Primary cutaneous follicle center lymphoma
# Primary cutaneous follicle center lymphoma

Primary cutaneous follicle center lymphoma is a type of lymphoma. It was recognized as a distinct disease entity in the 2008 WHO $classification.^{:218}$ PCFCL had been previously conceived as a variant of follicular lymphoma $(FL).^{:218}$

## Cause

Unlike FL, PCFCL is not typically associated with t(14;18) $translocation^{:218}$ although presence of that translocation does not exclude $PCFCL.^{:314}$ It is usually not associated with overexpressed $Bcl-2.^{:218}$

PCFCL represents about 55% to 60% of primary cutaneous B-cell lymphomas $(PCBCL);^{:373}$ primary cutaneous marginal zone lymphoma and diffuse large B-cell cell lymphoma, leg type are the other primary cutaneous B-cell $lymphomas.^{:373}$ The cause of PCFCL is $unknown.^{:312}$

## Treatment

Surgical removal and/or radiotherapy is given for localized $disease.^{:314}$ Radiation using multiple radiation fields is given if the disease has wider extent with grouped $lesions.^{:314}$ For the less common situation of more extensive disease (still confined to skin), rituximab without chemotherapy is $used.^{:314}$ Intralesional interferon alpha (IFN-α) and intralesional rituximab have been $used.^{:314}$ Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management and overall survival remains excellent.

## Prognosis

Spread from the skin is unusual, and the prognosis is $excellent^{:218}$ with a 5-year survival of over $97%.^{:314}$ The International Extranodal Lymphoma Study Group identified elevated LDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is prognostic of disease-free status.

