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Skin - Nonmelanocytic tumors

Lymphoma and related disorders

Diffuse large B-cell lymphoma


Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 16 July 2012, last major update June 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

See also Lymphoma chapter: diffuse large B cell - primary cutaneous-general, primary cutaneous-leg

General
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● Primary cutaneous cases, by definition, arise in skin with no known extracutaneous disease within 6 months of initial diagnosis
● Represents 20% of primary cutaneous lymphomas
● Others are extranodal marginal zone lymphoma, follicular lymphoma, T cell lymphomas
● Usually men (85%), mean age 64 years (Am J Clin Pathol 2002;117:574)
● Better outcome than other large B cell lymphomas, although similar to primary cutaneous follicular lymphoma - relapse common but estimated 5 year survival is 97%
● CD10+ tumors in head and neck have increased risk of relapse
● Can divide into follicular center type (CD10+, bcl6+, CD138- but without bcl2 translocations) and those with post-follicular center or activated B cell phenotype (CD10-, bcl2 usually negative)
● Secondary cases are more like classic follicular lymphoma (Am J Surg Pathol 2003;27:356)

Flow cytometry:
● Reactive T cells are 65-90% of cells (Arch Pathol Lab Med 1999;123:1236)

Treatment:
● Surgery, localized radiotherapy
● Systemic chemotherapy only for generalized skin lesions or disseminated lesions

Case reports
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● t(14;18)(q32;q21) involving IgH and MALT1 (not bcl2, distinguish MALT1 from bcl2 by FISH, Hum Pathol 2003;34:1212)
● Two patients with T cell rich B cell lymphoma (Mod Pathol 2001;14:10)

Clinical images
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Various images

Gross description
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● Solitary or grouped papules, plaques or tumors, usually in head, neck and trunk of older patients

Micro description
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● Either (a) mixed large and small B cells, (b) grade 3 follicular lymphomas (usually in head/neck and CD10+) or (c) monomorphous large cell (Am J Surg Pathol 2001;25:307)

Micro images
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Various images


A: Grenz zone; B: occasional large cells; C: CD3+ reactive lymphocytes; D: CD20+ tumor cells

Positive stains
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● CD20; variable bcl2 and bcl6
● Reactive T cells: CD3, CD45RO (UCHL-1)

Negative stains
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● Tumor cells: CD3 (positive in background cells), CD10 (usually)


Leg

General
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● 5-10% of primary cutaneous diffuse large B cell lymphomas cases
● Usually elderly patients
● May have poorer prognosis (Am J Surg Pathol 2003;27:1538), although somewhat controversial (Hum Pathol 2002;33:937)

Case reports
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● 97 year old man (Dermatol Online J 15;7:6)

Clinical images
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Diffuse large B cell lymphoma, leg type

Gross description
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● Solitary or grouped red/blue nodules

Micro description
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● Dense, diffuse large cells infiltrating entire dermis, usually thin grenz zone, cells resemble immunoblasts (large oval vesicular nuclei with prominent nucleoli) or centroblasts (large noncleaved nuclei, prominent nucleoli)

Micro images
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Diffuse large B cell lymphoma, leg type

End of Skin - Nonmelanocytic tumors > Lymphoma and related disorders > Diffuse large B-cell lymphoma


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