Primary cutaneous follicle center lymphoma

A type of skin lymphoma

Primary cutaneous follicle center lymphoma (PCFCL) is a type of non-Hodgkin lymphoma that primarily affects the skin. It is characterized by the proliferation of neoplastic B-cells that form follicle-like structures in the skin. PCFCL is considered an indolent lymphoma, meaning it typically has a slow progression and a favorable prognosis.

Pathophysiology

PCFCL arises from the B-cells of the follicle center, which is a part of the lymphoid tissue involved in the production of antibodies. These cells undergo malignant transformation and form clusters or nodules in the skin. Unlike systemic follicular lymphoma, PCFCL is confined to the skin and does not involve the lymph nodes or other internal organs at the time of diagnosis.

Clinical Presentation

Patients with PCFCL typically present with solitary or grouped skin lesions that may appear as nodules, plaques, or tumors. These lesions are usually located on the head, neck, or trunk. The skin lesions are often asymptomatic, but some patients may experience itching or tenderness.

Diagnosis

The diagnosis of PCFCL is based on a combination of clinical examination, histopathological analysis, and immunophenotyping. A skin biopsy is performed to obtain a sample of the lesion, which is then examined under a microscope. The histological features include the presence of follicle center cells, which are large cells with cleaved nuclei. Immunohistochemistry is used to confirm the B-cell origin of the lymphoma, with markers such as CD20, CD10, and BCL6 being positive.

Treatment

The treatment of PCFCL depends on the extent and location of the disease. Localized lesions can often be treated with radiation therapy, which is highly effective. In cases where the disease is more widespread, systemic therapies such as rituximab (an anti-CD20 monoclonal antibody) may be used. Surgical excision of the lesions is another option, particularly for isolated nodules.

Prognosis

PCFCL generally has an excellent prognosis, with a high rate of long-term survival. The risk of progression to systemic disease is low, and most patients achieve complete remission with appropriate treatment. However, recurrences can occur, and long-term follow-up is recommended.

Related pages

• Non-Hodgkin lymphoma
• B-cell lymphoma
• Cutaneous lymphoma
• Follicular lymphoma