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Pathology of Acquired Cholesteatoma

 

Dermatopathology Quiz Case 218

Diagnosis:  Cholesteatoma

 

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Since J. Cruveilhier described cholesteatoma as the "pearly" tumour of the middle ear in 1828, the pathogenesis of cholesteatoma remained controversial.

It is accepted that there are two distinct forms of middle ear cholesteatoma : Congenital and Acquired Cholesteatomas.

Four basic theories present the pathogenesis of acquired cholesteatoma:

- invagination of the tympanic membrane (retraction pocket cholesteatoma) ;

- basal cell proliferation ;

- epithelial in-growth through a perforation (the immigration theory) and

- squamous metaplasia of middle ear epithelium.

Acquired cholesteatomas:

These are associated with chronic otitis media leading to the accumulation of keratin debris in the middle ear or mastoid air cells.

There are inflammatory changes including prominent congestion and pockets of pus.

The lesion incites resorption of bone and can lead to deafness by destroying ossicles covered by stratified squamous epithelial tissue.

Squamous epithelium from external auditory canal penetrates the damaged tympanic membrane.

Pearly material of the cholesteatoma consists of dead, anucleate, keratin squames, the corneal layer of stratified squamous cell epithelium.

Age: presents over a wide age range

Site: Located in the upper and posterior part of the middle ear cleft.

Gross:  These are cystic white lesions of varying size with creamy or waxy granular material

Histological features:  These are lined by keratinizing squamous epithelium and filled with granulation tissue and keratin debris. Sometimes there are cholesterol clefts derived from the degenerated keratin and red cells (from hemorrhage), chronic inflammatory infiltrate and foreign body giant cell granulomas.

The squames are produced by a "matrix" composed of fully differentiated squamous epithelium similar to epidermis of skin but without adnexal structures.

Congenital cholesteatoma

Cholesteatoma of petrous apex:

Site: Petrous pyramid, cerebellopontine angle or the jugular fossa region.

It presents in middle aged patients with mandibular nerve pain, blurring of vision, and faintness related to the compression of the internal carotid artery.

 

Further reading

Immunomorphological evaluation of cholesteatoma.

Comparative analysis of the proliferative capacity of cholesteatomas.

Middle ear cholesteatoma: present-day concepts of etiology and pathogenesis.

Comparative analysis of the proliferative capacity of cholesteatomas.

Acquired and congenital cholesteatoma: determination of tumor necrosis factor-alpha, intercellular adhesion molecule-1, interleukin-1-alpha and lymphocyte functional antigen-1 in the inflammatory process.

Intracranial extension of acquired aural cholesteatoma.

Increased numbers of mast cells in human middle ear cholesteatomas: implications for treatment.

Cholesteatoma: a molecular and cellular puzzle.

Expression of p53 protein in human middle ear cholesteatomas: pathogenetic implications.

Differences in dendritic cells in congenital and acquired cholesteatomas.

 

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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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