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

I.DIAGNOSTIC EVALUATION:

Clinical Evaluation:

• Complete history.
  
• Complete examination of the head and neck.
  
  Includes nasal endoscopy to evaluate the superior nasal vault and posterior extent of lesion. Visual acuity and full extraocular motility should be documented. Evidence of proptosis or diplopia should be noted. Evaluation of cranial nerves with special attention to cranial nerves I, II, III, IV, V, and VI. Presence or absence of palpable lymph nodes in the neck should also be determined. If positive, the location, size, mobility, relationship to carotid and other adjacent structures and staging should be noted.
  
• Transnasal biopsy of primary.

Imaging studies: 

• Panoramic x-ray (Panorex) and/or dental X-rays of the maxillary teeth
  
  When necessary to adequately assess the status of the patient's dentition in anticipation of radiation therapy.
  
• 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 head as initial study (Axial and coronal views and bone windows are necessary):
  
  To assess the extent of the primary tumor and potential involvement of the anterior cranial base, medial and inferior orbital walls, and sphenoid sinus.

MRI scan of head:

To assess intracranial extension, if the anterior cranial base is eroded or there is a question of tumor extension posteriorly into the orbit or into the cavernous sinus. It may be useful to differentiate superior ethmoid or sphenoid sinus involvement by tumor from obstruction with retained secretions.

Laboratory tests:

Pre-anesthesia laboratory tests (According to institutional guidelines)

• Liver enzymes

Consultations:

• Neurosurgery consult
  
  Because of the potential for craniofacial resection if there is superior ethmoid/nasal cavity involvement or extension into the anterior cranial fossa as shown by CT/MRI scans.
  
• Radiation oncology consult
  
  In anticipation of either preoperative, postoperative or definitive radiation therapy.
  
• Medical Oncology consult
  
  For evaluation and treatment of patients with lymphoma, melanoma, advanced esthesioneuroblastoma patients with distant metastases, or patients who are not suitable for surgery.
  
• Ophthalmology consult
  
  Documentation of visual acuity, evaluation of any extraocular motility disturbances and proptosis. Optionally, for assistance in determination of the surgical treatment of the eye if the lamina papyracea has been eroded, and for postoperative evaluation and management of diplopia or epiphora.
  
• Reconstructive (microvascular) surgery.
  
  When it appears that the size of the superior defect will be large enough that it will require a free flap reconstruction.
  
• Prosthodontist
  
  If a maxillectomy must be performed and a prosthesis is necessary after surgery.
  
• Dental consultation
  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.
  
• 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.
  
• Speech Therapy (Optional)

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A biopsy usually is obtained in an outpatient setting, in the clinic, using local anesthesia and appropriate endonasal instruments.

NOTE: If a biopsy has been performed elsewhere, it is imperative that a review of the pathology at the treating institution be performed, with special studies done as deemed necessary, to confirm the diagnosis.

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 Primary tumor:

Surgical therapy with curative intent is considered in patients without evidence of distant metastases, and who have no medical contraindications to surgery, if consistent with a reasonable functional outcome. The decision on whether a craniotomy needs to be performed in conjunction with the facial resection, the so-called craniofacial resection, is dictated by the superior extent of the disease. If the tumor does not extend to the roof of the ethmoid or the cribriform plate, the resection can be performed from below utilizing the lateral rhinotomy or facial de-gloving approach, or endoscopic techniques in selected cases. 

Primary tumor (not including lymphoma, plasmacytoma):

There are two schools of thought. One is for adequate surgical resection of the primary as the initial treatment modality followed by postoperative radiotherapy and the other includes preoperative radiotherapy followed by an adequate surgical resection. There may be an advantage in treating neoplasms with a high potential for early distant metastases (rhabdomyosarcoma, small cell carcinoma) with chemotherapy initially, followed by surgery or radiation therapy. Chemotherapy may also be advantageous in the treatment of advanced (Stage C) esthesioneuroblastoma.

Eye:

If the bony orbit is intact preoperatively, the eye should be spared. Exenteration of the orbit is performed when there is bony erosion of the lamina papyracea or floor of the orbit with involvement of the periorbita, or tumor is present at the orbital apex. In selected cases, a segment of periorbita may be resected, with meticulous frozen section control, preserving the eye without jeopardizing the oncologic validity of the resection. (see below - alternative therapy). 

Reconstruction of superior defect:

Dural defects should be closed to obtain a water-tight seal whenever possible. Dural defects that occur when the dura is raised from the cribriform plate can usually be closed primarily. Larger dural defects can be closed using free grafts of pericranium, fascia lata, cadaver dura, or other types of fasciae. The suture repair can be reinforced with fibrin glue. The next layer is composed of a pericranial flap with the pedicle based inferiorly. Reinforcement of the nasopharyngeal surface with cartilage or bone grafts, fasciae, or a split thickness skin graft is optional.

Surgical treatment includes: 

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

Reattachment of medial canthal ligament with slight over-correction as compared to the unresected side.

Dacryocystrhinostomy or placement of nasolacrimal tube stents to prevent epiphora

Postoperative care includes:

• Perioperative antibiotic prophylaxis for a minimum of 48 hours.
  
• Hospitalization for 5-14 days with the first night postoperatively in an intensive care unit setting.
  
• If packing is used, packing in cavity for 7-10 days with anti-staphylococcal antibiotic coverage during this period. 
  
• Lumbar drain as needed postoperatively. A lumbar drain is not necessary in all cases, but is helpful when the dural closure is not water-tight.
  
• Bed rest with slight head elevation for 1-3 days followed by ambulation.
  
• Suture removal: 7 - 10 days postoperatively.
  
• CT scan on day 1 postoperatively and then on a prn basis.
  
  Tracheostomy may be necessary as part of the initial surgery, to prevent the possibility of tension pneumocephalus in patients undergoing extensive craniofacial resection.

A lternative Therapy:

For patients in whom adequate surgical resection is not consistent with a reasonable functional outcome or perioperative morbidity, definitive radiation therapy might be employed. In patients with advanced squamous cell carcinoma, esthesioneuroblastoma and SNUC an appropriate chemotherapy regimen may be used, in combination with radiation therapy, surgery or both, under the auspices of an IRB approved investigational protocol.

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Indications: All malignant tumors of this region treated surgically

Timing:

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

Total dose 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 includes a brachytherapy boost when indicated by pathological findings such as unsatisfactory margins.

Neck:

• Elective treatment of both sides of the neck is indicated when the tumor extends to the nasopharynx or the soft palate. Otherwise, the neck is treated only when palpable cervical metastases present. In that case an appropriate neck dissection is performed and both sides of the neck are treated with radiation postoperatively.

V.FOLLOW UP:

Cavity cleaning 2-3 weeks postoperatively and again at 4-6 weeks; at this time patient begins cavity irrigations.

Complete examination of the head and neck. 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. Exam following chemotherapy as needed.
  
• 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. (Optional)
  
• Liver enzymes, yearly. (Optional)
  
• 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. 

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Deutsch DB, Levine PA, Stewart FM, Firerson HF Jr., Cantrell RW: Sinonasal undifferentiated carcinoma: A ray of hope. Otolaryngol Head Neck Surg 1993, 108(6):697-700.

Carrau RL, Snyderman CH, Janecka IP, Sekhar L, Sen C, D'Amico F. Antibiotic prophylaxis in cranial base surgery. Head & Neck 1991; 13:311-317.

Snyderman CH, Costantino PD, Sekhar LN. Anterior approaches to the cranial base. Brain Surgery: Complication Avoidance and Management 1993; 2:2265-2281.

Snyderman CH, Janecka IP, Sekhar LN, Sen CN, Eibling DE. Anterior cranial base reconstruction: Role of galeal and pericranial flaps. Laryngoscope 1990; 100:607-614.

Snyderman CH, Sekhar LN, Sen CN, Janecka IP. Malignant skull base tumors. Neurosurgery Clinics of North America 1990; 1:243-259.

Eibling DE, Janecka IP, Snyderman CH, Cass SP. Meta-analysis of outcome in anterior skull base resection for squamous cell and undifferentiated carcinoma. Skull Base Surgery 1993; 3:123-129.

Carrau RL, Segas J, Nuss DW, Snyderman CH, Johnson JT. Role of skull base surgery for local control of sarcomas of the nasal cavity and paranasal sinuses. Eur Arch Otorhinolaryngol 1994; 251:350-356.

Janecka IP, Sekhar LN. Anterior and anterolateral craniofacial resection. Surgery of cranial base tumors 1993; 147-156.

Cantrell RW. Esthesioneuroblastoma. Surgery of cranial base tumors; 1993; 471-476.

Janecka IP, Sekhar LN, Myers EN. Nasal/paranasal sinus carcinoma. Surgery of cranial base tumors 1993; 497-505.

Carrau RL, Segas J, Nuss DW, Snyderman CH, et al. Squamous Cell Carcinoma of the Sinonasal Tract Invading the Orbit. Laryngoscope, 109:230-235, 1999.

McCary WS, Levine PA, Cantrell RW: Preservation of the eye in the treatment of sinonasal malignant neoplasms with orbital involvement: A confirmation of the original treatise. Arch of Otolaryngol Head Neck Surg 122:657-659, 1996.

Levine PA, Scher RL, Jane JA, Persing JA, Newman SA, Miller J, Cantrell RW: The craniofacial resection-eleven year experience at the University f Virginia: Problems and solutions. Arch of Otolaryngol Head Neck Surg 101:665-669, 1989.