Lymphoma - Non B cell neoplasms - Indolent T-lymphoblastic proliferations

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Lymphoma - Non B cell neoplasms

T/NK cell disorders

Indolent T-lymphoblastic proliferations

Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 28 September 2011, last major update September 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Epidemiology
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● Rare, often in tonsils and oropharynx

Etiology
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● In tonsils, may arise from TdT positive cells that participate in lymphopoiesis (Am J Clin Pathol 2001;116:12)
● Unclear if hyperplastic or neoplastic
● Thymus-like function of the pharyngeal epithelium from which the thymus is embryologically derived may play a role in the striking oropharyngeal tropism

Sites
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● Tonsils, oropharynx, also liver, retroperitoneum
● No systemic dissemination

Clinical features
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● May recur repeatedly but no systemic involvement

Case reports
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● 58 year old woman with associated hepatocellular carcinoma (J Korean Med Sci 2010;25:309)
● Involvement of oropharynx in myasthenia gravis patient with multiple local recurrences over 11 years without systemic dissemination (Am J Surg Pathol 2001;25:411)
● Recurrent upper aerodigestive tract masses over 16 years, treated by excision, no chemotherapy or radiation therapy (Am J Surg Pathol 1999;23:977)
● Associated with Castleman lymphadenopathy (Leuk Lymphoma 2009;50:306)

Micro description
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● Diffuse infiltrate of lymphoblasts admixed with scattered, small, mature lymphocytes and cells with cytologic features intermediate between lymphoblasts and lymphocytes, may expand the interfollicular areas
● Mitotic figures relatively abundant
● Overall appearance similar to the histologic appearance of normal thymic cortex
● Foci of fibrosis containing lymphoblasts in “Indian file” arrangement characteristic of lymphoblastic lymphoma may occur
● Scattered mature and immature granulocytes and plasma cells are also present

Micro images
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With hepatocellular carcinoma

Positive stains
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● TdT, CD1, CD3, CD4 and CD8, CD43, CD45RO, Ki-67 (high), CD10 (weak)

Negative stains
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● Cytokeratin, CD20, CD15, CD30, EBV

Flow cytometry
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● Immunophenotype similar to T-ALL/LBL or cortical thymocytes

Genetics and Molecular
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● No clonal TCR gene rearrangements

Differential diagnosis
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● T cell lymphoblastic lymphoma: distinction is difficult on morphologic and immunophenotypic grounds, need lack of clonality by molecular studies

End of Lymphoma - Non B cell neoplasms > T/NK cell disorders > Indolent T-lymphoblastic proliferations

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