine + sublabial (Sardana's) Approach
- infratemporal Approach
- nasal endoscopic Approach
- transmaxillary Approach
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
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.[4]
References
- ^ 00021 at CHORUS
- ^ "juvenile nasopharyngeal angiofibroma" at Dorland's Medical Dictionary
- ^ Raphael Rubin; David S. Strayer; Emanuel Rubin (2008). Rubin's Pathology: clinicopathologic foundations of medicine. Lippincott Williams & Wilkins. pp. 1071–. ISBN 978-0-7817-9516-6. Retrieved 29 June 2010.
- ^ Textbook of Family Medicine, 8th Edition. Chapter 19 p.329. Rakel, MD. ISBN 978-1-4377-1160-8