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This is a tumor of neoplastic follicle centre cells, including centrocytes and variable numbers of centroblasts, with a follicular and diffuse growth pattern that generally presents on the head or trunk. This is the most common type of primary cutaneous B-cell lymphoma. It mainly affects adults.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes 9690/3 or 9709/3.)
Patients usually present with lesions on the scalp, forehead or trunk. Lesions on the leg have a less favorable prognosis. Regional lymph node involvement is uncommon, but possible
Disease dissemination to distant nodes or other organs/tissue is rare and usually happens in the late stages of the disease.
Differences between follicle centre lymphoma and follicular lymphoma: 1. Follicle centre lymphoma is a cutaneous malignancy with only rare involvement of regional lymph nodes. 2. Follicular lymphoma commonly occurs in nodes and extranodal sites.
Note: If there is involvement of lymph node(s) that are not regional for the skin site involved, or involvement of bone marrow or organ(s), do not code histology primary cutaneous follicle centre cell lymphoma and do not code skin as the primary site. Code the histology to follicular lymphoma (See histology codes: 9690, 9691, 9695 or 9698)
Systemic therapy is only given for patients with very extensive cutaneous disease, extremely thick skin tumors, or with extracutaneous disease.