Cholesteatoma

Cholesteatoma
	Diagnosis in short
	; Keratinaceous debris consistent with cholesteatoma. H&E stain. (WC/Nephron)
LM	keratinaceous debris (key feature), +/-squamous epithelium, +/-macrophages and giant cells giant cell, +/-chronic inflammation (lymphocytes)
LM DDx	cholesterol granuloma, squamous cell carcinoma, epidermal inclusion cyst - history different
Site	middle ear - see head and neck pathology
Clinical history	+/-family history, +/-history of trauma
Signs	whitish mass in the middle ear
Prognosis	benign

Cholesteatoma is a head and neck pathology ditzel.

General

Squamous epithelium in the middle ear - leading to accumulation of keratinaceous debris.^[1]
- Keratosis obturans - accumulation in the external ear canal - is considered to be a different process;^[2] though some consider it a synonym.^[3]
The etiology is not well understood.^[4]^[5]
- Theories include migration/hyperplasia, and metaplasia.^[5]
Rarely transforms into squamous cell carcinoma.^[6]^[7]

Classification

May be subdivided into:^[4]

Acquired - due to trauma, surgery or infection.
Congenital.

Gross

Whitish mass in the middle ear.^[8]

Image

Cholesteatoma. (WC)

Microscopic

Features:^[9]

Keratinaceous debris - key feature.
Squamous epithelium.
Macrophages +/- giant cell (containing keratinceous debris).
Chronic inflammation (lymphocytes).

DDx:

Cholesterol granuloma.^[10]
Squamous cell carcinoma.^[6]
Epidermal inclusion cyst - history different.
- Obscure immunostains different.^[11]

Images

Cholesteatoma --very low mag.
Cholesteatoma -- low mag.
Cholesteatoma -- intermed. mag.
Cholesteatoma -- intermed. mag.
Cholesteatoma -- high mag.

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Submitted as "Left Mastoid Cholesteatoma", Excision:
	- Keratinaceous debris and benign squamous epithelium, consistent with cholesteatoma.

Soft tissue, left ear ("left ear keratosis"), excision:
	- Keratinaceous debris, squamous epithelium and bone (consistent with cholesteatoma).

Block letters

SOFT TISSUE (CHOLESTEATOMA), SITE NOT FURTHER SPECIFIED, REMOVAL:
	- KERATINACEOUS DEBRIS, COMPATIBLE WITH CHOLESTEATOMA.

TISSUE ("CHOLESTEATOMA"), LEFT, REMOVAL:
	- KERATINACEOUS DEBRIS AND GIANT CELLS, COMPATIBLE WITH CHOLESTEATOMA.

Micro

The section shows keratinaceous debris and benign squamous epithelium with a granular layer. There is no significant cellular atypia and giant cells are not present.

References

↑ URL: http://www.harrisonspractice.com/practice/ub/view/Harrisons%20Practice/141015/all/otitis_media_and_mastoiditis. Accessed on: 16 March 2011.
↑ Piepergerdes MC, Kramer BM, Behnke EE (March 1980). "Keratosis obturans and external auditory canal cholesteatoma". Laryngoscope 90 (3): 383–91. PMID 7359960.
↑ Shire JR, Donegan JO (September 1986). "Cholesteatoma of the external auditory canal and keratosis obturans". Am J Otol 7 (5): 361–4. PMID 3538893.
↑ ^4.0 ^4.1 Nevoux, J.; Lenoir, M.; Roger, G.; Denoyelle, F.; Ducou Le Pointe, H.; Garabédian, EN. (Sep 2010). "Childhood cholesteatoma.". Eur Ann Otorhinolaryngol Head Neck Dis 127 (4): 143-50. doi:10.1016/j.anorl.2010.07.001. PMID 20860924.
↑ ^5.0 ^5.1 Louw, L. (Jun 2010). "Acquired cholesteatoma pathogenesis: stepwise explanations.". J Laryngol Otol 124 (6): 587-93. doi:10.1017/S0022215109992763. PMID 20156369.
↑ ^6.0 ^6.1 Rothschild, S.; Ciernik, IF.; Hartmann, M.; Schuknecht, B.; Lütolf, UM.; Huber, AM.. "Cholesteatoma triggering squamous cell carcinoma: case report and literature review of a rare tumor.". Am J Otolaryngol 30 (4): 256-60. doi:10.1016/j.amjoto.2008.06.011. PMID 19563937.
↑ Takahashi, K.; Yamamoto, Y.; Sato, K.; Sato, Y.; Takahashi, S. (Jan 2005). "Middle ear carcinoma originating from a primary acquired cholesteatoma: a case report.". Otol Neurotol 26 (1): 105-8. PMID 15699729.
↑ Al Balushi, T.; Naik, JZ.; Al Khabori, M. (Jan 2013). "Congenital cholesteatoma in identical twins.". J Laryngol Otol 127 (1): 67-9. doi:10.1017/S0022215112002757. PMID 23217274.
↑ Iino Y, Toriyama M, Ohmi S, Kanegasaki S (1990). "Activation of peritoneal macrophages with human cholesteatoma debris and alpha-keratin". Acta Otolaryngol. 109 (5-6): 444–9. PMID 1694387.
↑ URL: http://path.upmc.edu/cases/case273/dx.html. Accessed on: 14 January 2012.
↑ Dallari, S.; Cavani, A.; Bergamini, G.; Girolomoni, G. (Mar 1994). "Integrin expression in middle ear cholesteatoma.". Acta Otolaryngol 114 (2): 188-92. PMID 7515549.

[1] URL: http://www.harrisonspractice.com/practice/ub/view/Harrisons%20Practice/141015/all/otitis_media_and_mastoiditis. Accessed on: 16 March 2011.

[2] Piepergerdes MC, Kramer BM, Behnke EE (March 1980). "Keratosis obturans and external auditory canal cholesteatoma". Laryngoscope 90 (3): 383–91. PMID 7359960.

[3] Shire JR, Donegan JO (September 1986). "Cholesteatoma of the external auditory canal and keratosis obturans". Am J Otol 7 (5): 361–4. PMID 3538893.

[pmid20860924-4] 4.0 ^4.1 Nevoux, J.; Lenoir, M.; Roger, G.; Denoyelle, F.; Ducou Le Pointe, H.; Garabédian, EN. (Sep 2010). "Childhood cholesteatoma.". Eur Ann Otorhinolaryngol Head Neck Dis 127 (4): 143-50. doi:10.1016/j.anorl.2010.07.001. PMID 20860924.

[pmid20156369-5] 5.0 ^5.1 Louw, L. (Jun 2010). "Acquired cholesteatoma pathogenesis: stepwise explanations.". J Laryngol Otol 124 (6): 587-93. doi:10.1017/S0022215109992763. PMID 20156369.

[pmid19563937-6] 6.0 ^6.1 Rothschild, S.; Ciernik, IF.; Hartmann, M.; Schuknecht, B.; Lütolf, UM.; Huber, AM.. "Cholesteatoma triggering squamous cell carcinoma: case report and literature review of a rare tumor.". Am J Otolaryngol 30 (4): 256-60. doi:10.1016/j.amjoto.2008.06.011. PMID 19563937.

[pmid15699729-7] Takahashi, K.; Yamamoto, Y.; Sato, K.; Sato, Y.; Takahashi, S. (Jan 2005). "Middle ear carcinoma originating from a primary acquired cholesteatoma: a case report.". Otol Neurotol 26 (1): 105-8. PMID 15699729.

[pmid23217274-8] Al Balushi, T.; Naik, JZ.; Al Khabori, M. (Jan 2013). "Congenital cholesteatoma in identical twins.". J Laryngol Otol 127 (1): 67-9. doi:10.1017/S0022215112002757. PMID 23217274.

[pmid1694387-9] Iino Y, Toriyama M, Ohmi S, Kanegasaki S (1990). "Activation of peritoneal macrophages with human cholesteatoma debris and alpha-keratin". Acta Otolaryngol. 109 (5-6): 444–9. PMID 1694387.

[10] URL: http://path.upmc.edu/cases/case273/dx.html. Accessed on: 14 January 2012.

[pmid7515549-11] Dallari, S.; Cavani, A.; Bergamini, G.; Girolomoni, G. (Mar 1994). "Integrin expression in middle ear cholesteatoma.". Acta Otolaryngol 114 (2): 188-92. PMID 7515549.

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