Sloan Caroline, Xiong Qun-Bin, Crivaro Anne, Steinman Sharon, Bagg Adam
From the Department of Pathology and Laboratory Medicine, Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia, and.
St Luke's Hospital at St Luke's University Health Network, Allentown, PA.
Am J Clin Pathol. 2015 Feb;143(2):274-82; quiz 307. doi: 10.1309/AJCP06VIKFMTRCXD.
Mantle cell lymphoma in situ (MCLIS) consists of immunophenotypically defined but histologically inapparent neoplastic cells restricted to narrow mantle zones, without expansion or invasion beyond the mantle zone. We report a unique case of MCLIS associated with a much more manifest nodal marginal zone lymphoma (MZL) in an inguinal lymph node, porta hepatis lymph node, and bone marrow.
Biopsies from all three locations were evaluated using standard H&E-stained sections, immunohistochemistry, flow cytometry, metaphase cytogenetics, and/or fluorescence in situ hybridization (FISH).
This case is unique for three reasons. First, the histologically covert mantle cell lymphoma was multifocal, detected in all three locations using one or more of flow cytometry, immunohistochemistry, cytogenetics, and FISH. Second, the MCLIS was always accompanied by a more histologically dominant MZL. Third, where evaluable, it did not grow in an appreciable mantle zone distribution, presumably due to destruction of the normal nodal architecture by the neoplastic MZL cells and the resulting absence of recognizable follicles and mantle zones.
This unique case provides new insight into the pathogenesis of MCLIS.
原位套细胞淋巴瘤（MCLIS）由免疫表型定义但组织学上不明显的肿瘤细胞组成，这些细胞局限于狭窄的套区，未超出套区扩展或浸润。我们报告了一例独特的MCLIS病例，其与腹股沟淋巴结、肝门淋巴结及骨髓中更明显的结节性边缘区淋巴瘤（MZL）相关。
对来自所有三个部位的活检组织进行评估，采用标准苏木精-伊红（H&E）染色切片、免疫组化、流式细胞术、中期细胞遗传学及/或荧光原位杂交（FISH）技术。
该病例独特之处有三点。其一，组织学隐匿的套细胞淋巴瘤呈多灶性，通过流式细胞术、免疫组化、细胞遗传学及FISH技术中的一种或多种在所有三个部位均检测到。其二，MCLIS始终伴有组织学上更占优势的MZL。其三，在可评估的部位，其并非以明显的套区分布生长，推测是由于肿瘤性MZL细胞破坏了正常淋巴结结构，导致无法识别滤泡和套区。
这一独特病例为MCLIS的发病机制提供了新见解。
