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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.
All variants of follicular lymphoma (NOS, grade 1, grade 2, and grade 3) were once called “follicle centre lymphoma.” Although that term is obsolete, it is sometimes used to describe follicular lymphoma. You will also see “follicle centre” in the pathology reports for follicular lymphoma. However, the primary site and other sites of involvement will differ between follicular lymphoma and follicle centre lymphoma. Follicle centre lymphoma is a cutaneous malignancy with only rare involvement of regional lymph nodes. Follicular lymphoma commonly occurs in nodes and extranodal sites. (See the Heme DB Abstractor Notes for both neoplasms for information on clinical presentation and common primary sites.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see codes 9690/3 or 9709/3.) This disease usually presents with solitary or localized skin lesions on the scalp, the forehead or the trunk. Also presents on the leg with a less favorable prognosis. Although it is not common, the regional lymph nodes may be involved. Disease dissemination to distant nodes or other organs/tissue is rare and usually happens in the late stages of the disease. Patient presents with firm erythematous to violaceous plaques, nodules or tumors of variable size. Systemic therapy is only given for patients with very extensive cutaneous disease, extremely thick skin tumors, or with extracutaneous disease.
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.