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背景与目的:原发性乳腺恶性淋巴瘤(primary breast lymphoma,PBL)发病率低,预后较差。本研究探讨PBL的临床病理特点、诊断及治疗方法。方法:选择2001年1月—2012年1月经福建省肿瘤医院淋巴瘤内科收治的23例PBL患者的临床资料,均在福建省肿瘤医院接受手术或化疗并进行随访。回顾性分析总结患者的临床及病理特点、治疗和生存情况。结果:23例患者全部为女性,发病年龄27～87岁,中位年龄46岁。均为非霍奇金淋巴瘤,78%的患者为ⅠE期或ⅡE期。按照WHO 2001淋巴瘤病理分类标准,22例B细胞性淋巴瘤[19例弥漫大B细胞性淋巴瘤,1例黏膜相关淋巴组织结外边缘区B细胞淋巴瘤,1例小淋巴B细胞淋巴瘤,1例介于弥漫大B(diffuse large B-cell lymphoma,DLBCL)和伯基特(Burkitt lymphoma,BL)之间不能分类的B细胞淋巴瘤],1例ALK阳性的间变T细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)。16例行肿块切除术加术后化疗(4例加术后放疗),2例行根治术加术后放化疗,3例仅行单纯手术,1例Ⅳ期患者粗针穿刺活检取得病理后仅行全身化疗,1例Ⅳ期患者合并肾衰放弃治疗。19例患者在首次治疗后取得临床完全缓解(complete remission,CR),但随访中有10例复发,部位见于同侧及对侧乳腺,肝、脾、中枢神经系统(central nervous system,CNS)、骨及骨髓。有3例中枢侵犯者治疗期间未行鞘内预防。随访时间为4～114个月,中位16个月。全组死亡12例,中位生存时间为20个月,1、2、3、5年生存率分别为63.8%、47.1%、45.1%和41.0%。中位无进展时间为12个月,1、3、5年无进展生存率分别为46.2%、41.2%和26.9%。结论:PBL多为弥漫大B细胞型,预后差;PBL采用局部切除术联合放、化疗是目前主要的治疗模式;对于中高度恶性的PBL,首次治疗时接受CNS预防是必要的。
BACKGROUND & OBJECTIVE: Primary breast lymphoma (PBL) has a low incidence and poor prognosis. This study was to investigate the clinicopathological features, diagnosis and treatment of PBL. Methods: The clinical data of 23 patients with PBL who were admitted to Fujian Provincial Tumor Hospital from January 2001 to January 2012 were enrolled in the Fujian Tumor Hospital and were followed up. Retrospective analysis of the clinical and pathological features of patients, treatment and survival. Results: All 23 patients were female, with a mean age of onset of 27-87 years and a median age of 46 years. All non-Hodgkin’s lymphoma, 78% of patients with stage Ⅰ E or Ⅱ E. According to WHO 2001 classification criteria of lymphoma, 22 cases of B-cell lymphoma [19 cases of diffuse large B-cell lymphoma, 1 case of mucosa-associated lymphoid tissue extranodal marginal zone B-cell lymphoma, 1 case of small lymphoma B-cell lymphoma , One case of unclassifiable B-cell lymphoma between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL)], one ALK-positive anaplastic T-cell lymphoma (anaplastic large cell lymphoma, ALCL). Sixteen patients underwent radical resection combined with postoperative chemotherapy (4 patients treated with postoperative radiotherapy), 2 patients undergoing radical operation and postoperative chemoradiotherapy, 3 patients undergoing simple surgery, and 1 patient undergoing gross needle biopsy at stage IV. Line of systemic chemotherapy, 1 case of Ⅳ patients with renal failure give up treatment. Nineteen patients achieved complete remission (CR) after the first treatment. However, 10 patients relapsed during the follow-up. The sites were found in the ipsilateral and contralateral breast, liver, spleen, central nervous system (CNS) Bone and bone marrow. There were 3 cases of central aggression in the treatment of intrathecal prophylaxis. Follow-up time was 4 to 114 months, with a median of 16 months. The whole group of deaths in 12 cases, the median survival time was 20 months, 1, 2, 3, 5-year survival rates were 63.8%, 47.1%, 45.1% and 41.0%. The median progression-free time was 12 months. The progression-free survival rates at 1, 3 and 5 years were 46.2%, 41.2% and 26.9%, respectively. Conclusion: PBL is mostly diffuse large B cell type with poor prognosis. Partial excision of PBL combined with radiotherapy and chemotherapy is the main treatment modality. For middle and high grade PBL, it is necessary to receive CNS prophylaxis in the first treatment.