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Primary Parapharyngeal
Tumors
Primary parapharyngeal tumors
• Most of the tumors in parapharyngeal
space are metastatic disease or direct
extension from adjacent spaces
• 0.5% of all head and neck tumors
• Benign tumor 80%
• Malignant tumor 20%
Anatomy
• Potential deep neck
space
• Shaped as an
inverted pyramid
• Base of the pyramid:
skull base
• Apex of the pyramid:
greater cornu of the
hyoid bone
Anatomy
• Superior:
small portion
of temporal
bone
• Inferior:
junction of the
posterior belly
of the digastric
and the hyoid
bone
Anatomy
• Medial: pharyngobasilar fascia and
pharyngeal wall
• Lateral:
• medial pterygoid muscle fascia
• Mandibular ramus
• Retromandibular portion of the deep lobe of the
parotid gland
• Posterior belly of digastric muscle
Anatomy
• Lateral: two ligaments
– Sphenomandibular ligament
– Stylomandibular ligament
• Posterior: vertebral fascia and paravertebral
muscle
• Anterior: pterygomandibular raphe and medial
pterygoid muscle fascia
Anatomy
• Tensor-
vascular-styloid
fascia separates
parapharyngeal
spaces to two
compartments:
– Prestyloid
– Poststyloid
Anatomy
• Prestyloid compartment contents:
– Retromandibular portion of the deep lobe of
the parotid gland
– Minor or ectopic salivary gland
– CN V branch to tensor veli palatini muscle
– Ascending pharyngeal artery and venous
plexus
– Most fat
Anatomy
• Poststyloid compartment contents
– Carotid artery
– Internal jugular vein
– CN IX to XII
– Cervical sympathetic chain
– Glomus tissues
Differential diagnosis
• Location of the tumor
– Prestyloid:
• salivary gland neoplasm
• lipoma
• rare neurogenic tumors
– Poststyloid:
• Schwannoma
• Paraganglioma
• neurofibroma
Salivary gland neoplasm
• Most common primary parapharyngeal tumor
(40%-50%)
• Pleomorphic adenoma is most common
• From deep lobe of the parotid gland
– extend through the stylomandibular tunnel, “dumbbell”
appearance on CT scan
– displace tonsil and soft palate and cause obstruction
of nasopharynx
• From minor salivary gland lying in
parapharyngeal fat
Salivary gland neoplasm
• Malignant parapharyngeal salivary gland
– Frequency varies from 24% to 75%
– Mucoepidermoid carcinoma
– Adenoid cystic carcinoma
– Acinic cell carcinoma
– Malignant mixed carcinoma
– Squamous cell carcinoma
– Adenocarcinoma
– Malignant Warthin’s tumor
Neurogenic tumor
• Second most common primary parapharyngeal
tumor
– Schwannoma
• Vagus nerve
• Cervical sympathetic chain
– Paraganglioma
• Vagal paraganglioma
• Carotid body tumors
– Neurofibroma
– Malignant neurogenic tumor
Clinical presentation
• Clinical detection is difficult
• Tumor size 2.5 to 3.0 cm to be detected
clinically
• Asymptomatic mass
– Mild bulging of soft palate or tonsillar region
– Palpable mass at angle of mandible
Clinical symptoms
• Prestyloid
– Serous otitis media
– Voice change
– Nasal obstruction
– Dyspnea
• Poststyloid
– Compress CN 9th
, 10th
, 11th
, 12th
or sympathetic chain
– Hoarseness, dysphagia, dysarthria, Horner’s
syndrome
• Cranial nerve paralysis, pain, trismus suggest
malignancy
CT scan
• Locates tumor to prestyloid vs poststyloid
– Prestyloid tumor displace carotid artery posteriorly
– Poststyloid tumor displace carotid artery anteriorly
• Fat plane between mass and parotid
• Enhancement of lesion
– Schwannoma, paraganglioma, hemangioma,
hemangiopericytoma, aneurysm
• Bone erosion due to malignancy
• Limited soft tissue detail
MRI
• Most useful study
• Relationship of mass and carotid more
easily seen than with CT
• Characteristic appearances of tumor types
on MRI allows preoperative Dx in 90-95%
of patients
MRI
• Pleomorphic
adenoma
– Low intensity on T1
– High intensity on T2
– Displace carotid
posteriorly
MRI
• Schwannoma
– High intensity on T2
– Displace carotid
anteriorly
MRI
• Paraganglioma
– “salt and pepper”
• Angiogram
– Define vascular anatomy
– Carotid occlusion test
– Tumor embolization 1 day prior to surgery
• FNA
Transparotid approach
• For deep lobe of parotid lesion
• Superficial parotidectomy with facial nerve
preservation
• Retract facial nerve from the deep parotid
lobe
• Dissect posterior and inferior around
mandible
• Improve access by mandibulotomy
Transcervical approach
• For poststyloid tumor
• Transverse incision at
level of hyoid
• Submandibular gland
removed or retracted
• Incision through the fascia
deep to the submandibular
space
• Increase exposure by
releasing digastric,
stylohyoid, styloglossus
from hyoid, cut
stylomandibular ligament,
mandibulotomy
Cervical-parotid approach
• Extend cervical incision up infront of ear
• Identify facial nerve
• Divide posterior belly digastric
• Divide styloglossus, stylohyoid close to
styloid process
• Divide stylomandibular ligament
• Can combine with mandibulotomy
Cervical-parotid approach
Indications
• Can be used to remove majority of the
parapharyngeal tumor
– All deep lobe parotid tumors and extraparotid
salivary tumors
– Low grade malignant tumors of deep lobe of
parotid
– Many poststyloid tumors, including most
neurogenic tumors and small paragangliomas
Cervical-transpharyngeal
• “Mandibular swing”
• Midline lip splitting or
visor flap
• Mandibulotomy anteriorly,
incise along floor of
mouth to anterior tonsillar
pillar
• Identify hypoglossal
nerve and lingual nerve
• Divide styloglossus and
stylopharyngeus muscle
• Need tracheotomy
Cervical-transpharyngeal
indications
• All vascular tumors that extend into the
superior portion of the parapharyngeal
space
• Malignant tumor invaded skull base or
vertebral body
Conclusion
• Rare tumor in an complex anatomical area
• Subtle clinical presentation
• Radiographic imaging is important
• Prestyloid vs poststyloid space
• Surgery is the main treatment
• When not to operate

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Primary Parapharyngeal Tumors: Rare Head and Neck Lesions

  • 2. Primary parapharyngeal tumors • Most of the tumors in parapharyngeal space are metastatic disease or direct extension from adjacent spaces • 0.5% of all head and neck tumors • Benign tumor 80% • Malignant tumor 20%
  • 3. Anatomy • Potential deep neck space • Shaped as an inverted pyramid • Base of the pyramid: skull base • Apex of the pyramid: greater cornu of the hyoid bone
  • 4. Anatomy • Superior: small portion of temporal bone • Inferior: junction of the posterior belly of the digastric and the hyoid bone
  • 5. Anatomy • Medial: pharyngobasilar fascia and pharyngeal wall • Lateral: • medial pterygoid muscle fascia • Mandibular ramus • Retromandibular portion of the deep lobe of the parotid gland • Posterior belly of digastric muscle
  • 6. Anatomy • Lateral: two ligaments – Sphenomandibular ligament – Stylomandibular ligament • Posterior: vertebral fascia and paravertebral muscle • Anterior: pterygomandibular raphe and medial pterygoid muscle fascia
  • 7. Anatomy • Tensor- vascular-styloid fascia separates parapharyngeal spaces to two compartments: – Prestyloid – Poststyloid
  • 8. Anatomy • Prestyloid compartment contents: – Retromandibular portion of the deep lobe of the parotid gland – Minor or ectopic salivary gland – CN V branch to tensor veli palatini muscle – Ascending pharyngeal artery and venous plexus – Most fat
  • 9. Anatomy • Poststyloid compartment contents – Carotid artery – Internal jugular vein – CN IX to XII – Cervical sympathetic chain – Glomus tissues
  • 10. Differential diagnosis • Location of the tumor – Prestyloid: • salivary gland neoplasm • lipoma • rare neurogenic tumors – Poststyloid: • Schwannoma • Paraganglioma • neurofibroma
  • 11. Salivary gland neoplasm • Most common primary parapharyngeal tumor (40%-50%) • Pleomorphic adenoma is most common • From deep lobe of the parotid gland – extend through the stylomandibular tunnel, “dumbbell” appearance on CT scan – displace tonsil and soft palate and cause obstruction of nasopharynx • From minor salivary gland lying in parapharyngeal fat
  • 12.
  • 13. Salivary gland neoplasm • Malignant parapharyngeal salivary gland – Frequency varies from 24% to 75% – Mucoepidermoid carcinoma – Adenoid cystic carcinoma – Acinic cell carcinoma – Malignant mixed carcinoma – Squamous cell carcinoma – Adenocarcinoma – Malignant Warthin’s tumor
  • 14.
  • 15.
  • 16. Neurogenic tumor • Second most common primary parapharyngeal tumor – Schwannoma • Vagus nerve • Cervical sympathetic chain – Paraganglioma • Vagal paraganglioma • Carotid body tumors – Neurofibroma – Malignant neurogenic tumor
  • 17. Clinical presentation • Clinical detection is difficult • Tumor size 2.5 to 3.0 cm to be detected clinically • Asymptomatic mass – Mild bulging of soft palate or tonsillar region – Palpable mass at angle of mandible
  • 18. Clinical symptoms • Prestyloid – Serous otitis media – Voice change – Nasal obstruction – Dyspnea • Poststyloid – Compress CN 9th , 10th , 11th , 12th or sympathetic chain – Hoarseness, dysphagia, dysarthria, Horner’s syndrome • Cranial nerve paralysis, pain, trismus suggest malignancy
  • 19. CT scan • Locates tumor to prestyloid vs poststyloid – Prestyloid tumor displace carotid artery posteriorly – Poststyloid tumor displace carotid artery anteriorly • Fat plane between mass and parotid • Enhancement of lesion – Schwannoma, paraganglioma, hemangioma, hemangiopericytoma, aneurysm • Bone erosion due to malignancy • Limited soft tissue detail
  • 20. MRI • Most useful study • Relationship of mass and carotid more easily seen than with CT • Characteristic appearances of tumor types on MRI allows preoperative Dx in 90-95% of patients
  • 21. MRI • Pleomorphic adenoma – Low intensity on T1 – High intensity on T2 – Displace carotid posteriorly
  • 22. MRI • Schwannoma – High intensity on T2 – Displace carotid anteriorly
  • 24. • Angiogram – Define vascular anatomy – Carotid occlusion test – Tumor embolization 1 day prior to surgery • FNA
  • 25. Transparotid approach • For deep lobe of parotid lesion • Superficial parotidectomy with facial nerve preservation • Retract facial nerve from the deep parotid lobe • Dissect posterior and inferior around mandible • Improve access by mandibulotomy
  • 26. Transcervical approach • For poststyloid tumor • Transverse incision at level of hyoid • Submandibular gland removed or retracted • Incision through the fascia deep to the submandibular space • Increase exposure by releasing digastric, stylohyoid, styloglossus from hyoid, cut stylomandibular ligament, mandibulotomy
  • 27. Cervical-parotid approach • Extend cervical incision up infront of ear • Identify facial nerve • Divide posterior belly digastric • Divide styloglossus, stylohyoid close to styloid process • Divide stylomandibular ligament • Can combine with mandibulotomy
  • 29. Indications • Can be used to remove majority of the parapharyngeal tumor – All deep lobe parotid tumors and extraparotid salivary tumors – Low grade malignant tumors of deep lobe of parotid – Many poststyloid tumors, including most neurogenic tumors and small paragangliomas
  • 30. Cervical-transpharyngeal • “Mandibular swing” • Midline lip splitting or visor flap • Mandibulotomy anteriorly, incise along floor of mouth to anterior tonsillar pillar • Identify hypoglossal nerve and lingual nerve • Divide styloglossus and stylopharyngeus muscle • Need tracheotomy
  • 32. indications • All vascular tumors that extend into the superior portion of the parapharyngeal space • Malignant tumor invaded skull base or vertebral body
  • 33. Conclusion • Rare tumor in an complex anatomical area • Subtle clinical presentation • Radiographic imaging is important • Prestyloid vs poststyloid space • Surgery is the main treatment • When not to operate