Treatment of Primary Breast ALCL with Absent ALK Expression and No Breast Implants

This page addresses first-line management of primary breast anaplastic large cell lymphoma (ALCL) in patients with absent anaplastic lymphoma kinase (ALK) expression and no breast implants — a rare and clinically distinct lymphoma presentation that falls outside the better-characterised breast implant-associated ALCL category.

Clinical Scenario

Systemic ALCL that is ALK-negative presenting primarily within the breast (PB-ALCL, ALK−) without associated breast implants is an uncommon and incompletely characterised entity. The absence of ALK expression and the non-implant context define a specific subset that requires dedicated management consideration, separate from both BIA-ALCL and ALK-positive systemic ALCL.

Treatment Approach (Partial Overview)

Evidence in this rare setting supports chemotherapy-based treatment, with one approach incorporating brentuximab vedotin alongside a combination chemotherapy backbone. The complete protocol — including all regimen options, selection criteria, and the full clinical algorithm — is available via the structured evidence-based resource below.

Full regimen details, including all treatment options and decision criteria, are behind the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jpra.2022.01.004

There have been limited cases of systemic ALCL, ALK− presenting primarily within the breast (PB-ALCL, ALK−).

Both cases responded well to treatment with BV and cyclophosphamide, doxorubicin, etoposide and prednisone (CHEP) chemotherapy or CHOP chemotherapy alone.

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