Zhang Chun, Yi Yuanxue, Chen Chunyan, Wang Jianrong, Liu Zhu
Department of Pathology, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
Department of Orthopaedics, Chongqing Corps Hospital of Chinese People's Armed Polices, No. 90 Wei-guo Road, Nan'an, Chongqing, China.
Diagn Pathol. 2015 Dec 29;10:215. doi: 10.1186/s13000-015-0450-6.
Discordant lymphoma is defined by the simultaneous presence of two or more distinct types of lymphomas at different anatomic sites. With fewer than 20 studies reporting cases of discordant lymphoma to date, the incidence of this condition is believed to be very low.
Here, we report a case of discordant lymphoma in a 34-year-old female patient that involved mediastinal large B-cell lymphoma and nodular sclerosis Hodgkin lymphoma in the right supraclavicular lymph nodes. The patient presented with a mass in the mediastinum and enlargement of the right supraclavicular lymph nodes, but no obvious signs of lymphoma. Histological examination revealed that the encapsulated mediastinal mass contained medium- or large-size tumor cells with lightly stained cytoplasm and round vesicular nuclei as well as a high percentage of mitotic cells; strongly positive immunohistochemical staining for PAX5, CD20, and CD79a also was observed. Examination of biopsied right supraclavicular lymph node tissues revealed separation by collagen fibers, extensive inflammatory cell infiltration, and large-size tumor cells, such as Reed-Sternberg cells. These tissues stained strongly positive for PAX5 and CD30, weakly positive for CD15, and negative for Epstein-Barr viral RNA. We also found monoclonal gene rearrangement in the immunoglobulin heavy chain gene in the mediastinal large B-cell lymphoma, but no monoclonal gene rearrangement in the nodular sclerosis Hodgkin lymphoma. These findings suggested that these two lymphomas were not of a common clonal origin. The patient was treated by surgical excision of the mediastinal mass followed by radio-chemotherapy, and no metastasis or recurrence occurred during a follow-up period of 32 months.
A review of previously reported cases indicated that the clinical manifestations and pathological features of discordant lymphoma are diverse due to variation in the types of lymphomas involved. Physicians must have an awareness of discordant lymphoma to avoid incorrect and missed diagnoses, especially considering that the true incidence may not be as low as previously believed.
异质性淋巴瘤的定义是在不同解剖部位同时存在两种或更多不同类型的淋巴瘤。迄今为止，报告异质性淋巴瘤病例的研究少于20项，据信这种疾病的发病率非常低。
在此，我们报告一例34岁女性异质性淋巴瘤病例，该病例累及纵隔大B细胞淋巴瘤和右锁骨上淋巴结结节硬化型霍奇金淋巴瘤。患者表现为纵隔肿块和右锁骨上淋巴结肿大，但无明显淋巴瘤体征。组织学检查显示，包膜完整的纵隔肿块包含中等或大尺寸的肿瘤细胞，其细胞质淡染，核呈圆形泡状，且有高比例的有丝分裂细胞；还观察到PAX5、CD20和CD79a的免疫组化染色呈强阳性。对活检的右锁骨上淋巴结组织检查发现有胶原纤维分隔、广泛的炎症细胞浸润以及大尺寸肿瘤细胞，如里德-斯腾伯格细胞。这些组织PAX5和CD30染色呈强阳性，CD15染色呈弱阳性，爱泼斯坦-巴尔病毒RNA染色呈阴性。我们还在纵隔大B细胞淋巴瘤中发现免疫球蛋白重链基因的单克隆基因重排，但在结节硬化型霍奇金淋巴瘤中未发现单克隆基因重排。这些发现表明这两种淋巴瘤并非源自共同的克隆。患者接受了纵隔肿块手术切除，随后进行放化疗，在32个月的随访期内未发生转移或复发。
对既往报道病例的回顾表明，由于所涉及的淋巴瘤类型不同，异质性淋巴瘤的临床表现和病理特征多种多样。医生必须认识到异质性淋巴瘤，以避免错误和漏诊，特别是考虑到其实际发病率可能不像之前认为的那么低。
