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Mediastinum

Thymus

Thymic dysplasia


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 23 February 2013, last major update December 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Congenital thymic alteration due to developmental arrest
● Lack of differentiation of thymic epithelium, responsible for absence of Hassal's corpuscles, is main feature (Histopathology 1992;21:499)

Clinical features
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● Associated with severe combined immunodeficiency syndrome, ataxia-telangiectasia, chromosomal instability syndromes, Nezelof syndrome (Arch Pathol Lab Med 1987;111:1118)
● Incomplete form of DiGeorge syndrome is congenital anomaly with a constellation of findings that includes thymic hypoplasia (J Cutan Pathol 2008;35:380); complete form has absent thymus

Gross description
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● Small thymus (< 5g)

Micro description
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● Tubules and rosettes of primitive appearing epithelium without segregation into cortical and medullary regions
● No Hassallís corpuscles, no/rare lymphocytes

Differential diagnosis
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Acute thymic involution

End of Mediastinum > Thymus > Thymic dysplasia


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