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Salivary glands

Lymphomas

MALT lymphoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 27 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Similar to MALT lymphoma elsewhere; low grade monocytoid B cells surround ducts and produce lymphoepithelial lesions
● Often associated with Sjogren’s syndrome (Mod Pathol 2000;13:4) or benign lymphoepithelial lesion, perhaps due to chronic antigenic stimulation

Clinical features
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● Most common lymphoma of salivary glands - 3% of tumors of major salivary glands; also occur in minor salivary glands
● Often bilateral, 30% multiglandular
● Cystic, 1-6 cm (median 2 cm), with ill demarcated margins (on imaging)
● May occur in children, post-transplantation (Am J Surg Pathol 2000;24:100), associated with Sjogren's syndrome, HIV, scleroderma, sialolithiasis, Waldenstrom macroglobulinemia (Am J Clin Pathol 2001;116:683), Warthin tumor
● Indolent, excellent prognosis, although 35% recur at 5 years
● Treat with radiotherapy

Diagnosis
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● Monoclonal by immunohistochemistry or flow cytometry; monocytoid infiltrates in regional lymph nodes
● Monoclonality in lymphoid infiltrates by PCR is insufficient for diagnosis

Case reports
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● Chronic sclerosing sialadenitis / Kuttner’s tumor (Am J Surg Pathol 2001;25:1546)

Micro description
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● Monocytoid cells surround ducts; low grade B cells
● May be Helicobacter pylori positive (tumor may regress after treatment)
● May have amyloid deposits, transformation to diffuse large B cell lymphoma

Micro images
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52 year old man


Parotid gland

Positive stains
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● CD19, CD20, CD22
● CXCL12, nuclear FOXP1, nuclear BCL10
● Rarely CD5+ (Hum Pathol 2012;43:1436)

Negative stains
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● CD5 (usually), CD10, BCL1/cyclin D1, CD45RD, EBV, HHV8

Molecular / cytogenetics description
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4 types of translocations (some organ specificity):
  ● t(14;18)(q32;q21): IGH / MALT1 (10-20% of salivary gland MALT)
  ● t(11;18)(q21;q21): API2 / MALT1
  ● t(1;14)(p22;q32): BCL10 / IGH
  ● t(3;14)(p14.1;q32): FOXP1 / IGH.t

● t(11;18)(q21;q21) is specific
● Also DAP-K methylation, A20 deletion, aneuploid

Differential diagnosis
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Kuttner tumor: may coexist

End of Salivary glands > Lymphomas > MALT lymphoma


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