原发性乳腺淋巴瘤10例临床病理特征 及预后分析

Primary Breast Lymphoma: Clinicopathological Features and Prognostic Analysis of 10 Cases

摘要: 目的：探讨原发性乳腺淋巴瘤(PBL)的临床病理特征、治疗及预后。方法：回顾分析徐州医科大学附属医院2015年1月至2023年12月收治的10例PBL患者的临床病理特点、免疫表型及随访信息，并复习相关文献。结果：10例均为女性，发病年龄27~58岁，平均42.6岁；左乳4例，右乳5例，双乳1例，均为发现无痛性进行性增大肿块就诊。镜下均见异型淋巴细胞浸润乳腺组织生长，表达B细胞或T细胞标记，其中乳腺原发弥漫性大B细胞淋巴瘤(PB-DLBCL) 8例，7例非GCB型，1例GCB型；1例为黏膜相关淋巴组织结外边缘区(MALT)淋巴瘤伴大B转化；1例为T淋巴母细胞淋巴瘤(T-LBL)。10例患者仅1例伴B症状，同时累及骨髓。根据Lugano改良的Ann Arbor分期标准，IE期4例，IIE期4例，IV期2例。10例PBL中，1例为孕中期时发现，1例为孕晚期发现。治疗多以R-CHOP方案化疗4~6个疗程伴自体干细胞移植为主。截止2024年1月，除1例患者因肿瘤溶解综合征直接死亡外，9例患者在随访13~68个月后，1例为复发后死亡、1例复发后存活、1例因其他疾病死亡，余均存活并未复发。结论：PBL罕见，其诊疗需结合临床、影像及分子病理特征进行多维度评估。病理亚型是决定治疗方案的核心因素，其中妊娠期CD5阳性PB-DLBCL罕见报道。

Abstract: Objective: This paper aims to investigate the clinicopathological features, treatment, and prognosis of primary breast lymphoma (PBL). Methods: A retrospective analysis was conducted on 10 PBL patients treated at the Affiliated Hospital of Xuzhou Medical University from January 2015 to December 2023. Clinicopathological features, immunophenotypes, and follow-up data were evaluated, with a review of relevant literature. Results: All 10 cases were female, aged 27~58 years (mean 42.6 years). Tumors occurred in the left breast (4 cases), right breast (5 cases), and both breasts (1 case). All patients presented with painless, progressively enlarging masses. Histopathology revealed infiltrating atypical lymphocytes in breast tissue, with expression of B-cell or T-cell markers. Eight cases were classified as primary breast diffuse large B-cell lymphoma (PB-DLBCL): 7 non-GCB subtype and 1 GCB subtype. One case was mucosa-associated lymphoid tissue (MALT) lymphoma with large B-cell transformation, and one case was T-lymphoblastic lymphoma (T-LBL). Only one patient exhibited B symptoms and concurrent bone marrow involvement. According to the Lugano-modified Ann Arbor staging system, 4 cases were stage IE, 4 were stage IIE, and 2 were stage IV. Among the 10 cases, one was diagnosed during mid-pregnancy and another during late pregnancy. Treatment primarily involved rituximab-based combination chemotherapy (R-CHOP) (4~6 cycles) combined with autologous stem cell transplantation. As of January 2024, one patient died directly from tumor lysis syndrome, while nine patients showed varying outcomes after a median follow-up of 13~68 months: one died post-recurrence, one survived post-recurrence, one died of other diseases, and the remaining seven remained alive without recurrence. Conclusion: PBL is rare, and its diagnosis and treatment require a multidimensional evaluation integrating clinical, imaging, and molecular pathological features. Histopathological subtype is the core determinant of treatment strategy. Notably, a case of gestational CD5-positive primary breast diffuse large B-cell lymphoma (PB-DLBCL) is rarely reported.