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Lymphoma - B cell neoplasms

Follicular lymphoma-cutaneous

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 1 February 2011, last major update February 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

General
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● By definition, arises in skin with no known extracutaneous disease within 6 months of initial diagnosis
● Note: this topic is identical in the Lymphoma-B cell and Skin-nonmelanocytic tumors chapters

Terminology
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● Also called primary cutaneous follicular cell lymphoma (PCFCL)

Etiology
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● Often has pathogenesis different from most nodal and secondary cutaneous follicular lymphomas (Am J Surg Pathol 2005;29:69)

Clinical features
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● Localized papulonodular lesions, often in head, neck and trunk, with excellent prognosis (5 year survival of 95%)
● Mean age 64 years
● By definition, arises in skin with no known extracutaneous disease within 6 months of initial diagnosis
● In Scottish Highlands, cutaneous B cell lymphomas (all subtypes) are associated with Borrelia burgdorferi (Lyme disease, Am J Surg Pathol 2000;24:1279)

Treatment
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● Radiation therapy for localized disease
● Combination chemotherapy for multifocal disease
● Antibiotics for Borrelia associated disease
● Avoid overtreatment, as these tumors have an excellent prognosis

Clinical images
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HIV+ man with secondary lesions on back

Gross description
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● Solitary or grouped papules, plaques or tumors

Micro description
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● Has follicular, follicular and diffuse, diffuse or floral-like growth patterns
● Follicular/nodular, follicular and diffuse, diffuse or floral-like growth patterns
● Germinal center cytology of predominantly centrocytes (cleaved follicular cells) with admixed centroblasts (large transformed cells, Am J Surg Pathol 2001;25:875)

Micro images
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Various images

   

HIV+ man with secondary lesions on back - follicular lymphoma grade 3a

Immunostains

Positive stains
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● CD19, CD20, CD79a
● bcl6+ and usually CD10+ centrocytes and centroblasts
● Similar to noncutaneous follicular lymphoma except that is commonly bcl2 negative or has weaker staining than admixed T-cells; compare with secondary cutaneous involvement by follicular lymphoma, which is bcl2+ (J Clin Oncol 2007;25:1581)

Negative stains
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● CD5

Molecular
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● t(14;18) in only 20-30% by PCR (Mod Pathol 2001;14:913, Mod Pathol 2001;14:828, Am J Surg Pathol 2000;24:694); FISH may be more sensitive (Am J Surg Pathol 2006;30:529) and specific than PCR (Am J Surg Pathol 2004;28:748)

Differential diagnosis
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● Cutaneous MALT lymphoma: negative for bcl6 and CD10, positive for bcl2, although colonized follicles [CD21+] are bcl6 and bcl2 positive (Am J Surg Pathol 2001;25:732)
● Disseminated follicular lymphoma involving skin: usually positive for bcl2 and t(14;18) (Mod Pathol 2001;14:913)
● Lymphoid hyperplasia: less crowding of follicles, bcl2 doesn’t stain germinal centers
● Primary cutaneous large B-cell lymphoma, leg type: IgM positive (Am J Surg Pathol 2010;34:1043)

End of Lymphoma - B cell neoplasms > Follicular lymphoma-cutaneous

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