Treatment of Primary Ovarian Lymphoma in Extra-Gastric Extranodal Marginal Zone Lymphoma
Clinical Scenario
This protocol covers primary ovarian lymphoma occurring in the setting of extra-gastric extranodal marginal zone lymphoma (EMZL) or non-gastric marginal zone lymphoma (NMZL). The therapeutic strategy is shaped by disease extent — localised versus disseminated — and whether an infectious aetiology has been identified.
Treatment Approach (Partial Overview)
Localised extra-gastric EMZL is primarily addressed with involved-site radiotherapy. When a specific causative microorganism is detected, targeted antimicrobial therapy may be used. Symptomatic or disseminated presentations involve systemic regimens — the full selection of options, eligibility criteria, and sequencing are detailed in the complete protocol.
References
DOI: 10.1016/j.annonc.2025.07.014
- ISRT can be recommended as the preferred treatment of localised extra-gastric EMZL (24 Gy; lower doses can be considered according to the site and clinical setting) [III, B].
- Specific antimicrobial therapy can be recommended in non-gastric MZL where an aetiopathogenetic microorganism [Campylobacter jejuni, Chlamydia psittaci, hepatitis C virus (HCV)] is detected [IV, B].
- R—chlorambucil [I, A].
- BR [III, A].
- R monotherapy [III, B].
- R—lenalidomide [III, C; not EMA or FDA approved].
- R-CVP [III, C].