Nasopharyngeal angiofibroma is an

angiofibroma Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane (i.e. the outer membrane lining body cavities such as the mouth and nose) lesions in which individuals have: 1) benign papules, i.e. pinhead-sized elevation ...

also known as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma of the nasal cavity. It is a histologically

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...

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. Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction with profuse epistaxis.

Signs and symptoms

* Frequent chronic

epistaxis A nosebleed, also known as epistaxis, is bleeding from the nose. Blood can flow down into the stomach, and cause nausea and vomiting. In more severe cases, blood may come out of both nostrils. Rarely, bleeding may be so significant that low bloo ...

or blood-tinged nasal discharge * Nasal obstruction and

rhinorrhea Rhinorrhea, rhinorrhoea, or informally runny nose is the free discharge of a thin mucus fluid from the nose; it is a common condition. It is a common symptom of allergies ( hay fever) or certain viral infections, such as the common cold or COVID- ...

* Facial dysmorphism (when locally invasive) * Conductive hearing loss from eustachian-tube obstruction *

Diplopia Diplopia is the simultaneous perception of two images of a single object that may be displaced horizontally or vertically in relation to each other. Also called double vision, it is a loss of visual focus under regular conditions, and is often v ...

, which occurs secondary to erosion into superior orbital fissure and due to third and sixth nerve palsy. * Proptosis when having intraorbital extension. * Rarely

anosmia Anosmia, also known as smell blindness, is the loss of the ability to detect one or more smells. Anosmia may be temporary or permanent. It differs from hyposmia, which is a decreased sensitivity to some or all smells. Anosmia can be due to a nu ...

, recurrent

otitis media Otitis media is a group of inflammatory diseases of the middle ear. One of the two main types is acute otitis media (AOM), an infection of rapid onset that usually presents with ear pain. In young children this may result in pulling at the ear, ...

, 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 Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

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 non neoplastic growth) * Rhinosporidiosis (as bleeding point is here too) *

Malignancy Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

—

nasopharyngeal carcinoma Nasopharyngeal carcinoma (NPC), or nasopharynx cancer, is the most common cancer originating in the nasopharynx, most commonly in the postero-lateral nasopharynx or pharyngeal recess ( fossa of Rosenmüller), accounting for 50% of cases. NPC occurs ...

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...

plasmacytoma Plasmacytoma is a plasma cell dyscrasia in which a plasma cell tumour grows within soft tissue or within the axial skeleton. The International Myeloma Working Group lists three types: solitary plasmacytoma of bone (SPB); extramedullary plasmac ...

rhabdomyosarcoma Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. There are four subt ...

Chordoma Chordoma is a rare slow-growing neoplasm thought to arise from cellular remnants of the notochord. The evidence for this is the location of the tumors (along the neuraxis), the similar immunohistochemical staining patterns, and the demonstration ...

Nasopharyngeal cyst Nasopharyngeal cyst refers to cystic swelling arising from midline and lateral wall of the nasopharynx. The commonest cyst arising from lateral wall is the nasopharyngeal branchial cyst, whereas the mucus retention cysts are the commonest to aris ...

Pyogenic granuloma A pyogenic granuloma or lobular capillary hemangioma is a vascular tumor that occurs on both mucosa and skin, and appears as an overgrowth of tissue due to irritation, physical trauma, or hormonal factors. It is often found to involve the gums ...

Staging

There are many different staging- systems published. One of the most used is that of Radkowsky:

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 * maxillary swing Approach or Facial translocation 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- # 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

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.Textbook of Family Medicine, 8th Edition. Chapter 19 p.329. Rakel, MD.

References

External links

{{Respiratory neoplasia Tumour of the respiratory system