Institute of Hematology and Medical Oncology "L. & A. Seràgnoli", University of Bologna, Bologna, Italy.
Clin Lymphoma Myeloma Leuk. 2013 Jun;13(3):258-65. doi: 10.1016/j.clml.2012.10.003. Epub 2012 Dec 14.
Lymphomas with involvement of soft tissues as a primary event are very rare. The published studies have a small sample size, most of them being reported as case reports.
In this article we describe our experience with soft tissue non-Hodgkin lymphomas (NHL) diagnosed and treated in our institution over a 15-year period. Moreover, we systematically review the available data from the literature in the past 2 decades, considering all the published series and case reports available from 1990 to 2011 using a PubMed access.
In the monocentric analysis, 16 consecutive patients treated at our Institution from 1996 to 2011 were considered. In the literature search, we selected 16 case reports (18 patients) and 5 case series (49 patients), including a total of 67 patients. Eighty-three patients were finally considered in the combined analysis. The most common histologic subtype was diffuse large B cell lymphoma (DLBCL) (>50% of cases in both groups). In both analyses we observed an inferior outcome for DLBCL compared with indolent B-cell NHL (5-year progression free survival: 34% vs. 64%, respectively, in the combined analysis; P = .01). Furthermore, the prognosis in the DLBCL group appears to be worse compared with the historical data of DLBCL patients treated with chemoimmunotherapy.
Though indolent soft tissue B-cell NHLs appear to have a good outcome, soft tissue DLBCLs represent an anatomic-clinical entity with aggressive features, and dismal prognosis. Strategies of first-line therapy intensification could be considered. Studies aiming to a better biologic characterization of this peculiar entity are warranted.
原发性软组织淋巴瘤非常罕见。已发表的研究样本量较小，其中大多数为病例报告。
本文描述了我们机构在过去 15 年中诊断和治疗的软组织非霍奇金淋巴瘤（NHL）的经验。此外，我们系统地回顾了过去 20 年文献中的可用数据，使用 PubMed 检索，考虑了 1990 年至 2011 年发表的所有系列病例报告。
在单中心分析中，我们考虑了 1996 年至 2011 年在我们机构治疗的 16 例连续患者。在文献检索中，我们选择了 16 例病例报告（18 例患者）和 5 例病例系列（49 例患者），共 67 例患者。在联合分析中最终共考虑了 83 例患者。最常见的组织学亚型为弥漫性大 B 细胞淋巴瘤（DLBCL）（两组中均超过 50%的病例）。在这两种分析中，与惰性 B 细胞 NHL 相比，DLBCL 的结果较差（联合分析中，5 年无进展生存率分别为 34%和 64%，P =.01）。此外，与接受化疗免疫治疗的 DLBCL 患者的历史数据相比，DLBCL 组的预后似乎更差。
尽管惰性软组织 B 细胞 NHL 似乎有较好的结果，但软组织 DLBCL 是一种具有侵袭性特征和不良预后的解剖学临床实体。可能需要考虑一线治疗强化策略。需要进一步研究以更好地对这一特殊实体进行生物学特征分析。
