Site:
Parapharyngeal space (Prestyloid and retro-styloid)
Histology:
Salivary neoplasms, neurilemomas, paragangliomas, most common (80% Benign, 20% Malignant.)
Stage:
T1-N0 and T2-N0

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

I.DIAGNOSTIC EVALUATION:

Clinical Evaluation:

Parapharyngeal tumors often present as an asymptomatic mass discovered on routine physical examination or by incidental imaging studies. Therefore, early detection is difficult.

  • Complete history and physical examination

    Important historical information includes recording the presence and duration of pain (throat, neck, headache), sense of throat fullness, dysphagia, voice change, ear fullness, pressure or hearing loss, awareness of a parotid area mass or neck mass(es). History of prior surgery to the head and neck area especially salivary glands, prior radiation therapy, alcohol, tobacco use, and general medical health should be obtained.

    If on the basis of history or imaging studies a paraganglioma is suspected, inquire about labile hypertension, tremulousness, headache, pallor, palpitations, and sweating.
  • Complete examination of the head and neck.

    Includes careful inspection of the oral cavity and oropharynx for medial displacement and bulging of the soft palate, tonsil, and hypopharynx. Bimanual palpation of this area should be done, with one hand in the pharynx and the other hand below the angle of the jaw region. Flexible fiberoptic examination of the nasopharynx with attention to the eustachian tube region, and evaluation of the larynx and hypopharynx with assessment of vocal cord mobility. Record all cranial nerve function including palatal deviation (upper X) X, XI, XII, V2, V3, VII. Carefully palpate the parotid gland, upper neck, and record size of any masses, consistency, presence of pulsations, mobility, and presence or absence of other neck masses or adenopathy.
  • Biopsy of primary

    Transoral biopsy of the tumor prior to surgery should not be performed. Fine-needle aspiration of prestyloid lesions either transorally or from the external approach is generally accurate in confirming the presence of a pleomorphic adenoma. Because of the variety of lesions encountered in the parapharyngeal space, FNA may augment the initial assessment, but for retrostyloid lesions often is inconclusive. For suspected metastatic or primary malignancy, FNA directed by CT or ultrasound may be helpful.

Imaging Studies:

Radiographic study of all parapharyngeal space tumors is essential. A computed tomography (CT) scan with contrast medium or a magnetic resonance imaging (MRI) study with gadolinium should be performed in all cases. Currently MRI provides the most useful preoperative information with triplaner views, to determine the tumor's extent, relationship to surrounding structures, and tumor differentiating characteristics.

Angiography should be done when carotid artery involvement is suspected or for highly vascular tumors, or tumors that invade the cervical vertebral bodies or extend to the skull base or intracranially. MR angiography may replace conventional angiography in many of the above cases.

Carotid occlusion studies must be done if tumor resection is planned for extensive malignant retrostyloid tumors or extensive vascular tumors that surround the carotid artery at the skull base.

Laboratory Tests:

Obtain preoperative tests according to institutional guidelines. Tests to rule out a catecholamine-producing tumor include 24-hour urine collection for vanillylmandelic acid, metanephrines, dopamine, epinephrine, and norepinephrine. Serum catecholamines, can also be analyzed.

Consultations:

Interventional neuroradiologist: for consideration of tumor embolization preoperatively, for extensive paraganglioma and for paragangliomas that extend intracranially.

Neurosurgeon: for all tumors with intracranial extension or obvious erosion of the skull base, cervical spine, or extensive vascular lesions, the combined talents of two surgical disciplines is helpful.

Radiation therapy: for known metastatic tumors or obvious malignant tumors, postoperative or definitive radiotherapy consultation may be necessary.

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II. EXAMINATION UNDER ANESTHESIA AND BIOPSY:

Generally this has no role in parapharyngeal tumors with the exception of metastatic lesions from a head and neck primary site.

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III. DEFINITIVE AND ADJUVANT TREATMENT:

Primary tumor:

  • Surgery

    Definitive statements regarding management of parapharyngeal tumors are difficult due to the variety and variable extent of tumors encountered in this area. For the most common lesions, prestyloid salivary pleomorphic adenomas, an external surgical approach is preferred. This should be done by an experienced surgeon with an operation that provides adequate tumor visualization and complete tumor removal with preservation of surrounding nerves and vessels. The cervical-parotid approach or cervical approach alone is usually indicated. The need for mandibular osteotomy to assist exposure is required in less than 20% of cases. This surgical approach is useful for most lesions encountered in the parapharyngeal space. Decisions regarding the need to operate on retrostyloid benign tumors should consider factors such as the patient's age, health, and expected morbidity from the surgical removal. Observation is the best choice for an asymptomatic nerve tumor in an elderly patient. 
  • Radiation

    Post-operative radiation is indicated for primary and metastatic malignant tumors of the parapharyngeal space.

    Radiation may be used as primary or palliative treatment of paragangliomas. 
  • Chemotherapy

    Chemotherapy combined with radiation therapy may be considered in malignant lesions not amenable to surgical excision.
Neck:
  • Surgery 

    A modified or selective neck dissection may be performed for malignant salivary tumors involving the parapharyngeal space that have a propensity to spread to regional lymph nodes.   
  • Radiation 

    Postoperative radiation can be given to the N0 neck if indicated for the primary site. For high-risk neck disease determined by clinical or pathologic findings, (i.e. extracapsular disease, desmoplasia, multiple nodes, low nodes, atypical metastasis), postoperative radiation therapy is usually given.

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V. FOLLOW UP:

After the removal of most benign tumors, clinical evaluation and imaging studies should be performed yearly or biannually to rule out recurrence. Recurrence of benign pleomorphic adenomas can occur up to 20 years, so follow-up at progressively longer intervals should be continued life?long.

For malignant tumors, the exact frequency of follow-up and testing will depend on the exact tumor type. In general:

1st year  post-treatment post-treatment
2nd year  post-treatment 2-4 months
3rd year   post-treatment  3-6 months
4th & 5th years post-treatment  6-12 months
After 5 years  post-treatment Annually
  • Imaging studies of the parapharyngeal space: 1-2x a year during the first 5 years and only if new symptoms or findings occur after that time 

  • Annual chest x-ray for malignant lesion

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VI. BIBLIOGRAPHY:

Olsen, K.D. Tumors & surgery of the parapharyngeal space ? Laryngoscope, Vol. 104 No 5 Part 2 Supplement No. 63 May 1994

Som, P.M., Biller, H.F. and Lawson, W.: Tumors of the Parapharyngeal Space: Preoperative Evaluation, Diagnosis and Surgical Approaches. Ann Otol Rhinol Laryngol, 90:3?15, 1981

Gluckman, J.L.: Parapharyngeal Mass, Retrieved May 21, 1999, from the World Wide Web: