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Tumors of the Ear and Temporal Bone

Tumors of the Ear and Temporal Bone

Tumors of the Ear and Temporal Bone

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Ear Tumors 101 - Benign Beginnings

  • Classification by Nature & Site:
    • Nature: Benign (non-cancerous), Malignant (cancerous).
    • Site: External Ear, Middle Ear, Inner Ear, Temporal Bone.
  • Common Benign Tumors & Features:
    • Exostosis (Surfer's Ear):
      • Multiple, bilateral bony growths in External Auditory Canal (EAC).
      • Linked to cold water exposure.
    • Osteoma:
      • Solitary, unilateral, pedunculated bony tumor in EAC.
      • Often at bony-cartilaginous junction.
    • Ceruminous Adenoma/Pleomorphic Adenoma:
      • From ceruminous glands in outer EAC; slow-growing.
  • General Benign Tumor Traits:
    • Slow growth, well-circumscribed.
    • Non-invasive locally.

⭐ Exostoses are typically bilateral and multiple, often linked to cold water exposure.

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Glomus Goodness - Vascular Vibes

  • Origin: Paraganglionic cells (neural crest).
  • Types:
    • Glomus Tympanicum: Arises from Jacobson's nerve on cochlear promontory.
    • Glomus Jugulare: Arises from jugular foramen (CN IX, X, XI involvement).
  • Symptoms: Pulsatile tinnitus, conductive hearing loss (CHL).
    • 📌 Signs:
      • Brown's sign: Blanching of reddish mass behind TM on pneumatic otoscopy.
      • Phelps' sign (HRCT): Absence of bone between carotid canal & jugular fossa.
      • Aquino's sign: Blanching of tumor with carotid compression.

⭐ Glomus tympanicum typically presents as a reddish-blue mass behind an intact tympanic membrane.

  • Fisch Classification (Glomus Jugulare):
    • Type A: Middle ear. B: Tympanomastoid. C: Infralabyrinthine, extending to petrous apex. D: Intracranial extension.
  • Diagnosis:
    • HRCT: Bone erosion ('moth-eaten' appearance).
    • MRI + Gadolinium: 'Salt & pepper' appearance (flow voids & hemorrhage).
    • Angiography: Defines vascular supply. Otoscopy: Glomus tumor
  • Management: Surgery (pre-op embolization), Radiotherapy (Gamma Knife).

Malignant Mayhem - Canal Invaders

  • Squamous Cell Carcinoma (SCC): Most common (>80% temporal bone malignancies).
    • Locations: External Auditory Canal (EAC), Pinna, Middle Ear.
    • Risk Factors: Chronic Suppurative Otitis Media (CSOM), chronic otitis externa, radiation, HPV.
  • Other Malignancies:
    • Basal Cell Carcinoma (BCC): Pinna primarily.
    • Adenoid Cystic Carcinoma: Perineural spread common.
    • Rhabdomyosarcoma: Most common ear sarcoma in children.
  • Clinical Features:
    • Persistent, severe otalgia (out of proportion).
    • Chronic, blood-stained otorrhoea.
    • Hearing loss.
    • Facial palsy (poor prognostic sign).
    • Granulation tissue in EAC.
  • Staging: University of Pittsburgh Staging System (T1-T4 for SCC).

⭐ Facial nerve palsy in the context of chronic ear discharge and pain is highly suggestive of malignancy until proven otherwise.

Tumor Tactics - Dx & Tx Plan

  • Diagnosis (Dx):

    • History & Clinical Exam (Otoscopy, Cranial Nerves)
    • Audiometry: Type & degree of hearing loss
    • Imaging:
      • HRCT Temporal Bone: Bony erosion, extent
      • MRI: Soft tissue, perineural/intracranial spread
      • PET-CT: Staging/metastasis
    • Biopsy: Histological Dx (incisional/excisional)

      ⭐ Biopsy is mandatory for any suspicious lesion in the external auditory canal or middle ear to rule out malignancy.

  • Treatment (Tx) Plan:

    • Benign: Observation (e.g., small exostoses) or Surgical excision if symptomatic.
    • Malignant: Multimodal approach (MDT essential: ENT, NeuroSx, Onco[Rad/Med])
      • Surgery: Sleeve resection, Lateral Temporal Bone Resection (LTBR), Subtotal Temporal Bone Resection (STBR), Radical Temporal Bone Resection (RTBR) (stage-dependent)
      • Radiotherapy (RT): Adjuvant/Primary
      • Chemotherapy (CT): Palliative, Neoadjuvant, Concurrent with RT

CT and MRI of temporal bone tumor

High‑Yield Points - ⚡ Biggest Takeaways

  • SCC is the most common malignancy of the EAC and temporal bone.
  • Glomus tumors are most common in the middle ear, causing pulsatile tinnitus & Brown's sign.
  • Vestibular schwannoma: Most common CPA tumor; presents with unilateral SNHL.
  • Osteoma: Most common benign tumor of the EAC.
  • Progressive facial palsy with an ear mass suggests malignancy or facial nerve neuroma.
  • Rhabdomyosarcoma: Most common childhood middle ear malignancy.

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Practice Questions: Tumors of the Ear and Temporal Bone

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Which cancers can cause referred otalgia (referred pain in the ear)? Select the most comprehensive answer.

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Audible Bruit over the _____ maybe heard in glomus tumor

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Audible Bruit over the _____ maybe heard in glomus tumor

mastoid

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