Primary ovarian lymphoma
ICD-10 C85.7 · ICD-11 2B33.5&XA1QK0

Treatment of Primary Ovarian Lymphoma in PCNSL When First-Line Therapy Has Failed or Disease Has Relapsed

This protocol addresses the clinical situation in which primary ovarian lymphoma presents in the context of primary central nervous system lymphoma (PCNSL) that has not responded to initial treatment, has relapsed early, or has relapsed after a period of remission.

Clinical Scenario

The relevant setting is primary central nervous system lymphoma (PCNSL), newly diagnosed. The challenge addressed here arises when the disease fails to achieve or sustain the expected response to induction therapy, or when late relapse occurs after consolidation.

Prior Treatment & Failure Condition

The previous line of treatment used high-dose methotrexate (HD-MTX)-based induction regimens — including MATRix, MPV, MBVP, R-MT, and related combinations — aiming for responsive or stable disease, with thiotepa-based consolidation in fit responders. This protocol is indicated when that goal was not met: disease remained refractory, relapsed early after induction, or relapsed late following consolidation.

Next-Step Approach (Partial Overview)

Subsequent management depends on patient fitness and the timing of relapse. Fit patients and those with late relapse have distinct pathways; patients not suitable for intensive treatment have separate options — including certain monotherapy or cellular therapy approaches.

The complete regimen selection, eligibility criteria, and sequencing are available in the full structured protocol →
Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.annonc.2025.07.014

Fit patients with refractory or early relapsed PCNSL can be treated with HD-ifosfamide- or HD-cytarabine-based combinations followed by ASCT or WBRT according to previous treatment [III, B].

Patients with refractory or early relapsed PCNSL unfit for polyChT may be treated with WBRT [V, C] or ibrutinib, lenalidomide monotherapy, temozolomide monotherapy or CAR-T therapy [III, B].

Patients with late relapse of PCNSL can be re-treated with HD-MTX, employing the same or a similar ChT regimen as used in first-line treatment and consolidated with ASCT or WBRT in responsive disease [IV, B].

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