Primary cutaneous follicle center lymphoma is a type oflymphoma.[1] It was recognized as a distinct disease entity in the 2008 WHO classification.[2]: 218 PCFCL had been previously conceived as a variant offollicular lymphoma (FL).[2]: 218
Unlike FL, PCFCL is not typically associated with t(14;18)translocation[2]: 218 although presence of that translocation does not exclude PCFCL.[2]: 314 It is usually not associated with overexpressedBcl-2.[2]: 218
Surgical removal and/orradiotherapy is given forlocalized disease.[2]: 314 [6] Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions.[2]: 314 [3][5][6] For the less common situation of more extensive disease (still confined to skin),rituximab without chemotherapy is used.[2]: 314 [3][5][6] Intralesionalinterferon alpha (IFN-α)[7] and intralesional rituximab have been used.[2]: 314 [7] Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management[6] and overall survival remains excellent.[8]
Spread from the skin is unusual, and theprognosis is excellent[2]: 218 with a 5-year survival of over 97%.[2]: 314 The International Extranodal Lymphoma Study Group identified elevatedLDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess acutaneous lymphomainternational prognostic index (CLIPI), which isprognostic of disease-free status.[6]
