1. DIAGNOSTIC EVALUATION
2. TREATMENT
3. FOLLOW UP
4. BIBLIOGRAPHY

I. DIAGNOSTIC EVALUATION

• Complete clinical evaluation.

• Complete history and physical examination

• Complete examination of the head and neck:
  
  Head and neck examinations should focus on the extent of the disease, intracranial extension and involvement of cranial nerve structures. Documentation of the visual acuity, presence or absence of proptosis and examination of other sites depending on the region involved. Region I includes tumors that involve the anterior cranial fossa; Region II tumors that involve the middle fossa and Region III tumors that arise about the ear canal, temporal bone with extension to and possible involvement of the posterior fossa.

Biopsy of Primary:

• In general, this can be done endoscopically for tumors involving regions 1 or 3 and using CT-guided needle biopsy for tumors involving Region II. Occasionally, open biopsy is needed for tumors of Region II.

Imaging Studies:

• Chest x-rays, PA and lateral

• CT - MRI scan with contrast to assess both the bony extent of the primary neoplasm and the soft tissue involvement of the skull base or intracranial extension.
  Balloon occlusion test and blood flow scan if internal carotid artery is involved.

Laboratory Tests:

• Pre-anesthesia laboratory test (according to institutional guidelines).

Consultations:

• Neurosurgery - if craniotomy is anticipated.

• Radiation Therapy- since radiation therapy is necessary in the majority of advanced malignant neoplasm

• Plastic Surgery - if free flap reconstruction is anticipated.
  
• Some facial deformity or impaired function may result
  
• Facial prosthesis may be necessary.
  
  

Consent:

Patient must understand that:

• Patient's knowledge of incision will be necessary.

• Radiation may be necessary pre- or post operatively.

• Craniotomy may result in neurologic deficits (detail depends on site) including the possibility of permanent diplopia or blindness, facial numbness, deafness, or facial paralysis.

• The need for pedicled or free-flap repair.

• Risk of complications (40-50%), free flap failure (3-5%), wound breakdown (15%).

Top

Primary tumor:

Exposure and resection of the tumor may be accomplished using lateral rhinotomy, facial degloving approach, maxillotomy approach, maxillectomy, or other facial incision. Tumors involving Region II will require craniotomy combined with a cervico-facial incision and mobilization of the parotid gland, which places the facial nerve at risk. Orbital-zygomatic osteotomies and mandibular condylectomy are usually required for access. For region III tumors, subtotal or total temporal bone resection with sacrifice of the facial nerve is necessary in the majority of patients. Occipital craniotomy and combination approaches may be necessary.

Surgical Treatment:

• Tracheostomy

• Lumbar subarachnoid drain

• Neuro monitoring

• Eye protection

• Incision

• Soft tissue dissection

• Osteotomy/Ostectomy

• Orbital exenteration

• Tumor resection

• Intraoperative frozen section

Reconstruction:

• Defect repair with free flap, pedicled flap or free graft

• Osteosynthesis

• Palatal prosthesis

• Intraosseous implants

Post Operative Care:

• Hospitalization: 14 to 21 days

• Oral intake initiated within 24-48 hours in patients with secured prosthesis.

• Low pressure suction to drain when necessary.

• Oral hygiene with dental water pik 2-3 times per day.

• Tracheostomy care. If necessary.

• Lumbar drain usually needed for 3-7 days.

• Suture removal from facial and neck wounds 6-10 days.

Post Operative Radiation:

• Indicated for many malignant lesions that transgress the skull base.

• Total doses and fractionation: The total dose range is 50-70 Gy that is given in daily fraction of 1.8 to 2.0 Gy over 5-6 weeks. Proton beam therapy should be considered in patients with clival chordomas.

Timing:

• Radiation is initiated within 4-6 weeks postoperatively.

Top

• Follow-up appointments are scheduled on an individual basis determined by the risk of recurrence, the need for supportive therapy (speech, swallowing, physiotherapy, social and psychological support).
• Periodic examination by the Head and Neck surgeon, neurosurgeon, radiation and medical oncologist. After all treatment is completed, a general formula is modified according to the individual characteristics:
  
  

  First year post treatment:	q. 1 months.
  Second year post treatment:	q. 2 months.
  Third year post treatment:	q. 3-4 months.
  Fourth and Fifth year:	q. 6 mos.

  
  
  
• Chest x-ray yearly
• CT scan/MRI at 6 months to establish a baseline and then at 3-12 months depending on the clinical findings

Top

Donald PJ: Surgery of the Skull Base; Lippincott-Raven, Philadelphia, 1998.

Skull base surgery: anatomy, biology, and technology. Editors, Ivo P. Janecka, Klaus Tiedemann. Philadelphia: Lippincott-Raven, 1997.

Surgery of cranial base tumors. Editors, Laligam N. Sekhar, Ivo P. Janecka. New York: Raven Press, 1993.

Kraus DH, Shah JP, Arbit E, Galicich JH, Strong EW. Complications of craniofacial resection for tumors involving the anterior skull base. Head & Neck 1994; 16(4):307-312

Dias FL, Sá GM, Kligerman J, Lopes HF, Wance JR, Paiva FP, Benévolo A, Freitas EQ. Complications of anterior craniofacial resection. Head & Neck; October 1998, pp 1-9.

Dos Santos LR, Cernea CR, Brandao LG, Sigueira MG, Vellutini EA, Velazco OP, Cruz OL, Morais-Besteiro J, Freitas CA. Results and prognostic factors in skull base surgery. American Journal of Surgery 1994; 168(5):481-484.