Neoplasms of Ear and Lateral Skull Base

Back To Search

Neoplasms of Ear and Lateral Skull Base

Otology/Audiology
Author Credentials 

Scott B. Shapiro, MD and Ravi N. Samy, MD, FACS
Otolaryngology-Head and Neck Surgery
University of Cincinnati
Cincinnati, OH

 

Module Summary

Due to the heterogeneous tissue and varied structures in the ear and lateral skull base, the neoplasms that may arise are similarly varied. They range from benign lesions (for which observation is acceptable) to aggressive malignancies which require wide resection, often with sacrifice of vascular or neural structures. In general, for benign neoplasms, optimal management depends on weighing the risks of tumor progression and against potential morbidity of resection or other treatment. Imaging is a critical part of diagnosis and surgical planning for both benign and malignant neoplasms.

Module Learning Objectives 
  1. List and describe the benign and malignant neoplasms that commonly affect the ear and lateral skull base.
  2. Perform the required workup of a patient with a diagnosed or suspected neoplasm of the ear and lateral skull base.
  3. Identify ear and lateral skull base neoplasms based on their imaging characteristics.
  4. Illustrate the anatomy and structures within the temporal bone and how involvement of these structures by neoplasms produces symptoms.
  5. Classify various neoplasms within their staging systems.
  6. Expound on the management strategies and various surgical approaches to ear and lateral skull base neoplasms.

 

Embryology

Learning Objectives 
  1. Describe the origin and development of the temporal bone and structures within the ear and lateral skull base.
References 
  1. Eliomaria C, Mattioli F, Alicandri-Ciufelli M, et al. Ear Emrbyology. In: Presutti L and Marchioni D, editors. Endoscopic Ear Surgery. New York, NY: Thieme; 2015. p31-37. 

Anatomy

Learning Objectives 
  1. Illustrate the anatomic relations of and structures within the temporal bone.
  2. Recognize the pathways by which malignancies spread into or out of the temporal bone.
  • Fissures of Santorini
  • Eustachian tube
  • Foramen of Huskhe
  • Stylomastoid foramen
  • Jugular foramen
  • Carotid canal
  • Petrotympanic fissure

 

References 
  1. Ryzenman JM, Kumar A. Anatomy and Embryology of the Ear. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e30-e99.
  2. Salzman KL and Robson CD. Temporal Bone Overview. In: Koch BL, Hamilton BE, Hudgins PA, et al., editors. Diagnostic Imaging Head and Neck. 3rd ed. Philadelphia, PA: Elsevier; 2017. p. 998-1003.
  3. Harnsberger HR. CPA-IAC Overview. In: Koch BL, Hamilton BE, Hudgins PA, et al., editors. Diagnostic Imaging Head and Neck. 3rd ed. Philadelphia, PA: Elsevier; 2017. p. 1198-1201.

Pathogenesis

Learning Objectives 
  1. Recognize that primary squamous cell carcinoma can arise from the external auditory canal skin.
  2. Describe what is known about how two commonly encountered skull base neoplasms develop.
  • Vestibular schwannoma
  • Paraganglioma
    • Recognize that paragangliomas of the head and neck may be named according to their location (glomus tympanicum, jugulare, vagale, etc.) but represent a similar disease process arising in different locations
References 
  1. Heubi CH, Pensak ML Malignant Tumors of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1186-e1196.
  2. Neff BA, Welling DB, Akhmametyeva E, Chang LS. The molecular biology of vestibular schwannomas: dissecting the pathogenic process at the molecular level. Otol Neurotol 2006;27:197–208. [EBM level 5]
  3. Williams MD. Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics. Head Neck Pathol. 2017;11(3):278-287. [EBM level 5]

Basic Science

Learning Objectives 
  1. Summarize the molecular and genetic biology of head and neck paraganglioma, vestibular schwannoma, and squamous cell carcinoma of the temporal bone.
References 
  1. Dahia PL. Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity. Nat Rev Cancer (2014) 14:108–19.  [EBM Level 5]
  2. Neff BA, Welling DB, Akhmametyeva E, Chang LS. The molecular biology of vestibular schwannomas: dissecting the pathogenic process at the molecular level. Otol Neurotol 2006;27:197–208. [EBM level 5]
  3. Carlson M, Smadbeck JB, Link MJ, et al. Next Generation Sequencing of Sporadic Vestibular Schwannoma. Otol Neurotol 2018;39(9):e860-e871. [EBM Level 4]
  4. Allanson BM, Low TH, Clark JR, et al. Squamous Cell Carcinoma of the External Auditory Canal and Temporal Bone: An Update. Head NeckPathol. 2018;12(3):407-418.

Incidence

Learning Objectives 
  1. Recognize that primary malignancy arising from tissue within the temporal bone is relatively uncommon; malignant lesions in this region more commonly occur via spread from adjacent skin or parotid tissue.
  2. List the types of neoplasms that can rise from structures in the ear and skull base.
  • Malignant neoplasms
    • Squamous cell carcinoma, represents 85% of primary malignancies of temporal bone, (though most malignancies are a result of secondary spread from adjacent structures)
    • Basal cell carcinoma
    • Melanoma
    • Rhabdomyosarcoma
    • Ceruminous gland tumor
    • Malignancies of glandular tissue (e.g. adenoid cystic, adenocarcinoma)
    • Malignancies of central nervous system tissue (e.g. astrocytoma, neuroblastoma)
    • Metastatic lesions (e.g. breast, lung, renal, thyroid)
  • Locally-aggressive benign neoplasms
    • Chondroblastoma
    • Hemanigiopericytoma
    • Plasmacytoma
    • Hemangioendothelioma
    • High-grade meningioma
    • Endolymphatic sac tumor
  • Benign neoplasms
    • Schwanomma
    • Paraganglioma
    • Low-grade meningioma
    • Osteoma
    • Lipoma
    • Adenoma
    • Hemangioma
    • Chordoma

 

 

References 
  1. Heubi CH, Pensak ML. Malignant Tumors of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1186-e1210.
  2. Santa Maria PL, Lustig LR, Jackler RK. Benign Neoplasms of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1062-e1144.
  3. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.28-30.
  4. Arriaga MA and Brackmann DE. Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.484-501.

Genetics

Learning Objectives 
  1. Describe the syndromes and genetic basis that account for a subset of paragangliomas and vestibular schwannomas.
References 
  1. Asthagiri AR, Parry DM, Butman JA, et al. Neurofibromatosis type 2. Lancet. 2009;373(9679):1974–1986. [EBM level 5]
  2. Chetty, R. Familial paraganglioma syndromes. J Clin Pathol. 2010;63:488‐491. [EBM level 5]

 

Patient Evaluation

Learning Objectives 
  1. Recognize that presenting symptoms of ear and lateral skull base neoplasms are variable and often related to which anatomic structures within the temporal bone are involved by an individual neoplasm.
  • Recognize facial nerve weakness, positive fistula test, other cranial neuropathies
  • Symptoms can mimic chronic otitis media or externa
  1. Recognize that imaging should be obtained before biopsy is performed of masses in the middle ear due to the risk of injuring the facial nerve, temporal lobe, and great vessels (i.e., internal carotid artery or jugular bulb).
  2. Recognize that CT and/or MR imaging is an important part of diagnosis and defining the extent of disease.
  3. Obtain the appropriate laboratory tests and imaging studies to rule out rare cases of hormone-secreting tumors or multicentricity during work-up of a paraganglioma. 
  4. Recognize that audiometric testing is required during work-up of a patient with an ear or skull base neoplasm.
  5. Recognize that angiography with balloon-occlusion testing should be performed when sacrifice of the carotid artery is considered during surgery.

 

References 
  1. Heubi CH, Pensak ML. Malignant Tumors of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1186-e1210.
  2. Santa Maria PL, Lustig LR, Jackler RK. Benign Neoplasms of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1062-e1144
  3. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.28-30.
  4. Arriaga MA and Brackmann DE. Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.484-501.

Measurement of Functional Status

Learning Objectives 
  1. Utilize commonly used metrics of functional status in determining the optimal management strategy and assessing outcomes for patients with ear and skull base tumors. 
  • Hearing status is often reported in terms of the American Academy of Otolaryngology-Head and Neck Surgery Hearing Classification Status
  • Facial nerve function is often described in terms of the House-Brackmann grading scale
  • The general health well-being may be quantified in terms of a performance status or scale (e.g. Karnofsky performance status)

 

References 
  1. Committee on Hearing and Equilibrium Guidelines for the Evaluation of Hearing Preservation in Acoustic Neuroma (Vestibular Schwannoma): Committee on Hearing and Equilibrium. Otolaryngology–Head and Neck Surgery, 1995;113(3), 179–180.
  2. House JW, Brackmann DE 1985. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93: 146–147.
  3. Karnofsky DA, Abelmann WH, Craver LF, et al. The Use of the Nitrogen Mustards in the Palliative Treatment of Carcinoma – with Particular Reference to Bronchogenic Carcinoma. Cancer. 1948;1(4):634-56.

Imaging

Learning Objectives 
  1. Differentiate commonly encountered ear and lateral skull base neoplasms by their CT and MRI characteristics.
  2. Recognize the role of CT and MRI in diagnosis and surgical planning and that they are often complementary, providing osseous and soft tissue detail, respectively.
References 
  1. Salzaman KL, Robson CD, Harnsberger HR, et al. Temporal Bone. In: Koch BL, Hamilton BE, Hudgins PA, et al, editors. Diagnostic Imaging Head and Neck. 3rd ed. Philadelphia, PA: Elsevier; 2017. p. 998-1194.
  2. Harnsbeger HR and Robson CD. CPA-IAC. In: Koch BL, Hamilton BE, Hudgins PA, et al, editors. Diagnostic Imaging Head and Neck. 3rd ed. Philadelphia, PA: Elsevier; 2017. p. 1198-1244.

Pathology

Learning Objectives 
  1. Recognize characteristic histologic appearance of benign lesions such as vestibular schwannoma and paraganglioma.
  2. Recognize characteristic histologic appearance of malignant lesions (most commonly squamous cell carcinoma).

 

References 
  1. Wippold FJ, Lubner M, Perrin RJ, et al. Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns. AJNR Am J Neuroradiol. 2007;28:1633–38. [EBM level 5]
  2. Tischler AS and deKrijger RR. 15 Years of Paraganglioma: Pathology of pheochromocytoma and paraganglioma. Endocr Relat Cancer. 2015;22:T123–T133. [EBM level 5]
  3. Williams MD. Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics. Head Neck Pathol. 2017;11(3):278-287. [EBM level 5]
  4. Weedon D. Squamous cell carcinoma. Skin pathology. 3rd ed. Elseiver, Philadelphia, PA. 2010; p.691-700.

 

Treatment

Learning Objectives 
  1. Recognize that some ear and lateral skull base neoplasms, such as paraganglioma and vestibular schwannoma, for which a diagnosis can be made based on imaging, do not always require treatment when diagnosed. Observation with serial imaging, clinical, and audiologic testing can be a valid management strategy. Often, the demonstration of radiologic growth of these neoplasms plays a role in determining treatment.
  2. Recognize that in syndromic or familial variants, vestibular schwannomas or paragangliomas may be bilateral, and this may affect treatment strategy.
  3. Recognize that pre-operative embolization may be used if significant intra-operative hemorrhage is expected during surgical resection of temporal bone neoplasms, particularly with paragangliomas.
  4. Compare and contrast various management strategies for an individual neoplasm. 

Vestibular schwannoma

  • Optimal treatment of vestibular schwannoma depends on tumor and patient characteristics. The goal is to preserve function. Management may include surgical resection, observation, and/or stereotactic radiation therapy.

Paraganglioma

  • Optimal treatment of paragangliomas of the temporal bone is controversial due to significant differences in their growth rate, location, symptoms, and clinical characteristics. 
  • Management may include observation, surgical resection, radiation (often stereotactic), and/or embolization with the goal of preserving function.

Facial nerve tumors

  • Explain that the management options for facial nerve tumors include observation, surgical resection with nerve grafting, or surgical decompression. In rare cases, radiation has been used to control tumor growth, although long-term data is poor. Optimal management is controversial, particularly the timing of surgery.
  • Surgical resection with nerve grafting has modest facial nerve outcomes (maximum House-Brackmann grade III or IV), but patients have better functional outcomes after surgery with earlier treatment.
  • Observation may be an option for slow growing tumors or those that appear stable in size in serial imaging (or in the elderly or infirm).
  • Surgical decompression rather than resection may be an option to achieve normal or near-normal facial nerve function (House- Brackmann grade I or II).
  1. Recognize that in general, most other solid tumors of the temporal bone are treated with surgical removal to prevent complications of mass effect/growth on structures within the temporal bone and to obtain a definitive pathologic diagnosis.

Squamous cell carcinoma

  • Explain that squamous cell carcinoma of the temporal bone is generally treated with surgical resection and adjuvant radiation.

 

References 
  1. Heubi CH, Pensak ML. Malignant Tumors of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1186-e1210.
  2. Santa Maria PL, Lustig LR, Jackler RK. Benign Neoplasms of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1062-e1144.
  3. Shelton C and Warren FM. Facial Nerve Tumors. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 314-322.
  4. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 28-30.
  5. Wackym PA, Kee AY, Schroeder J. Stereotactic Radisurgery of Skull Base Tumors. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 690-706.

Medical Therapies

Learning Objectives 
  1. Recognize that for squamous cell carcinoma of the temporal bone, pre-operative chemoradiation or definitive chemoradiation may achieve comparable disease-free survival compared to surgery with adjuvant radiation, although data on long-term efficacy is lacking. Most chemotherapy protocols have utilized a combination of chemotherapeutics such as cisplatin or carboplatin, 5-fluorocuracil, and/or docetaxel. There may also be a role for neo-adjuvant chemotherapy for unresectable tumors.
References 
  1. Takenaka Y, Cho H, Nakahara S, et al. Chemoradiation therapy for squamous cell carcinoma of the external auditory canal: A meta‐analysis. Head Neck. 2015;37:1073-1080. [EBM Level 1]

Pharmacology

Learning Objectives 
  1. Recognize that paragangliomas of the head and neck infrequently secrete catecholamines (most often norepinephrine but sometimes epinephrine or dopamine). This most commonly manifests clinically as a hypertension with headaches, palpitations, and diaphoresis.
  • Urine or plasma testing for the catecholamine metabolites metanephrine and normetanephine will identify patients with secreting tumors.
  • Functional imaging utilizing radiolabeled octreotide (111In-DTPA-pentetreotide)  as well as positron emission (PET) or single photon emission computed tomography (SPECT) can be useful for identifying head and neck paragangliomas or detecting multicentricity.
  • The hemodynamic effects of excess catecholamine requires special management peri-operatively beginning with: 1) alpha-adrenergic blockade 1-2 weeks prior to surgery 2) intra-operative anti-hypertensive management utilizing multiple agents during tumor dissection, and 3) withdrawal of vasodilators after tumor removal and other adjunctive techniques for management of post-operative hypotension.

 

References 
  1. Colen TY, Mihm FG, Mason TP, Roberson JB. Catecholamine-secreting paragangliomas: recent progress in diagnosis and perioperative management. Skull Base. 2009;19(6):377–385. [EBM level 5]
  2. Timmers HJ, Taieb D, Pacak K. Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma. Horm Metab Res. 2012;44:367–372. [EBM level 5]

Surgical Therapies

Learning Objectives 
  1. Recognize that the surgical approach required for resection of ear and skull base neoplasms is determined based on the nature of the tumor, its size and location, and status of hearing and cranial nerves. Often, sacrifice of hearing and vestibular function is required; other cranial nerves tend to be preserved for resection of benign lesions. Sometimes, combined approaches are necessary, including the utilization of endoscopic or facial disarticulation approaches.
  2. Compare and contrast various approaches to the skull base for an individual neoplasm:
  • Malignancy of the external auditory canal
    • Recognize that the medial extent of tumor defines the extent of surgical resection and often must be combined with parotidectomy and neck dissection
      • Partial/lateral temporal bone resection
      • Subtotal temporal bone resection
      • Total temporal bone resection
  • Approaches to lateral skull base tumors
    • Trans otic-capsule approaches sacrifice hearing and vestibular function
      • Translabyrinthine
      • Transcochlear
      • Transotic
    • Posterior approaches may preserving hearing by exposing the skull base via an approach posterior to the otic capsule
      • Retrosigmoid
      • Retrolabyrinthine
    • Superior approaches access petrous apex and internal auditory canal from above
      • Middle fossa (standard or extended middle cranial fossa)
    • Anterior approaches allow access to the infratemporal fossa and middle skull base
      • Transfacial approach
      • Fisch infra-temporal fossa approaches
    • Paraganglioma, other neoplasms of the middle ear and jugular foramen
      • Transcanal approach
      • Mastoidectomy with extended facial recess
      • Mastoid-neck approach
        • May utilize facial nerve re-routing
      • Fisch infra-temporal fossa approaches

 

References 
  1. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 28-41.
  2. Arriaga MA. Overview of Transtemporal Skull Base Surgery In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 458-460.
  3. Goddard JC, McRacken TR, House JW. Translabyrinthine Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.520-530.
  4. Kuthubutheen J and Chen JM. Transotic Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.548-556.
  5. Goddard JC and McRackan TR. Transcochlear Approach to Cerebellopontine Angle Lesions. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.557-566.
  6. Santa Maria PL, Jackler RK, Sim DW. Retrosigmoid Approach to Tumors of the Cerebellopontine Angle. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.531-547.
  7. Shelton C, Brackmann DE, House WF. Middle Fossa Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.512-519.
  8. Friedman RA, Aaron KA. Extended Middle Fossa Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.567-572.
  9. Arriaga MA and Brackmann DE. Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.484-501.
  10. Chan J, Carrau RL, Prevedello DM, et al. Anterior and Subtemporal Approaches to the Infratemporal Fossa. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.573-588.

Rehabilitation

Learning Objectives 
  1. Choose appropriate strategies to restore function after facial nerve injury:
  • Eye care
  • Nerve graft
  • Facial reanimation
  1. Recognize that vestibular rehabilitation is important for patients with treatment or tumor-related vestibular weakness.
  2. Choose an appropriate strategy for optimizing auditory performance after treatment or tumor-related hearing loss:
  • Hearing aid
  • Osseointegrated implant
  • Cochlear implant
  • Auditory brainstem implant
  1. Discuss techniques to improve function and reduce morbidity after treatment or tumor related-lower cranial nerve deficits.
  • Mandibular division of trigeminal nerve
  • Abducens nerve (postoperative ophthalmology consultation)
  • Glossopharyngeal nerve (postoperative speech and swallow therapy)
  • Vagus nerve
    • Vocal ford medialization
  • Spinal accessory nerve
  • Hypoglossal nerve

 

References 
  1. Hetzler LT and Jackson NM. Care of the Eye in Facial Paralysis and Facial Reanimation Techniques. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.653-669.
  2. Wilkinson EP, Luxford WM, Slattery III WH, et al. Hypoglossal Facial Anastamosis. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.670-677.
  3. Netterville JL and Sullivan CA. Rehabilitation of Lower Cranial Nerve Deficits after Neurotologic Skull Base Surgery. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.520-530.
  4. Gurgel RK and Tjellstrom. Bone-Anchored Cochlea Stimulator. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.350-361.
  5. Sanna M, Free R, Merkus P, et al. Cochlear Implantation. Surgery for Cochlear and Other Auditory Implants. New York, NY; Thieme; 2016. p.66-73.
  6. Sanna M, Free R, Merkus P, et al. Auditory Brainstem Implantation. Surgery for Cochlear and Other Auditory Implants. New York, NY; Thieme; 2016. p.138-148. 

Staging

Learning Objectives 
  1. Recognize that for squamous cell carcinoma of the temporal bone, the American Joint Committee on Cancer (AJCC) applies the same staging system used for cutaneous malignancies of other sites. However, due to the unique anatomy of the temporal bone, other staging systems have been proposed and are in use.
  • Moody Modification of Arriaga - University of Pittsburgh Staging System
  • University of Cincinnati Grading System
  1. Recognize that several staging systems have been proposed and used for vestibular schwannoma, mainly based on tumor size and location (e.g., Koos).
  2. Classify paragangliomas according to their location:
  • Carotid body
  • Glomus jugulare
  • Glomus tympanicum
  • Glomus vagale
  1. Classify paragangliomas of the temporal bone with commonly use staging systems:
  • Modified De La Cruz
  • Fisch
  • Glasscock-Jackson

 

References 
  1. Wu H, Zhang L, Han D, et al. Summary and consensus in 7th International Conference on acoustic neuroma: An update for the management of sporadic acoustic neuromas. World J Otorhinolaryngol Head Neck Surg. 2016;2:234. [EBM level 5]
  2. Arriaga MA and Brackmann DE. Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.484-501.
  3. Heubi CH, Pensak ML. Malignant Tumors of the Temporal Bone. In: Pensak ML, Choo DI, editors. Clinical Otology. 4th ed. New York, NY: Thieme; 2015. (e-book) eISBN 978-1-60406-913-6 p. e1186-e1210.
  4. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 28-41.

Case Studies

  1. 68 year-old man with unilateral conductive hearing loss and a chronically draining ear is found to have a soft tissue mass involving the external auditory canal. Evaluation of the CT scan demonstrated that the lesion extends through the tympanic membrane into the mesotympanum. Biopsy of the external auditory canal tissue shows inflammatory tissue.
  • Repeat biopsy should be performed as malignancy of the external auditory is often secondarily infected, and initial biopsies may only show inflammatory changes. This may necessitate a transmastoid approach with posterior tympanotomy and intraoperative frozen section.
  • If repeat biopsy shows squamous cell carcinoma after final pathologic evaluation, the patient will require partial/lateral temporal bone resection. This includes a complete mastoidectomy and extended facial recess approach, separation of incudo-stapedial joint and ossicular attachments, drilling anteriorly to the glenoid fossa (at the protympanum) with removal of all structures lateral to the cochlea, facial nerve, and geniculate ganglion.
  1. 31 year-old male with unilateral conductive hearing loss has a violet hue behind the tympanic membrane. He has no other cranial nerve deficits.
  • Do not biopsy. Biopsy of middle ear masses without imaging can result in injury to great vessels, facial nerve, or bleeding from vascular tumors. Imaging should be obtained first.

CT shows an enhancing soft tissue density involving the entirety of the middle ear and extending posteriorly into the mastoid air cells and anteriorly into the jugular bulb. The carotid artery is not involved, and there is no intracranial involvement. MRI shows it to be a T1 hypointense, T2 hyperintense lesion with “salt and pepper” flow voids on gadolinium-enhanced imaging.

  • These are imaging characteristics consistent with a paraganglioma. Treatment is controversial but patient dependent and may include observation with serial imaging and audiologic testing, stereotactic radiation, and/or surgical resection. Of note, complete surgical resection of paragangliomas that involve the jugular bulb, carotid artery, or dura may result in lower cranial nerve or vascular injury; this is an additional risk compared to tumors confined to the tympanic and tympanomastoid spaces. Also, facial nerve transposition or a fallopian bridge technique may be necessary for these tumors.
  • If surgery is planned, angiography is performed to assess risk of carotid injury and subsequent neurologic injury as well as the vessels feeding the lesion.  Pre-operative embolization is to reduce intra-operative bleeding. A carotid balloon occlusion test is also performed.  Surgical resection would include a complete mastoidectomy with an extended facial recess approach, extending the operative field into the neck, control and ligation of the jugular vein, and resection of the tumor in continuity with the jugular bulb. Facial nerve re-routing may provide additional exposure, but a fallopian bridge technique may be utilized.  Ossicular reconstruction may be performed as well, although postoperative osseointegrated device placement may be utilized instead.

 

 

Complications

Learning Objectives 
  1. Describe the risks and possible complications of surgical approaches and other treatment modalities for temporal bone neoplasms as well as the management options if these complications occur:
  • Sensorineural, conductive, or mixed hearing loss
  • Vestibular weakness/injury
  • Vascular injury
  • CSF leak
  • Hydrocephalus
  • Meningitis
  • Facial nerve injury
  • Lower cranial nerve injury
  • Thromboembolic (e.g., deep venous thrombosis and pulmonary embolism)

 

References 
  1. Wilkinson EP, Brackmann DE, Lupo JE. Management of Postoperative Cerebrospinal Fluid Leak. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. 646-652.
  2. Arriaga MA and Yu-Lan MY. Malignancies of the Temporal Bone: Limited and Radical Temporal Bone Resection. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p. 28-41.
  3. Arriaga MA and Brackmann DE. Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.484-501.
  4. Shelton C, Brackmann DE, House WF. Middle Fossa Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.512-519.
  5. Santa Maria PL, Jackler RK, Sim DW. Retrosigmoid Approach to Tumors of the Cerebellopontine Angle. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.531-547.
  6. Goddard JC, McRacken TR, House JW. Translabyrinthine Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.520-530.
  7. Kuthubutheen J and Chen JM. Transotic Approach. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.548-556.
  8. Goddard JC and McRackan TR. Transcochlear Approach to Cerebellopontine Angle Lesions. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.557-566.
  9. Chan J, Carrau RL, Prevedello DM, et al. Anterior and Subtemporal Approaches to the Infratemporal Fossa. In: Brackmann DE, Shelton C, Arriaga MA, editors. Otologic Surgery. 4th ed. Philadelphia, PA; Elsevier; 2016. p.573-588.

Review

Review Questions 
  1. What is the preferred treatment for squamous cell carcinoma involving the pre-auricular skin, parotid gland, external auditory canal, and tympanic membrane? 
  2. A 77 year-old man with moderate-severe mixed unilateral hearing loss and mild dysphagia is found to have a mass involving the middle ear, mastoid, and jugular bulb. Imaging findings are consistent with a paraganglioma. What are the options for management and the risks and potential benefits of each? What other studies or information might you obtain to assist in decision-making?
  3. A 45 year-old man who underwent an MRI for headaches is found to have a mass along the jugular bulb with characteristics consistent with a paraganglioma. He has had difficult-to-control hypertension. What additional testing should be obtain prior to surgery? If the tumor secretes catecholamines, how should the patient be management peri-operatively?
  4. During the surgical approach to a meningioma of the cerebello-pontine angle, the sigmoid sinus is injured and significant bleeding is occurring. What are the immediate and delayed steps in managing this complication?
Resources 

Learner must Sign In to access AAO-HNSF education activities.

  1. Annual Meeting Webcast (AMW):