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Tumours of Temporal Bone

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Tumours of the middle ear & temporal bone are rare, with an estimated incidence of 1 in 5,000–20,000 otological patients or 6 cases per million in the general population. The disease is slightly more common in females and affects the age group of 40-60 years. Squamous cell carcinoma is the most common histologic type, accounting for over 80% of cases, followed by basal cell carcinoma. Despite advances in surgical techniques and radiotherapy, their prognosis remains poor, highlighting the importance of early detection.

Risk Factors for Temporal Bone Tumours

  1. Genetic Factors:
    • Mutations in the NF2 gene (chromosome 22) are linked to certain temporal bone tumours.
    • Hereditary paragangliomas are associated with genes on chromosome 11q23.
    • Von Hippel-Lindau (VHL) disease (chromosome 3p25) is linked to endolymphatic sac tumours (ELSTs).
  2. Radiation Exposure: A history of radiotherapy for head and neck conditions increases the risk of temporal bone malignancy, with a latency period of 5–30 years. The radiation-induced tumours, such as squamous cell carcinoma and osteosarcoma are aggressive and metastasize early with poor survival rates.
  3. Chronic Suppurative Otitis Media (CSOM) and Papillomatosis (Inverted papilloma):
    • CSOM is associated with squamous carcinoma of the temporal bone, though the exact mechanism is unclear.
    • Human papillomavirus (HPV) types 16/18 and 11 have also been reported to be additional risk factors.

Clinical features:  Temporal bone tumours often present with non-specific symptoms such as

  1. Otalgia,
  2. Chronic foul-smelling blood-stained discharge,
  3. Hearing loss,
  4. Vertigo
  5. Presence of granulations or polyps on examination

All above symptoms mimic benign conditions like otitis externa or chronic suppurative otitis media (CSOM) and therefore complicate its early detection. Given the rarity of malignancy, distinguishing between a CSOM and a malignant tumour, the diagnosis is often made in a late stage when the patient presents with facial palsy or bleeding from the ear. However, the pain associated with these tumours is typically more severe than that caused by non-neoplastic conditions.

Spread of Tumour.  The tumour of the external auditory canal spread anteriorly into the parotid gland and temporomandibular joint. The tumour of the middle ear destroys middle structures first and can spread anteriorly in the Eustachian tube & nasopharynx, superiorly in the middle cranial fossa, medially in the internal ear and inferiorly to involve the jugular foramen and lower cranial nerves. It may spread in a petrous pyramid towards its apex. Thus, the clinical features of temporal bone tumours may include:

  • Trismus
  • Hemifacial spasm or progressive facial weakness
  • Lower cranial nerve palsies (IX, X, XI and XII)
  • Headache and localized pain
  • Cervical metastases.

Diagnostic Imaging:

  • Biopsy is essential for definitive diagnosis.
  • CT scans reveal bone erosion, while MRI with gadolinium enhancement shows soft tissue infiltration. Both help assess the tumour stage, including dural involvement and infratemporal fossa spread. Angiography is also useful in the assessment of disease.

Staging: No specific staging system exists for temporal bone tumours under AJCC or IUCC. 

Treatment: A combination of surgery and radiotherapy gives better results.

Classification of Temporal Bone Tumours: Tumours of the temporal bone are classified based on their origin:

  1. Cutaneous neoplasms: Squamous cell carcinoma, basal cell carcinoma, malignant melanoma.
  2. Glandular neoplasms: Ceruminous adenoma, pleomorphic adenoma, adenoid cystic carcinoma, middle ear adenoma, endolymphatic sac tumours.
  3. Vascular/haematological: Haemangioma, haemangiopericytoma, lymphoma, Langerhans cell histiocytosis
  4. Paraganglioma: Glomus tympanicum, glomus jugulare, glomus vagale. (Detailed Glomus Tumour link: https://www.entlecture.com/glomus-tumour/ )
  5. Bone tumours: Osteoma, rhabdomyosarcoma, chondrosarcoma, Ewing sarcoma, chordoma.
  6. Neural tumours: Schwannomas, meningiomas.
  7. Developmental and congenital tumours: Chordoma, dermoid, teratoma.

 

Cutaneous Neoplasms:

  1. Squamous cell carcinoma: Squamous cell carcinoma (SCC) is the most common malignant tumour of the temporal bone, with an incidence of fewer than 6 cases per million annually, primarily affecting males in their seventh decade. Early-stage tumours have favourable outcomes (80–100% survival), while advanced disease (T4) has a poorer prognosis (~40% survival). Treatment involves surgical management: lateral temporal bone resection for tumours lateral to the tympanic membrane, extended temporal bone resection for advanced disease (often requiring facial nerve sacrifice and dura/brain resection), and neck dissection for node-positive disease. Reconstruction uses free tissue transfer (e.g., anterolateral thigh flap), and adjuvant radiotherapy (60Gy) improves survival, especially for advanced cases.

Glandular Neoplasms:

  1. Glandular tumours of the external auditory canal:
    1. Ceruminous adenoma: A benign tumour of ceruminous glands, treated with wide excision as local recurrence is common; radiotherapy is ineffective.
    2. Pleomorphic adenoma: A benign tumour that requires careful wide excision to avoid local seeding of the local tissues by spillage of the tumour during excision.
    3. Adenoid cystic carcinoma: It is an aggressive malignant tumour, with major local tissue destruction, & perineural and vascular invasion. Managed with aggressive surgical resection and radiotherapy, though prognosis remains poor.
    4. Ceruminous adenocarcinoma: It is a rare malignant tumour, treated with surgery with post-operative radiotherapy.

  2. Middle ear adenoma: A benign tumour of middle ear mucosa, confined to the middle ear cleft with no bony erosion or metastasis, treated with local excision (tympanotomy or mastoid approach); no role of radiotherapy; excellent prognosis.
  1. Endolymphatic sac tumours (ELSTs): Endolymphatic sac tumours (ELSTs) are rare, slow-growing but locally aggressive neoplasms often associated with Von Hippel-Lindau (VHL) disease. The most prominent clinical feature is progressive unilateral sensorineural hearing loss, accompanied by tinnitus and vertigo. ELSTs are highly vascular and exhibit aggressive bone invasion without metastatic spread; treated with surgical resection; excellent prognosis for both survival and hearing preservation; radiotherapy is reserved only for palliative cases. The tumour may spread into the cerebellopontine angle and posterior fossa. 

Miscellaneous Tumours:

  1. Inverted papilloma: Rare, with potential for malignant transformation. Treated with surgery and radiotherapy if carcinoma is present.
  2. Haemangioma: Benign, often regresses spontaneously; surgery is reserved for symptomatic cases.
  3. Haemangiopericytoma: Rare and unpredictable; treated with wide excision and pre-operative embolization.
  4. Osteoma: Benign, often asymptomatic; surgery is considered if hearing loss occurs.
  5. Chordoma and chondrosarcoma: Aggressive tumours requiring multidisciplinary management, including surgery and proton beam radiotherapy. Prognosis is better for chondrosarcoma than chordoma.
  6. Secondary Tumours of the Temporal Bone: Secondary tumours may result from local spread (e.g., from the parotid or pharynx), distant metastases (e.g., breast, lung, kidney), or haematological malignancies. Symptoms include hearing loss, vertigo, and facial paralysis. Prognosis is poor, and management is individualized based on the primary tumour.

Temporal Bone Tumours in Childhood

  1. Rhabdomyosarcoma (RMS): Rhabdomyosarcoma (RMS) is a highly aggressive tumour originating from striated muscle tissue or pluripotential mesenchyme, with the potential for local spread and metastasis. Although rare, it is the most common soft tissue tumour in children under 12 years of age. In the head and neck region, RMS of the ear is the third most common site, following the nasopharynx and orbit. Common presentations include a mass, aural polyp, or ear discharge, with facial palsy often occurring early in the disease course.

    Diagnosis is confirmed through biopsy, as clinical and imaging findings alone are insufficient.  Management primarily involves chemotherapy and radiotherapy, with surgery playing a limited role. RMS has a poor prognosis, but treatment advancements have improved outcomes. Over time, survival rates have increased, with the 5-year survival rate now reaching 73%.

  2. Langerhans Cell Histiocytosis (LCH): It is characterized by the proliferation of histiocytes and can involve the temporal bone, often mimicking CSOM. Treatment depends on disease extent: unifocal disease has an excellent prognosis with local treatment, while disseminated disease is often fatal.

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Download full PDF Link: Tumours of Temporal Bone Best Lecture Notes Dr Rahul Bagla ENT Textbook

Reference Textbooks.

  • Scott-Brown, Textbook of Otorhinolaryngology-Head and Neck Surgery.
  • Glasscock-Shambaugh, Textbook of Surgery of the Ear.
  • P L Dhingra, Textbook of Diseases of Ear, Nose and Throat.
  • Hazarika P, Textbook of Ear Nose Throat And Head Neck Surgery Clinical Practical.
  • Mohan Bansal, Textbook of Diseases of Ear, Nose and Throat Head and Neck Surgery
  • Hans Behrbohm, Textbook of Ear, Nose, and Throat Diseases With Head and Neck Surgery.
  • Salah Mansour, Middle Ear Diseases – Advances in Diagnosis and Management.
  • Logan Turner, Textbook of Diseases of The Nose, Throat and Ear Head And Neck Surgery.
  • Rob and smith, Textbook of Operative surgery.
  • Anirban Biswas, Textbook of Clinical Audio-vestibulometry.
  • Arnold, U. Ganzer, Textbook of  Otorhinolaryngology, Head and Neck Surgery.

Author:

Dr. Rahul Bagla ENT Textbook

Dr. Rahul Bagla
MBBS (MAMC, Delhi) MS ENT (UCMS, Delhi)
Fellow Rhinoplasty & Facial Plastic Surgery.
Renowned Teaching Faculty
Mail: msrahulbagla@gmail.com
India

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Keywords: Rare temporal bone tumours, Squamous cell Carcinoma Temporal Bone, Endolymphatic sac tumour treatment, Glomus tympanicum surgery, Adenoid cystic carcinoma temporal bone, Ceruminous adenoma external ear, Middle ear adenoma symptoms, Radiation-induced temporal bone tumours, Von Hippel-Lindau disease and ELST, Temporal bone tumour diagnosis, Chondrosarcoma of the skull base, Chordoma temporal bone treatment, Haemangioma middle ear management, Inverted papilloma temporal bone, Rhabdomyosarcoma in children ear, Langerhans cell histiocytosis temporal bone, Chronic otitis media and temporal bone cancer, Temporal bone tumour staging systems, Skull base tumours and hearing loss, Rare ear tumours and facial palsy, Molecular genetics of temporal bone tumours

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