Treatment of Primary Breast ALCL, ALK-Positive, Arising Without Breast Implants
Primary breast anaplastic large cell lymphoma (PB-ALCL) with anaplastic lymphoma kinase (ALK) expression, occurring in patients with no breast implants, is an exceptionally rare entity. Its biology is defined by a specific chromosomal rearrangement that drives constitutive ALK pathway activation and tumour cell growth.
Clinical Scenario
This protocol addresses primary breast ALCL with confirmed ALK expression in the absence of breast implants. Fewer than 50 such cases have been reported in the published literature, making this presentation distinct from — and far rarer than — the implant-associated form of the disease.
Treatment Approach
Documented treatment has included surgical intervention combined with systemic chemotherapy. In at least one reported case, a targeted antibody-drug conjugate was incorporated alongside a chemotherapy backbone, with a notable clinical response observed. The complete regimen selection and criteria are detailed in the full structured protocol.
References
- Primary breast-ALCL (PB-ALCL), ALK+ is characterised by the presence of the t(2;5)(p23;q35) translocation juxtaposing the tyrosine kinase encoding domain of ALK to the nucleolar protein gene NPM1, resulting in the constitutive activation of ALK signalling and the upregulation of signalling pathways associated with cell proliferation and survival.
- Most cases of PB-ALCL arise in association with textured breast implants (Section 4); less than 50 cases of ALCL arising in the breast in the absence of implants have been reported.
- For example, one patient was successfully treated with surgery and Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) chemotherapy, and another case demonstrated an "excellent response" with cyclophosphamide, doxorubicin and prednisone (CHP) chemotherapy in combination with brentuximab vedotin (BV), even with antrum and bone marrow involvement.
DOI: 10.1016/j.jpra.2022.01.004
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