1. DIAGNOSTIC EVALUATION
2. EXAMINATION UNDER ANESTHESIA AND BIOPSY 
3. TREATMENT
4. POSTOPERATIVE RADIATION
5. FOLLOW UP
6. VI.BIBLIOGRAPHY

I. DIAGNOSTIC EVALUATION:

Clinical Evaluation:

• Complete history and physical examination
  
  Recording the presence and duration of symptoms such as pain, soreness of throat, otalgia, odynophagia, dysphagia, trismus and hoarseness. It should include history of risk factors such as the use of tobacco and alcohol, the occurrence an extent of weight loss and of all other medical conditions.

• Complete examination of the head and neck
  
  Includes examination of all the areas of the oral cavity, pharynx, and indirect laryngoscopy. If indirect laryngoscopy is not adequate, fiberoptic examination of the larynx and pharynx is necessary. The examination includes an assessment of the status of the dentition, as well as an evaluation of the status of the airway, documentation of vocal cord mobility, of the presence or absence of laryngeal crepitus, and of tumor extension to:
  
  - the medial, anterior and lateral wall of the pyriform sinus
  
  - the posterior pharyngeal wall or to the post cricoid region.
  
  Palpation of the neck bilaterally, recording the location (Group or Level I - VI), size, mobility, and relationship of the node(s) to adjacent structures.
  
  The staging of the primary and of the cervical lymph nodes must be documented.

Imaging Studies:

• Chest radiographs, PA and lateral 

  To rule out (1) A synchronous pulmonary tumor, (2) Acute or chronic pulmonary disease (3) Metastatic tumor. Abnormal findings on chest x-ray with suspicions lesions may need further imaging including a chest CT.

• CT scan of the primary and neck

  To assess the extent of the primary, its relation to the larynx, and extension into the post cricoid region, the paraglottic space, direct extension into the neck or invasion of the prevertebral fasciae. A CT scan with intravenous contrast is preferred provided the patient is not allergic to iodinated contrast material.

  To assess the presence or absence of cartilage invasion.
  
  In the absence of palpable adenopathy, they may be useful to assess the status of the cervical lymph nodes in patients that are obese or have a thick, muscular neck, and to rule out parapharyngeal and paratracheal adenopathy.
  
  When a large node is palpable in the neck, may be useful to clarify its relationship to the carotid artery, the paraspinal muscles or the cervical spine.

• Barium Swallow
  
  To rule out a synchronous primary tumor of the esophagus
  
  To ascertain the presence or absence of aspiration
  
  To assess mobility of tumor along the spine on deglutition 
  
  To assess the status of the stomach if a gastric transposition is considered for reconstruction.

• Preoperative tests according to institutional guidelines.
  
• Pulmonary function and arterial blood gases in the patients with COPD or who are candidates for partial laryngo-pharyngectomy.
  
• Baseline liver function tests (optional).

• Radiation therapy
  
  In anticipation of possible need for post-operative radiation therapy or to use radiation therapy as a definitive primary modality of treatment in early stage tumors.
  
• Dental
  
  To assess the status of the teeth and make recommendations considering that radiation therapy may be indicated. The evaluating dentist should be versed in the effects of radiotherapy on dentition. This evaluation should be done with knowledge of the treatment portals planned for the radiotherapy.
  
• Speech pathology
  
  For pre-operative counseling regarding possible post-operative speech and swallowing rehabilitation.

• Reconstructive (microvascular) surgery
  
  Based upon the amount of soft tissue to be resected and in anticipation of free tissue transfer to restore continuity of the alimentary tract.
  
• Internal Medicine, Cardiology, Pulmonology or Anesthesiology
  
  As needed to evaluate coexisting conditions that may preclude or increase the risk of general anesthesia, or may influence therapeutic decisions.
  
• Gastroenterology
  
  Placement of a gastrostomy may be necessary, prior to treatment, for nutritional rehabilitation.

To assess the superior and inferior limits of the tumor, its relationship to the apex of the pyriform sinus, the lateral pharyngeal wall, tonsillar fossa, base of tongue, the postcricoid region, the opposite pyriform sinus, and direct involvement of the larynx. To rule out the existence of other primary tumors in the aerodigestive tract.

• To assess the mobility of the tumor over the prevertebral fascia.
  
• In patients with advanced primary disease and airway impairment, the examination under anesthesia may require a tracheostomy to secure the airway. If this is necessary, the examination may be performed in conjunction with the definitive surgical procedure.
  
• Esophagoscopy is performed to evaluate extension into the post cricoid region or cervical esophagus.
  
• Bronchoscopy if indicated by clinical or radiographic findings.

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Primary tumor:

Treatment options for early hypopharyngeal cancer include: Partial pharyngectomy with or without partial laryngectomy or definitive radiotherapy.

Surgery:

T1 or T2 Carcinomas: 

Treatment options for early hypopharyngeal cancer include: Partial pharyngectomy with or without partial laryngectomy or definitive radiotherapy.

• Pyriform Sinus:

  Pyriform sinus cancer involving the medial wall of the pyriform sinus which do not involve the apex may be treated by partial laryngopharyngectomy. Patients with tumors of the medial anterior and lateral wall of the pyriform sinus which do not involve the apex of the sinus may be treated with a supracricoid hemilaryngopharyngectomy. This procedure is contraindicated in patients with chronic obstructive pulmonary disease or other co-morbid conditions which may accentuate postoperative pulmonary complications secondary to aspiration.

• Pharyngeal Wall:
  
  Tumors of the posterior hypopharyngeal wall may be resected through a transhyoid or lateral pharyngotomy. The resulting defect may be reconstructed with a STSG, a microvascular free tissue transfer utilizing a radial forearm flap or an open jejunal graft. The same performance criteria should be considered for this procedure as for partial laryngeal surgery.

• T3 and T4 Primary Tumors:
  
  Tumor with vocal cord fixation or massive tumors of the hypopharynx are treated primarily by surgery. In almost every instance, this requires a near total or total laryngectomy and partial or total pharyngectomy. These patients have a high likelihood of requiring reconstruction which may necessitate free jejunal transfer or flap reconstruction with either a pedicle flap (eg. myofascial, myocutaneous) or a radial forearm flap. Patients with extension into the post-cricoid region and cervical esophagus can be repaired, in most instances, with free jejunal transfer; however, a gastric transposition or a colon interposition may be necessary in patients with extensive cervical esophageal involvement.

Radiotherapy:

• T1 and T2 squamous carcinomas of the pyriform sinus, lateral and posterior hypopharyngeal walls may be treated with radiation therapy. Treatment includes comprehensive irradiation to both sides of the neck and the retropharyngeal and paratracheal lymph nodes. Selected patients who have a T1 or T2 primary tumor and advanced neck metastases (N2a or greater) may be treated with a neck dissection (levels I-V) followed by postoperative radiotherapy to the neck and definitive radiation to the primary tumor, as a method or organ preservation.
  
• Radiation therapy is not indicated as definitive treatment for advanced T3, T4, hypopharyngeal cancer. Many of these patients, however, do require postoperative radiation for adverse histopathologic findings at the primary site or regional lymph nodes.

Chemotherapy:

• Chemotherapy is not indicated for initial treatment of hypopharyngeal cancer, unless it is used under the auspices of an IRB approved, investigational protocol.

Treatment of the Neck:

In patients with hypopharyngeal cancer, the overall risk of occult lymph node metastases may be as high as 40-60%. Bilateral metastases are common. Retropharyngeal metastases may be present in patients with primaries of the pharyngeal walls. For this reason both sides of the neck and the retropharyngeal nodes must be treated electively with either surgery or radiotherapy.

Occult metastases may be present in levels II, III or IV. Level I and V are seldom involved in the absence of disease in levels II - IV. Level VI (paratracheal and perithyroidal lymph nodes) may be involved in patients with extensive T3 or T4 tumors of the pyriform sinus. 

Surgery:

• N0 Neck: Neck dissection (levels II - IV). Among patients undergoing total laryngectomy for pyriform sinus cancer, the ipsilateral level VI lymph nodes and hemi-thryoid are removed. Since bilateral metastases are common, both sides of the neck and the retropharyngeal nodes need to be addressed surgically. If this is not done, elective neck irradiation is necessary. 
  
  If lymph node metastasis is discovered at the time of the dissection, the procedure may need to be extended to include levels I and V and the sternocleidomastoid muscle, internal jugular vein and eleventh nerve. Unless these structures are directly invaded by tumor, they may be preserved.
  
• N1 Neck: Ipsilateral lateral neck dissection (levels II - IV) or a modified radical neck dissection with preservation of the spinal accessory nerve, sternocleidomastoid muscle and internal jugular vein, if possible. The structures directly involved by nodal metastases are resected. Levels I and V are included if indicated by the presence of metastasis. Level VI nodes and hemi-thyroid are removed in patients undergoing total laryngectomy.
  
• N2-3: Ipsilateral radical neck dissection or modified radical neck dissection levels I-V with preservation of the spinal accessory nerve, sternocleidomastoid muscle and internal jugular vein, if possible. Levels I and V are included if indicated by the presence of metastasis. Level VI nodes and hemi-thyroid are removed in patients undergoing total laryngectomy.
  
• Bilateral Neck Dissection: Among patient with extension into the post cricoid region or for tumors arising on the lateral posterior pharyngeal wall, or tumors arising in the pyriform sinus which extend to the lateral or posterior pharyngeal wall, bilateral neck dissection is indicated. The decision for the type of neck dissection is based upon the clinical presence or absence of lymph node metastasis and similar guidelines are used, as stated above. When possible, preservation of one jugular vein is desirable.

Reconstruction:

• Primary closure is preferable whenever possible.
  
• For patients with defects where primary closure of the pharynx is not possible or resection of the cervical esophagus is required, the reconstructive options include:
  
  A pedicle myocutaneous flap (pharyngeal patch)
  A free radial forearm flap (pharyngeal patch or tubed for circumferential defects)
  
  Free jejunal transfer for circumferential defects
  
  Gastric transposition or colon interposition is indicated for patients with extensive involvement of the cervical esophagus which requires total esophagectomy.

Adjunctive Surgical Management:

• Frozen section assessment of margins as needed to ensure adequate resection.
  
• Dental extractions, if necessary, for patients likely to receive postoperative radiotherapy.
  
• Placement of a nasogastric feeding tube.
  
• Gastrostomy or feeding jejunostomy is indicated for patients with a free jejunal transfer or gastric pull-up.

• Closed suction drainage of the neck.
  
• Tracheostomy.
  
• Peri-operative antibiotics.

Postoperative Care includes:

• Hospitalization for 7-14 days.
  
• Intensive care unit, as needed.
  
• Tube feedings until oral alimentation is reestablished.
  
• Low pressure suction to the drains.

• Removal of the drains when 24 hour output is less than 30-50 cc.
  
• Tracheostomy or stoma care.
  
• Sutures removed from the neck on the 7-10 postoperative day.
  
• For patients undergoing a laryngeal sparing procedure or partial laryngectomy, a modified barium swallow may be appropriate prior to initiating oral alimentation, to rule out significant aspiration.
  
• May require discharge with tracheotomy tube and feeding tube in place. Adequate training of patient and support personnel is needed before discharge can safely be effected. Ensure that a portable suction machine is available to the patient and consult a home-visiting nursing service (optional)
  
• Speech therapy and physical therapy, as needed

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Postoperative radiotherapy is indicated for:

T4 N0-N3 tumors.

T1 - 3 N0 tumors who have undergone resection of the primary and bilateral regional node dissection, when pathology reveals:

• Microscopically positive margins

• Presence of extensive intravascular or perineural invasion.

• Multiple histologically positive nodes at one level

• Positive nodes at multiple levels in the neck

• Presence of extracapsular tumor extension

     Timing:

• Radiation is initiated as soon as healing is deemed adequate (2 to 4 weeks). The usual dose range is 50 Gy to 70 Gy, in daily fractions of 1.8 to 2 Gy in 5 to 7 weeks. It may supplemented by a brachytherapy boost if dictated by pathology findings.

V.FOLLOW UP:

Follow-up appointments are scheduled on an individual basis determined by the risk of recurrence, to survey for the development of second primary tumors, to deal with morbidity from treatment (i.e. speech and swallowing problems as well as wound care), to provide social and psychological support, and to deal with comorbidity not directly related to the cancer itself.

• Periodic examinations by the head and neck surgeon may be necessary during radiation therapy in patients experiencing difficulty with nutritional intake, airway or pain control.

• Periodic examinations by the radiation oncologist and a dentist in patients that received radiation therapy

• After all treatment is completed a general formula which is modified according to the individual patient's characteristics is:
  
  1st year post treatment: 1-3 months
  
  2nd year post treatment: 2-4 months
  
  3rd year post treatment: 3- 6 months
  
  4th and 5th years: 4- 6 months
  
  After 5 years: Every 12 months

• Chest radiographs, yearly.

• Liver enzymes, yearly.

• Thyroid function tests should be monitored within the first year following completion of treatment if a thyroid lobectomy is performed (along with laryngectomy) or if the patient received radiation to the lower neck. These studies should be repeated according to clinical findings on follow-up examinations.

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Byers RM: Modified Neck Dissection: A Study of 967 cases from 1970-1980. AM J Surg 150:414-421, 1985.

Lindberg R: Distribution of cervical lymph node metastasis from squamous cell carcinoma of the upper respiratory and digestive tracts. Cancer 29:1446-1449, 1972.

Johnson JT, Bacon GW, Myers EN, Wagner RL: Medial vs. lateral wall pyriform sinus carcinoma: Implications for management of regional lymphatics. Head and Neck 16:401-405, 1995.

Clayman GL, Weber RS: Cancer of the hypopharynx and cervical esophagus. Cancer of The Head and Neck 3rd Ed, Chapter 21, Myers & Suen, eds, W.B. Saunders Philadelphia, 1995 in press.

Briant TDR, Bryce DP, Smith TJ: Carcinoma of the hypopharynx-a five year follow-up. J Otolaryngol 6:353, 1977.

Mendenhall WM, Parson JT, Devine JW et al: Squamous cell carcinoma of the pyriform sinus treated with surgery and/or radiotherapy. Head and Neck Surg 10:88, 1987.

El Badawi SA, Goepfert H, Fletcher GH et al: Squamous cell carcinoma of the pyriform sinus. laryngoscope 92:357, 1982.

Wang CC: Radiotherapeutic management of carcinoma of the posterior pharyngeal wall. Cancer 27:894, 1971.

Meoz-Mendez RT, Fletcher GH, Guillamondegui OM, Peters LJ: Analysis of the results of irradiation in the treatment of squamous cell carcinomas of the pharyngeal walls. Int J Radiat Oncol Biol Phys 4:579, 1978.

Garden AS, Morrison WH, Clayman GL, Ang KK, Peters LJ: Early squamous cell carcinoma of the hypopharynx: outcomes of treatment with radiation alone to the primary disease. Head & Neck 18:317-322, 1996.

Lefebvre J-L, Chevaliaer D, Luboinski B, Kirkpatrick A, Collette L, Sahmoud T: Larynx preservation in pyriform sinus cancer: preliminary results of a European organization for research and treatment of cancer phase III trial. Journal of the National Cancer Institute 88:890-9, 1996.

Laccourreve O, Merite-Drancy A, Brasnu D, Chabardes E, Cauchois R, Menard M, Laccourreve H: Supracricoid hemilaryngopharyngectomy in selected pyriform sinus carcinoma staged as T2. Laryngoscope 103(12):1373-9, 1993.