Lymphoma - B cell neoplasms - Plasmablastic lymphoma of oral mucosa type

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Lymphoma - B cell neoplasms

Plasmablastic lymphoma of oral mucosa type

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 7 January 2012, last major update March 2011
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Clinical features
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● Usually involves mucosa of oral cavity and jaw with local invasion and rapid dissemination to extraoral sites; also nasal cavity, paranasal sinuses, eyelid, orbit or occasionally other sites with similar morphology
● No serum monoclonal protein, no significant bone marrow involvement
● Usually HIV+ (73%), EBV+; occasionally HIV- but immunosuppressed or elderly patients without known immunodeficiency
● More common than non oral mucosal type
● Usually male, median age 48 years (range 11-86 years)
● Aggressive with poor prognosis (survival 1-16 months)
● Originates from plasmablast, a blastic B-cell with phenotype switched to that of a plasma cell
● May be associated with Castleman disease
● Secondary cases are associated with another plasma cell neoplasm
● Variant with plasmacytic differentiation: no gender predominance; median age 55 years (range 30-86 years); nodal or extranodal; may occur in oral cavity; only 33% HIV+; usually bcl6 and HHV8 negative; also aggressive - most patients die in first year after diagnosis

Case reports
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● 44 year old man with parotid gland tumor (Head Neck Pathol 2010;4:148)
● 47 year old HIV+ man with presentation as lung tumor (Arch Pathol Lab Med 2001;125:282)
● 51 year old man with 8 year history of HIV (Rare Tumors 2011;3:e11)
● 82 year old, HIV- man with cervical nodal tumor (Arch Pathol Lab Med 2004;128:581)
● Tumors with immunoblastic features in HIV+ patients with Kaposi’s sarcoma (Mod Pathol 2003;16:424)

Micro description
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● Monotonous proliferation of large lymphoid cells with immunoblastic features (abundant basophilic cytoplasm with occasional paranuclear hofs, open chromatin, prominent central nucleoli); cohesive tumor cells may resemble plasmablasts
● Tumor cells infiltrate in large cohesive masses with relatively well defined advancing edge
● Frequent mitotic figures, apoptotic cells, occasional tingible body macrophages, cells with plasmacytic features
● Variant with plasmacytic differentiation has predominance of immunoblasts and plasmablasts (rounded nuclei, coarse chromatin, smaller nuclei), also small cells with plasmacytic differentiation, and occasional binucleated cells with typical plasma cell cartwheel chromatin

Micro images
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44 year old man with parotid gland tumor

47 year old HIV+ man with presentation as lung tumor

51 year old man with 8 year history of HIV

82 year old, HIV- man with cervical nodal tumor

Positive stains
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● CD38, CD138, MUM1, EMA, EBV EBER, HHV8 (Am J Surg Pathol 2004;28:41), CD79a (50-85%)
● CD10, CD43, CD45RO, bcl2, bcl6, CD30, Ki67 index very high
● Variable cytoplasmic light or heavy chain expression, variable HHV8

Negative stains
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● CD3, CD19, CD20, CD45, PAX5, usually ALK-

Molecular
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● Monoclonal immunoglobulin heavy chain gene rearrangement

Molecular image
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PCR of IgH gene rearrangement

Differential diagnosis
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● Plasma cell myeloma: fewer blast-type cells, fewer mitotic figures, HHV8- (Am J Surg Pathol 2004;28:736)
● Burkitt lymphoma: CD20+

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Plasmablastic lymphoma of oral mucosa type

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