Juvenile nasopharyngeal angiofibroma| aetiology, diagnosis, treatment

Benign sino-nasal tumors are abnormal groths that do not spread nor invade other parts of the body and they are not life threatening. They are non-cancerous but they are usually surgically removed for prevention and they have recurrence rate as cancerous tumours.

Classification and types:

 Benign Tumors of the Nose and Paranasal Sinuses are classified into:

a. Epithelial

• Papilloma. 
• Adenoma.
  

b. Non-epithelial 

• Hemangioma. 
• Osteoma.
• Fibro-osseous lesions. 
• Chondroma.
• Neurolemmoma.

Detailed review of Common Benign Tumors

1. Osteoma

Pathology:

Types:

• Compact (Ivory) osteoma: common in frontal sinus.
• Cancellous (spongy) osteoma: common in ethmoidal sinus.

Site: It occurs in junction areas between membranous and cartilaginous bone where remnants of embryonal periosteum persist.

Incidence 

• It is commoner in females than in males.
• It is common in Middle East area.

Association: multiple osteomata may be associated with intestinal polyposis (Gardner syndrome).

Symptoms:

• Asymptomatic in most of the cases (discovered accidentally).
• Painless slowly growing swelling.
• Manifestations of pressure and encroachment on the surrounding structures e.g. headache, proptosis, mucocele, nasal obstruction, deterioration of vision.

Signs:

• Hard, well defined swelling.
• May be seen in the nose.

Investigations:  Plain X ray -C.T.

Treatment: 

• Surgical excision if symptomatizing.
• This can be done either externally, or endoscopically.

2. Fibrous dysplasia

Definition: It is not a real tumor. It is an obscure bone disease where normal osseous tissue is absorbed and replaced by fibrous tissue and poorly formed bone trabeculae.

Etiology: Unknown, may be developmental.

Incidence: 

• 2.5% of all bony neoplasm.
• Common in the 1st and 2nd decades.
• Common in females.

Pathology: 

According to the affected sites, it may be:

1. Monostotic: single site. In the head and neck, the maxilla is the commonest site.
2. Polystotic: several sites including long bones, mostly unilateral .It may be associated with cutaneous pigmentation and endocrinal disturbance as precocious puberty, diabetes mellitus or hyperthyroidism (Albright syndrome).

• The growth tends to slow down after puberty and stops after complete osseous maturity.
• It does not turn malignant except if irradiated.
• Histologically, there is a mixture of fibrous and osseous tissue.

Symptoms: 

Painless slowly growing swelling mostly in the maxilla and alveolar process causing:

• Disfigurement. 
• Loosening of teeth.
• Nasal obstruction. 
• Proptosis.
• Facial pain and numbness due to involvement of infraorbital foramen.

Signs: 

• Diffuse, ill defined, non-tender, hard bony swelling.

Investigations: 

• X-ray: hyperdensity of the facial bones with ground glass appearance.
• Increased serum alkaline phosphatase.
• Radioactive bone scan will show an increased uptake in all the affected sites.

Treatment:

• As the lesion tends to stabilize after puberty, no treatment is indicated unless there are manifestations.
• Limited trimming is indicated if the mass is disfiguring or causing pressure manifestations.

3. Papilloma

Pathology: Squamous cell papilloma (wart), which arises from skin of vestibule.

Clinically: Pedunculated warty growth.

Treatment: surgical excision.

4. Hemangioma

Clinico-pathological types:

a. Capillary hemangioma (Bleeding polypus of septum).

• It presents as a soft red blue polyp on the nasal septum causing epistaxis.
• Treatment is by surgical excision with cauterization of the base. Laser may be used.

b. Cavernous hemangioma:

• Commoner in children. 
• It arises from the lateral nasal wall especially the area of the inferior and middle turbinates. I
• t causes epistaxis and sometimes nasal obstruction if large.
• Treatment is by excision either surgically, by laser or by cryo-surgery.

c. Multiple familial telangiectasia (Osler-Rendu disease)

• Congenital vascular malformation of hereditary origin. 
• It runs in families. It may involve the nose, lips, face, oral mucosa, palate, tongue, pharynx and larynx.
• It causes recurrent epistaxis. 
• It is treated by laser or surgical excision of the affected nasal mucous membrane.

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