Treatment of Primary Breast Lymphoma in the Extra-Nodal Marginal Zone Subtype (PB-MZL)
This protocol addresses primary breast lymphoma presenting specifically as the extra-nodal marginal zone subtype (PB-MZL) — a rare lymphoma arising in breast tissue with distinct clinical behaviour that guides the treatment selected.
PB-MZL has a similar incidence and prognosis to primary breast follicular lymphoma (PB-FL), but follows a more indolent clinical course. This indolent nature is a key factor in determining the appropriate management strategy for this subtype.
For PB-MZL, management may involve targeted immunotherapy, local radiation, or both — with the clinical objective of achieving a complete response of the breast lymphoma. The full treatment algorithm, specific regimen, and selection criteria are available in the complete structured protocol.
Primary breast extra-nodal marginal zone lymphoma (PB-MZL) has a similar incidence and prognosis to PB-FL but is more indolent.
For example, one case report described a patient who opted for active surveillance but developed multiple new breast masses one year after her initial diagnosis which required the administration of rituximab monotherapy until a complete response was achieved.
However, the authors noted that irradiation and/or rituximab would be considered for this patient if local and/or systemic symptoms should occur.
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