Iyer Sunil Girish, Kuker Russ, Florindez Jorge A, Saul Eduardo, Trabolsi Asaad, Rodriguez Gregor, Chapman Jennifer R, Lossos Izidore S, Alderuccio Juan Pablo
Department of Medicine, Division of Hematology, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL, USA.
Department of Radiology, University of Miami Miller School of Medicine, Miami, FL, USA.
Leuk Lymphoma. 2022 Mar;63(3):591-598. doi: 10.1080/10428194.2021.1992764. Epub 2021 Oct 21.
Breast extranodal marginal zone lymphoma (EMZL) is a rare malignancy. We performed the largest published to date single-center retrospective analysis of 13 patients with breast EMZL focusing on clinical characteristics and treatment-related outcomes. The rarity of this disease at our center was concordant with the prevalence reported in the literature, with breast EMZL comprising 2% of 654 MZL cases. Most patients presented with stage I-II disease however four (30.8%) patients had stage IV disease mostly due to occult bone marrow (BM) involvement. Interestingly, EMZL was frequently non-FDG avid (66.7%) on staging PET/CT. With a median follow-up of 3.1 years (range 5 months to 10.2 years), the 3-year progression free survival was 68.7% (95%CI 30.2%-88.9%) and overall survival 80.2% (95%CI 40.3%-94.8%). No patient experienced higher-grade transformation. Herein we show that localized breast EMZL can be effectively treated with radiation therapy providing long term disease control.
乳腺结外边缘区淋巴瘤（EMZL）是一种罕见的恶性肿瘤。我们对13例乳腺EMZL患者进行了迄今为止已发表的最大规模单中心回顾性分析，重点关注临床特征和治疗相关结果。该病在我们中心的罕见情况与文献报道的患病率一致，在654例MZL病例中，乳腺EMZL占2%。大多数患者表现为I-II期疾病，然而，有4例（30.8%）患者为IV期疾病，主要是由于隐匿性骨髓（BM）受累。有趣的是，在分期PET/CT检查中，EMZL通常对FDG不摄取（66.7%）。中位随访时间为3.1年（范围为5个月至10.2年），3年无进展生存率为68.7%（95%CI 30.2%-88.9%），总生存率为80.2%（95%CI 40.3%-94.8%）。没有患者发生高级别转化。在此我们表明，局限性乳腺EMZL可以通过放射治疗得到有效治疗，实现长期疾病控制。
