This protocol addresses primary ovarian lymphoma arising in the setting of mantle cell lymphoma in patients who are unsuitable for high-dose cytarabine-based induction therapy and autologous stem cell transplantation (ASCT). When these intensified approaches are not feasible, a distinct set of first-line options applies.
For patients unsuitable for HD-cytarabine-based induction therapy and ASCT, one of the following options can be recommended. The choice among available regimens accounts for the patient's fitness, tolerability, and the availability of specific agents.
Management involves rituximab-based combination immunochemotherapy regimens, with certain options incorporating targeted agents. Maintenance strategies — where applicable — vary by the chosen induction regimen. The full evidence-based regimen selection, including preferred options and maintenance considerations, is detailed in the structured protocol.
For patients unsuitable for HD-cytarabine-based induction therapy and ASCT, one of the following options can be recommended:
BR [I, B; preferred] and consider R maintenance therapy for 2 years [II, B].
Bortezomib—R—cyclophosphamide—doxorubicin—prednisone (VR-CAP) [I, B] with R maintenance therapy for 2 years [IV, B].
R—bendamustine—cytarabine (R-BAC) [III, B] without R maintenance therapy [IV, D].
BR—acalabrutinib induction followed by acalabrutinib until progression or toxicity plus 2 years of R maintenance therapy [I, B].
R-cBTKi induction therapy (not EMA or FDA approved) followed by 2 years of R maintenance therapy plus cBTKi until progression or toxicity [I, B].
DOI: 10.1016/j.annonc.2025.07.014
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