primary cutaneous follicle centre lymphoma

primary cutaneous follicle centre lymphoma

An indolent, localised lymphoma of follicle centre cells—mostly centrocytes with some centroblasts—which comprises 12% of lymphomas, and is not typically associated with nodal disease.

Prognosis
Independent of histologic grade—based on number of centroblasts.

Sites
Most commonly in the head and neck; extracutaneous spread is rare. PCFCL in the legs and cases expressing FOX-P1 have a worse prognosis and require aggressive therapy similar to that used for diffuse large B-cell lymphoma (DLBCL).

Survival
5-year rate 90–95%; recurs in up to 40%.
References in periodicals archive ?
Primary cutaneous follicle centre lymphoma (PCFCL) is a separate diagnostic entity in the WHO 2008 classification [6] and also in the 2016 WHO criteria revision [7] accounting for approximately 60% of the primary cutaneous B-cell lymphomas [6].
The lack of bcl2-IgH fusion and the absence of lymphadenopathy favor a primary cutaneous follicle centre lymphoma with H/RS cells, a very rare diagnostic entity reported only in one prior case in the English medical literature.
Primary cutaneous follicle centre lymphoma (PCFCL) is most often noted in middle aged to elderly patients, slightly more frequent in men, and has predilection for the head, neck, and the trunk regions, forming single or multiple lesions, papules, plaques, or nodules.
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