Primary cutaneous follicle centre lymphoma natural history, complications and prognosis

Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.

• Primary cutaneous follicle centre lymphoma shows a predilection for the scalp, forehead, and trunk and dissemination to extracutaneous sites rarely occurs.^[1]
• Cutaneous relapses noted in ~30% of patients.^[2]
• Recurrences usually proximate to the initial site of the tumor.
• Untreated skin lesions increase in size, rarely metastases to other sites.

• Transformation of systemic follicular lymphoma (FL) into aggressive non-Hodgkin’s lymphoma is associated with poor prognosis and has been reported with a wide range of frequency (range between 10%–70%).
• While the annual risk of transformation of systemic follicular lymphoma (FL) is 3%.

• Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.
• The International Extranodal Lymphoma Study Group identified three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is prognostic of disease-free status.^[3]

• Elevated LDH
• More than two skin lesions
• Nodular lesions

• If left untreated, the lesions typically increase in size over years.
• In approximately 10 percent of patients may disseminate to extracutaneous sites.
  

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