1. DIAGNOSTIC EVALUATION
2. CONTENT
3. DEFINITIVE TREATMENT
4. POSTOPERATIVE IRRADIATION
5. FOLLOW UP
6. BIBLIOGRAPHY

I.DIAGNOSTIC EVALUATION:

Clinical Evaluation:

• Complete history and physical examination.

• Examination of the head and neck.
  
  Includes inspection and palpation of the tumor. Fiberoptic examination to assess intra nasal tumor extent as well as possible nasopharyngeal extension. Cranial nerve examination with specific attention to facial anesthesia (V1, V2, V3) and extra-ocular motility. Documentation of visual acuity, and of the presence or absence of proptosis. Evaluation of the patient's dentition and occlusion. The presence or absence of palpable lymph nodes in the neck should be documented. If palpable nodes are present, the location (Group or Level I - VI), size, mobility, relationship of the node(s) to adjacent structures should be noted.
  
  The staging of the primary and of the cervical lymph nodes must be documented.

•  Biopsy of primary
  
  Transoral or transnasal biopsy, depending on most accessible portion of the tumor, is usually possible in the outpatient setting.

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 with and without contrast (Axial and coronal views, and bone windows are necessary):
  
  To assess the bony extent of the primary tumor
  
  To assess the status of the cervical lymph nodes
  
• MRI may be indicated to assess perineural spread, cranial base or intracranial extension. Also to distinguish tumor from secretions accumulated due to obstruction.

Laboratory Tests:

• Pre-anesthesia Laboratory tests (According to institutional guidelines):

Consultations:

• Radiation therapy 
  
  In anticipation of possible need for postoperative radiation and to consider primary radiation therapy.

• 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.

• Ophthalmology 
  
  For any tumor that will require resection of periorbital bone. In particular must assess the status of the contralateral eye.

• Dental prosthetics
  
  To take dental impressions and prepare for possible need of a palatal prosthesis.

•  Internal Medicine, Cardiology or Anesthesiology 
  
  As needed to evaluate coexisting conditions that may preclude or increase the risk of general anesthesia, or may influence therapeutic decisions.
  
• Neurosurgery : If craniofacial resection is indicated.
  
• Reconstructive (microvascular) surgery: If free flap reconstruction is indicated.
  
• Medical Oncology - if chemotherapy is indicated.

Top

Patient must understand that

• Facial incisions may be necessary for access.

• May need to return to operating room for pack removal and prosthesis modification.

• Palate defect will be rehabilitated with a prosthesis or free tissue transfer.

• Radiation may be necessary pre or postoperatively.

• The tumor may recur.

• The possibility of permanent diplopia or blindness. 

• The possibility of a STSG or a flap for lining the cavity.

Top

Primary tumor:

Adequate surgical resection, if consistent with reasonable functional outcome and peri-operative morbidity, is the preferred initial treatment modality. Exposure and resection of the tumor may be accomplished transorally or require facial incisions (Weber-Ferguson approach). The surgical treatment includes:

• Frozen section evaluation of margins as needed to ensure adequate resection.

• Dental extractions if necessary

• Wire or screw fixation of prosthesis

• Tracheostomy is generally not necessary but patient may need overnight intubation

• Orientation of tumor specimen for the pathologist, by the surgeon.

Neck:

N₀: Generally not treated due to low likelihood of metastases (< 15%). The risk of nodal metastases is high if the primary tumor extends to the nasopharynx, soft palate, or mucosa of oral cavity. In such cases, bilateral elective irradiation is indicated.

N₁: Ipsilateral supraomohyoid neck dissection or modified radical neck dissection with preservation of the spinal accessory nerve, sternocleidomastoid muscle or internal jugular vein if possible. Particular attention to level I (submandibular and facial nodes).

N_2-3: Ipsilateral radical neck dissection or modified radical neck dissection with preservation of the spinal accessory nerve, sternocleidomastoid muscle or internal jugular vein if possible.
Indications for postoperative radiation to the neck are outlined below.

Reconstruction:

• Palatal prosthesis or free tissue transfer.

Postoperative care:

• Hospitalization for 5-14 days.
  
• Oral feeding may be initiated in 24-48 hours when a temporary prosthesis is secured in place at the time of surgery.
  
• Low pressure suction to drains, when neck dissection is done.
  
• Removal of drains when output < 30-50 ml/24 hrs.
  
• Oral care with power sprays or rinses 2-3 times a day.
  
• Tracheostomy care - if necessary
  
  Change tracheostomy tube as needed.
  
  If discharge with tracheostomy in place is anticipated - instruct patient and at least one relative on tracheostomy care, ensure that a portable suction machine is available to the patient, and consult a home-visiting nursing service (optional).
  
• Suture removal from facial and neck wounds in 5-10 days.

Top

Indications: 

For stage T_1-3, N₀:

• Microscopically positive margins.
  
  
• Presence of perineural invasion.

For stage T_1-3, N₊

•  Postoperative radiation to the neck is indicated in most cases with clinically palpable metastases, or when the histopathology of the neck dissection specimen reveals multiple positive nodes or extracapsular extension of tumor.

A baseline postoperative CT or MRI scan is necessary prior to radiation therapy.

Total doses and fractionation:

• These are determined by the clinical and pathological findings. The usual range is 50 - 70 Gy in daily fractions of 1.8 to 2.0 Gy in 5 to 8 weeks. This include a brachytherapy boost when indicated by pathological findings.
  
• Technique - three dimensional "conformal" planning and treatment.

T iming:

• Radiation is initiated within a reasonable period after healing has occurred.

Top

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 the patient received radiation to the lower neck. These studies should be repeated according to clinical findings on follow-up examinations.
  
• Repeat CT/MRI scan at 3-6 months to establish a baseline exam and then at 6-12 month intervals depending on the clinical findings. 

Top

Isaacs JH Jr, Mooney S, Mendenhall WM, Parsons JT: Cancer of the maxillary sinus treated with surgery and/or radiation therapy. Am Surgeon 56:327-330, 1990.

Spiro JD, Soo KC, Spiro RH: Squamous carcinoma of the nasal cavity and paranasal sinuses. Am J Surg 158:328-332, 1989.

Weymuller EA Jr: Neoplasms. In: Otolaryngology-Head and Neck Surgery, Second Edition, Volume 1, Chapter 53. Cummings CW (ed), St. Louis, CV Mosby Publishing Co, pp. 941-954, 1993.

Willatt DJ, Morton RP, McCormick MS, Stell PM: Staging of maxillary cancer. Which classification? Ann Otol Rhinol Laryngol 96:137-141, 1987.

Top

1. DIAGNOSTIC EVALUATION
   
2. CONTENT
   
3. TREATMENT
   
4. RADIATION
   
5. FOLLOW UP

Clinical Evaluation:

• Complete history and physical examination.

• Examination of the head and neck.
  
  Includes inspection and palpation of the tumor. Fiberoptic examination to assess intra nasal tumor extent as well as possible nasopharyngeal extension. Cranial nerve examination with specific attention to facial anesthesia (V1, V2, V3) and extra-ocular motility. Documentation of visual acuity, and of the presence or absence of proptosis. Evaluation of the patient's dentition and occlusion. The presence or absence of palpable lymph nodes in the neck should be documented. If palpable nodes are present, the location (Group or Level I - VI), size, mobility, relationship of the node(s) to adjacent structures should be noted.
  
  The staging of the primary and of the cervical lymph nodes must be documented.

•  Biopsy of primary
  
  Transoral or transnasal biopsy, depending on most accessible portion of the tumor, is usually possible in the outpatient setting.

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 suspicious lesions may need further imaging including a chest CT.
  
•  CT scan with and without contrast (Axial and coronal views, and bone windows are necessary):
  
  To assess the bony extent of the primary tumor
  
  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 retropharyngeal adenopathy.
  
• MRI may be indicated to assess perineural spread, cranial base or intracranial extension. Also to distinguish tumor from obstructive secretions.

Laboratory Tests:

• Pre-anesthesia laboratory tests (According to institutional guidelines)

Consultations to be considered:

• Radiation therapy 
  
  In anticipation of possible need for postoperative or intraoperative radiation and to consider primary radiation therapy.

• 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.

• Ophthalmology 
  
  For any tumor that will require resection of periorbital bone or orbital exenteration. In particular must assess the status of the contralateral eye.

• Dental prosthetics
  
  To take dental impressions and prepare for possible need of a palatal prosthesis.

•  Internal Medicine, Cardiology or Anesthesiology 
  
  As needed to evaluate coexisting conditions that may preclude or increase the risk of general anesthesia, or may influence therapeutic decisions.
  
• Reconstructive (Microvascular) surgery.
  
  Repair of dural, orbital and palatal defect may require repair with free tissue transfer..
  
• Medical Oncology 
  
  To consider the possibility of concomitant chemo-radiation in patients with disease extent (to nasopharynx, sphenoid sinus, cavernous sinus) that precludes achieving clear surgical margins.
  

Top

Patient must understand that

• palate resection will be rehabilitated by a prosthesis or free tissue transfer

• facial incisions may be necessary for access, and that bone removal will result in facial disfigurement

• orbital resection is necessary

• craniotomy may result in neurological change

• may need to return to operating room for pack removal and prosthesis modification may be necessary

• radiation will be necessary post-operatively

• radiation may cause:

• reduced function of the remaining eye (cataract, optic nerve damage)

• hypo pituitarism

• the tumor may recur.

• STSG or a flap may be necessary for cavity lining.

Top

Primary tumor:

Participation in IRB approved clinical trial encouraged. Combined modality treatment is required in most cases. Adequate surgical resection, if consistent with a reasonable functional outcome and perioperative morbidity, is the preferred initial treatment modality. Exposure and resection of the tumor will require facial incisions and often craniotomy.
The surgical treatment includes:

• Frozen section evaluation of margins as needed to ensure adequate resection.

• Dental extractions if necessary.

• Wire or screw fixation of prosthesis

• Tracheostomy

• Orientation of tumor specimen for the pathologist, by the surgeon.Frozen section evaluation of margins as needed to ensure adequate resection.

Neck:

N₀: Generally not treated. However, the risk of nodal metastases is high if the primary tumor extends to the nasopharynx, soft palate, or oral cavity. In such cases, bilateral elective irradiation is indicated.

N₁: Ipsilateral supraomohyoid neck dissection or modified radical neck dissection with preservation of the spinal accessory nerve, sternocleidomastoid muscle or internal jugular vein if possible. Particular attention to level I (submandibular and facial nodes).

N_2-3:Ipsilateral radical neck dissection or modified radical neck dissection with preservation of the spinal accessory nerve, sternocleidomastoid muscle or internal jugular vein if possible.

Indications for postoperative radiation to the neck are outlined below.

If primary tumor is not resectable with clear margins consider radiation or concomitant chemotherapy and radiation.

Reconstruction:

• Palatal prosthesis
  
• Repair of dural an soft tissue defects (pericranial flap and/or free tissue transfer)

Postoperative care:

• Hospitalization for 10-14 days.
  
• Tube feedings until oral intake is adequate.
  
• Low pressure suction to drains.

        Removal of drains when output < 30-50 ml/24 hrs.

• Oral care with power sprays or rinses 2-3 times a day.
  
• Tracheostomy care
  
  Change tracheostomy as needed.
  
  If discharge with tracheostomy in place is anticipated - instruct patient and at least one relative on tracheostomy care, ensure that a portable suction machine is available to the patient, and consult a home-visiting nursing service (optional).
  
• Suture removal from facial and neck wounds in 5-10 days.

Top

Postoperative radiation therapy is indicated in all patients with advanced maxillary sinus cancer. The usual dose range is 50 - 70 Gy in daily fractions of 1.8 to 2.0 Gy in 5 to 8 weeks. This include a brachytherapy boost when indicated by pathological findings.

A baseline postoperative CT or MRI scan is necessary prior to radiation therapy. 

Radiation is initiated within a reasonable period after healing has occurred.

In patients in whom an adequate surgical resection is not consistent with a reasonable functional outcome or perioperative morbidity, radiation therapy alone might be used. Combined treatment with chemotherapy and radiation therapy appears to be superior to radiation therapy alone in the treatment of certain advanced squamous cell carcinomas of the head and neck. The optimal sequencing and delivery (IV vs intraarterial) of the two modalities is under investigation. Patients may be enrolled in IRB approved clinical protocols addressing therapeutic issues such as this.

Top

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 the patient received radiation to the lower neck. These studies should be repeated according to clinical findings on follow-up examinations.
  
• Repeat CT/MRI scan at 3-6 months to establish a baseline exam and then at 6-12 month intervals depending on the clinical findings.