Patients >60 years or less medically fit are given cyclophosphamide, doxorubicin, vincristine, and prednisone while patients ≤60 years old are given CHOP plus etoposide or one of various other intensive chemotherapy regiments. The intensive chemotherapy regimens give 5 year overall survival rates of 70–93%. The role of radiation therapy for ALK-positive ALCL is unclear but has been used for patients who cannot tolerate or do not achieve complete responses to the drug regimens and to patients with organ-threatening or life-threatening tumorous infiltrates. The role of autologous or less preferably allogenic hematopoietic stem cell transplantation (transplantation using the individuals own bone marrow, i.e. autologous, or a donors, i.e. allogenic) after achieving a complete remission following induction therapy is also unclear. Individuals with relapsed or refractory disease are treated with brentuximab vedotin if they did not receive the drug previously or had not received it in the previous 6 months. A small study reported overall response rates, complete response rates, and disease-free survival rates at 24 months of 63%, 45%, and 54%, respectively, using this regimen. Those who attain a complete response on this drug and can tolerate it are than treated with bone marrow transplantation. Finally, patients who fail or relapse on these treatments are given salvage therapy regimens that have been used for relapsed or refractory aggressive B cell malignancies such as GDP (i.e.
The development of BIA-ALCL, it has often been suggested, may be at least in part a T-cell-induced, inflammation-driven cancer response to the implant. Treatment and prognosis
The treatment regimens for BIA-ALCL recommended by 1) a multidisciplinary expert review panel, 2) the National Comprehensive Cancer Network, and 3) the French National Cancer Institute (Agence Nationale de Sécurité du Médicament [ANSM]) are very similar, commonly used, and summarized here. BIA-ALCL staging is done to identify patients with BIA-ALCL confined to the implant, capsule, and effusion from more disseminated disease. The staging preferably employs the TMN system designed to stage solid tumors. This is based on historical data suggesting that BIA-ALCL progresses locally like solid tumors rather than liquid tumors such as other lymphomas. BIA-ALCL patients have surgical removal of the implant, capsule, and associated masses. Patients with localized disease (e.g. TMN stage 1A to 2A) that is completely excised by removal of the implant, the entire capsule, and any masses (must leave negative resection margins) receive no further therapy. About 85% of all BIA-ALCL patients should qualify to receive this treatment regimen. Patients with unresectable chest wall invasion, regional lymph node involvement (i.e. TMN Stage 2B to 4), or residual disease after surgery receive an aggressive adjuvant chemotherapy regimen such as EPOCH, CHOP, or CHOP plus etoposide. Alternatively, the immunotherapeutic drug, brentuximab vedotin, may be used as initial therapy alone or in combination with a chemotherapy regimen to treat disseminated disease.