د.حسين
L.4+5   Tumors of the pharynx

           Tumers of the pharynx are usually classified according to their site of origin (nasopharyngeal, oropharyngeal, &hypopharyngeal).

Nasopharyngeal Tumers

A. Benign tumers:
Benign tumers in the nasopharynx are rare & the commonest tumer is juvenile nasopharyngeal angiofibroma.
  
       Juvenile nasopharyngeal angiofibroma:
           It’s a benign fibrovascular tumer, usually arises from the sphenopalatine foramen (at the junction of the nasopharynx with the nasal cavity) forming a bilobed dumb-bell swelling straddling the foramen. It usually affects males between 8-16 yr. old due to hormonal activity &it has a high tendency to bleed (a cardinal symptom of the disease).
   Extension:
Anteriorly to the nasal cavity
Posteriorly to the sphenoid sinus
Medially to the nasopharynx
Laterally to the sphenopalatine foramen

Clinical features:
1.	Recurrent spontaneous sudden sever epistaxis (the cardinal symptom).
2.	Progressive nasal obstruction.
3.	Hyponasal speech (rhinolalia clausa) due to nasal obstruction.
4.	Conductive deafness due to Eustachian tube obstruction (unilateral).
5.	On examination a smooth, lobulated, rubbery mass is found in the nasopharynx which is reddish or gray in colour.
6.	Late features due to extension include:
-	Broadening of the nasal bridge.
-	Unilateral prominence of the cheek.
-	Proptosis (displacement of the globe of the eye).

Diagnosis:
1.	Clinical features.
2.	Radiology:
-	Plain x-ray: show sinusitis, destruction of the wall of the sinus.
-	CT scan: show the dimensions & extension of the tumer & its consistency.
-	MRI: show the details of the soft tissues & the mass.
-	  Angiography: shows the vascularity of the tumer  & also for preoperative embolization to decrease bleeding during surgery.
Note:
Biopsy is contraindicated because of uncontrolled bleeding.                                               

Major blood supply of angiofibroma:
1.	Ipsilateral internal maxillary artery.
2.	Contralateral =        =             =.
3.	 Ascending pharyngeal artery.
4.	Large tumers have blood supply from internal carotid & vertebral arteries. 

Treatment:
1.	Surgical excision of the tumer is the treatment of choice. The patient should be well prepared & nourished & we must prepare     4-6 pints of blood before operation. 
     
2.	Radiotherapy :
Usually used in the following:
-	Inoperable tumer.
-	Intracranial extension.
-	Recurrent tumer.

Complications of surgery:
1.	Palatal fistula.
2.	Anesthesia of the cheek.
3.	Ectropian of the lower eyelid.
4.	Crustation of the nasal cavity.
5.	Recurrence.

                                                                                                                                        

B.Malignant tumers of the nasopharynx:
 
    Malignant tumers in the nasopharynx are more common than benign tumers. Sequamous cell carcinoma is the most common malignant tumer specially occur in chines people other tumers are very rare as follows:

1.	Epithelial tumers: nasopharyngeal CA, adeno CA, adenoid cystic  CA, mucoepidermoid CA. 
      2.None epithelial tumers: 
       -Lymphomas 
       -Sarcomas (chondrosarcoma, osteosarcoma, rhabdomyosarcoma)
       -Malignant melanoma.       

Sequamous cell carcinoma

      It’s the commonest malignant tumer in the nasopharynx with varying degrees of differentiation.

Clinical types: 1.   Prolifrative (like a polyp causing obstructive  
                                symptoms).          
                         2.    Ulcerative (causing bleeding).
                         3.    Infiltrative (causing deep invasion).
Etiology:
  Unknown but many factors can play a role such as:
Smoking, alcohol, deep x-ray therapy, E.B Virus (especially in lymphoma).

Site:
The commonest site is the fossa of Rosen Muller & could occur in the posterosuperior wall.

Clinical features:
1.	 Metastases in the lymph nodes of the neck: Its often the presenting symptom unilateral or bilateral  & the first group involved are retropharyngeal lymph nodes (which can not be palpated) then to the cervical lymph nodes that’s why the disease discovered late.
2.	Symptoms of local invasion: (Trotters triad)
-	Conductive deafness (secretory otitis media).
-	Elevation & fixation of the homolateral soft palate.
-	Pain in the side of the head.
3.	Other symptoms of invasion:
-Internal strabismus (6th CN pulsy).
-Exophthalmos.
-Jugular foramen syndrome (9th, 10th  & 11th CN pulsy)
4.	Nasal obstruction in the prolifrative type.
5.	Epistaxis in the ulcerative type.

Diagnosis:
1.	Clinical features & post nasal space examination.
2.	Radiology:
-	Plain x-rays (postnasal space) show soft tissue mass & bone erosion.
-	CT scan shows the size, shape & extension of the mass & any bony erosion.
-	MRI for soft tissue details & intracranial extension.
3.	Biopsy: give the definitive diagnosis.

Treatment:
1.	Radiotherapy is the mainstay treatment for nasopharyngeal CA including the lymph node metastases.                                                                                                      
2.	Surgery: 
-Radical neck dissection for radiosensitive nodes & post radiation cervical metastases.
-Salvage surgery for recurrent tumers.

Tumors of the oropharynx

       The oropharynx extends from the level of the hard palate above to the hyoid bone below & is divided into:
1.	Palatine arch: which include the soft palate& the anterior tonsilar
                             pillars.
2.	Oropharynx proper:which include the lateral & the posterior 
                                       pharyngeal walls,base of the tongue,the 
                                       tonsils,posterior pillar & the valleculae.
        The reason for this is that the tumers in the palatine arch (especially SCC) behave differently (less aggressive & metastases later) than other parts of the oropharynx, so the treatment is different. The tumers are either benign (rare & mostly papilloma) or malignant (more common especially in elderly patient).

Pathology:
The oropharynx has three types of tissues which give rise 3-distinct tumers as follows:
1.	Sequamous epithelial lining: give rise to SCC which is the commonest type of tumer about 70% of oropharyngeal tumers.
2.	Tonsils & lymphoid tissue in the base of the tongue:       which give  
    rise to none Hodgkin’s lymphoma (about 25% of oroph. tumers).
3.	Minor  salivary  glands  within the  epithelia:      give rise to minor 
    salivary gland tumers(adeno CA ,adenoidcystic CA     ,mucoepidermoid CA)forming about 5% of the tumers.

Sequamous cell carcinoma:
       This tumer has 2-clinical types either exophytic (usually follows leukoplakia with superficial local spread) or ulcerative type (causing deep invasion of the soft tissues with occasional bleeding). The etiological factors involved are(reverse smoking in Asia, alcohol, mucosal atrophy associated with iron deficiency anemia, syphilis,human papilloma virus & elderly patient with tooth caries).

Lymphoma:
      Usually none Hodgkin’s lymphoma, which arises as nodular, swelling in the tonsil region or base of the tongue, followed by ulceration. In this tumer we must investigate for a generalized disease (especially for the liver & spleen) to do proper treatment .The risk factors are (Sjogrens disease, decreased immunity due to chronic malaria, Epstein-Barr virus EBV & human T-lymphotrophic virus type 1).

Minor salivary gland tumers:
      These are usually malignant tumers (opposite to major salivary gland tumers, which are usually benign tumers) the tumer is usually smooth non-ulcerated swelling, it usually arises in the tonsil area & is mostly adenoidcystic carcinoma. It spread slowly by perineural lymphatics to the lymph nodes, bone  & lung, but it has good prognosis (80%) if discovered early.

Clinical features of oropharyngeal tumers:
1.	Lymph node metastases in the neck (60%).
2.	Sore throat.
3.	Referred otalgia.
4.	Dysphagia & odenophagia.
5.	Plummy voice.
6.	Trismus.
7.	Bleeding.
8.	Fixation of the tongue.

Investigations:
1.	FNA fine needle aspiration cytology:
This investigation is easy to do & used for the neck mass to differentiate between reactive & carcinomatous lymph node & also between branchial cyst & metastatic lymph node.
2. Radiology: chest x-ray if lymphoma is suspected.
                        CT scan for the tumer, neck, chest, abdomen & pelvis.
                        MRI.
3.Ultrasound: for the lymph nodes & as a guide for FNA.
4. Biopsy: is the definitive diagnostic test.
5. Blood investigation: CBP & ESR with blood film.
6. Bone morrow aspiration & biopsy to diagnose lymphoma.

Treatment:
Sequamous cell carcinoma:
1.	Radiotherapy: usually used in SCC & localized lymphoma.
2.	Surgery:  very difficult & destructive procedure called:commando 
    operation (combined neck dissection,mandibulectomy & resection                      
    of  the oropharynx).
        
3.Surgery & radiotherapy: it gives the best results because of occasional failure of radiotherapy.       
4.Chemotherapy: has a limited role in SCC & used as adjuvant to  
radiotherapy & palliative in advanced tumers.
5.Cryosurgery: it decreases bleeding & pain during surgery usually
used in small recurrent tumers & benign tumers.   
6. Laser surgery: used in patient who cannot undergo reconstructive 
      surgery.
7. Reconstruction after surgery: used to restore the shape & function 
    of the area operated upon , many types of flaps can be used (local,
    axial,myocutanuous & free vascularised flaps.

Non-Hodgkin’s lymphoma:
1.	Localized: radiotherapy followed by chemotherapy.
2.	Disseminated: systemic chemotherapy.

Minor salivary gland tumers:
1.	Benign: local excision.
2.	Malignant: radical wide excision.
3.	No place for radiotherapy except for palliation.

Tumors of the hypopharynx

     The hypopharynx extend from the level of the hyoid bone above to the level of the lower border of the cricoid cartilage below, its lined by sequamous epithelia & have a rich submucosal lymphatic vessels (that’s why submucosal spread is very common to the esophagus & hence poor prognosis). It has 3-areas:
   Pyriform fossa: it’s a funnel shape channels on each side of the larynx bounded laterally by the thyroid cartilage & medially by the aryepiglottic fold.
   Post cricoid area: it consists of the mucosa covering the cricoid cartilage in the posterior part of the larynx, to the level of the inferior border of the cricoid cartilage.
  Posterior pharyngeal wall: connecting the 2-pyriform fossae.
Pathology: 
Benign tumers: Non-epithelial tumers (fibroma, lieomyoma).
                           Epithelial tumers (minor salivary gland tumers 
                            treated by local excision.
 Malignant tumers: Epithelial tumers mostly SCC,  tumers are minor salivary gland tumers.                                                                                                                                                                                  
Etiology:
1.	Female in post cricoid CA. 
2.	Elderly patient.
3.	Precipitating factors: alcohol, smoking & radiotherapy.
4.	Risk factors: tea, low serum cholesterol, vitamin-A deficiency & iron deficiency.

Clinical features of hypopharyngeal tumers:     
1.	Dysphagia: is the commonest symptom, which is constant & progressive, starting for solid & then for liquid food.
2.	 Pain: usually referred otalgia or odenophagia.
3.	 Hemoptysis: unusual & occurs in pyriform fossa tumer.
4.	 Hoarseness of voice: due to involvement of the larynx.
5.	 Neck mass: either the lymph node or the tumer invading the neck.
6.	 Weight loss: is very fast in these tumers.    

Investigations: 
1.	Full blood count.
2.	Serum iron study: especially in females.
3.	 Radiology: -plain x-ray (lateral of the neck & chest x-ray).
                         -CT & MRI.
                         -Contrast study for dysphagia.
4.	Endoscopy:
5.	 Biopsy: for the tumer & FNA for the metastatic lymph node.

Treatment:  
1.Pyriform fossa tumers:        small  tumers  by  radiotherapy  with or 
   without radical neck dissection , large tumers treated by   a massive     
   operation called:      total laryngopharyngoesophagectomy       with 
   stomach pull-up.
2.post-cricoid tumer: small tumers (<5 cm) treated by radiotherapy or 
   partial surgery with preservation of the larynx.  Large tumers if the 
 anterior spinal ligament is not involved treated by total laryngopharyngoesophagectomy    with stomach pull-up.
3.Neck: 
              N0 radiotherapy
              N1 (<3cm) radiotherapy & surgery for recurrent lymph node
              N2 N3 surgery & post operative radiotherapy.

4.Reconstruction after surgery:
    Most common method is gastric transposition (stomach pull-up). The colon & jejunum can be used also.
Paterson-Brown-Kelly syndrome: (Plummer-Vinson synd.)

   Disease of the postcricoid area of unknown etiology, which may be immune in origin & is a major predisposing factor for post cricoid CA.
Clinical features: 
1.	Progressive dysphagia: due to a web in the post cricoid area.
2.	Iron deficiency anemia with oral manifestation of anemia (angular stomatitis, glossitis, and cheilosis).
3.	Common in females (30-50 yr.).
4.	Associated with mucosal atrophy of the GIT.

Diagnosis:
By clinical features & investigations (CBP, serum iron study & Ba-swallow showing filling defect in the post cricoid area).

Treatment:
1.	Iron supplement.
2.	Esophagoscopy: to excise the web or to dilate the esophagus.