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Cutaneous Marginal Zone Lymphoma : Prognosis

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Cutaneous Marginal Zone Lymphoma (CMZL) - Treatment & Prognosis: CMZLs are indolent tumors and most are localized to the skin at presentation. For localized or scattered multifocal lesions, the treatment consists of surgery or low-dose radiation therapy.

Patients with extensive multifocal lesions can be treated with topical or intralesional steroids, intralesional interferon alpha or rituximab, or low-dose radiation. Systemic chemotherapy is rarely required and is reserved for patients with extracutaneous spread. In cases associated with B. burgdorferi infection, systemic antibiotics are given before using more aggressive therapies.

Most patients achieve remission, but relapses occur in 50% of cases, especially in those with multifocal lesions. Rare cases spread to involve lymph nodes, bone marrow, or other MALT sites (liver, spleen, parotid, orbit, breast, and GI tract). Prognosis is excellent with a 5-yr. disease-specific survival rate of >90%. Transformation to high-grade lymphomas is rare.

The image shows neoplastic infiltrate surrounding adnexal structures of skin from a case of CMZL.

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