Background: Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) most commonly follows an indolent course; however, a subset of patients display more advanced disease marked by recurrent and disseminated growth refractory to treatment. This study evaluated outcomes of advanced disease, specifically bilateral disease, lymph node involvement, organ metastasis, and/or disease-related death. Methods: Published cases of BIA-ALCL from 1997 to 2018 and unpublished cases at the authors' institution were retrospectively reviewed, and patients with advanced disease were selected. Treatment and outcomes were compared against a control of BIA-ALCL subjects without advanced disease. Results: Thirty-nine patients with advanced BIA-ALCL were identified who had bilateral disease (n = 7), lymph node and organ metastasis (stage IIB-IV, n = 24), and disease-related death (n = 8). Sixty-five patients were included in a comparison control group (stage 1A-1C). Treatment types for advanced disease patients were complete surgery, n = 16 (55.2%); limited surgery, n = 19 (65.5%); chemotherapy, n = 26 (89.7%); salvage chemotherapy, n = 11 (37.9%); radiation, n = 15 (51.7%); and autologous stem cell transplant, n = 6 (20.7%). The rates of complete remission for the bilateral and lymphadenopathy groups were 4 of 7 (57%, P < 0.001) and 16 of 24 (67%, P = 0.128), respectively. Compared with the control group, advanced disease patients had significantly longer time from diagnosis to definitive surgery (21 versus 8 months, P = 0.039) and a lower rate of complete surgery (59% versus 88%, P = 0.004). Conclusions: Advanced disease BIA-ALCL may be a consequence of a delay or suboptimal treatment of BIA-ALCL. Optimal adjuvant chemotherapy and indications for radiation for BIA-ALCL patients with advanced features are not yet clearly defined. Advanced disease is the end of the spectrum of cancer stages, and these patients substantiate the World Health Organization classification of BIA-ALCL as a lymphoma rather than benign or lymphoproliferative.
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