Tumours of the Ear and Skin

11 Tumours of the Ear and Skin

11.1 Incidence and Site

• Tumours of the ear are rare: ~70% pinna, 20% external auditory canal (EAC), 10% middle ear (ME)

11.2 Pinna

• Squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) most common (respective incidences vary country to country) (Figs. 11.1 and 11.2a)

• Rx: primary resection with appropriate margins; minor superficial lesions with no cartilage invasion or fixation may have radiotherapy (RT) alone

• Benign include nevus, epithelioma adenoides cysticum, molluscum contagiosum

11.3 EAC

• SCC (Fig. 11.2b)

figure 90% of malignant tumours, slow-growing, spread via cartilage portion and later into ME, parotid, sternocleidomastoid muscle

figure 20% lymph node (LN) spread (posteriorly to mastoid nodes, inferiorly to level III, anteriorly to preauricular)

figure Associated with chronic inflammation, RT

figure Rx: consider lateral temporal bone resection, ± neck dissection; post-operative RT

figure Prognosis very poor if node positive

• Adenocarcinoma: very aggressive (usually fatal within 4 years), often presenting with VII n palsy

• Adenoid cystic: most common “ceruminous” tumour; long natural history, painful

• Also adenoma, mucoepidermoid carcinoma; malignant melanoma very rare, as is BCC of EAC

11.4 Middle Ear

• Choristoma = normal tissue in abnormal site (e.g., salivary gland in ME): rare; may be attached to VII n

• Adenoma (benign): cause unilateral progressive conductive hearing loss (HL), TM intact in 75% cases; 2nd most common after glomus tumours, but still rare

• Adenocarcinoma: very rare; cause deafness, pain, VII n palsy

• SCC:

figure Most common epithelial ME malignancy, but rare

figure Association with chronic otitis media in 85%, irradiation

figure Extensive bony destruction, most easily through tegmen to middle cranial fossa (dura is a relatively strong barrier); intracranial invasion along VII n and VIII n in internal auditory canal; IX n and XII n involvement shows skull base involved; can also extend to temporomandibular joint, parotid, via eustachian tube to nasopharynx

figure LN involvement in 10 to 15% at presentation, rare distant metastasis; death by cachexia secondary to pain because cranial nerve (CN) involvement, though sometimes invasion of meninges leads to intracranial complications and erosion of jugular bulb or carotid is possible

figure Rx: surgery (petrosectomy) + RT; palliative

figure 5-year survival ~35% (better for EAC than ME); prognosis very poor if node positive

• Other benign tumours:

figure Osteoma

figure VII n schwannoma (usually at geniculate ganglion)

figure Hemangioma, neurogenic

• Other malignant tumours:

figure Sarcoma

figure Multiple myeloma

figure Malignant xanthoma

figure Rhabdomyosarcoma most common in children; may require surgery ± chemoradiotherapy

figure Also direct extension (e.g., from nasopharynx, external ear, parotid, meningioma) and distant metastases (kidney, lung, prostate, thyroid, breast)

11.5 Glomus Tumours

• Pathology:

figure Correct (WHO) term is paragangliomas; aka chemodectomas; arise from paraganglionic tissue (chemoreceptor cells)

figure Common embryological origin (neural crest) and association with neural structures

figure 90% adrenal (phaeochromocytomas); 10% nonadrenal (85% of which are abdominal, only 3% H&N); subdivided into abdominal and extra-abdominal

figure H&N paragangliomas associated with parasympathetic system

• Epidemiology:

figure Relative incidence: carotid > jugulotympanic > vagal

figure 5th decade, F>M, may be multiple (show multicentricity); ~10%, autosomal dominant; usually benign and slow-growing but ~5% malignant; rarely (<1%) secrete neuropeptides (e.g., serotonin, vasoactive intestinal peptide)

Jul 4, 2016 | Posted by in OTOLARYNGOLOGY | Comments Off on Tumours of the Ear and Skin

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