Huang Wenting, Qiu Tian, Zeng Linshu, Zheng Bo, Ying Jianming, Feng Xiaoli
Department of Pathology, National Cancer Center, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Oncotarget. 2016 Nov 29;7(48):78355-78362. doi: 10.18632/oncotarget.13058.
The 2008 World Health Organization (WHO) diagnostic criteria of histiocytic and dendritic cell neoplasms from hematopoietic and lymphoid tissues no longer required the absence of clonal B-cell/T-cell receptor gene rearrangements. It is true that the clonal B-cell/T-cell receptor gene rearrangements have been identified in rare cases of histiocytic and dendritic cell neoplasms, such as those with or following lymphoma/leukemia or in some sporadic histiocytic/dendritic cell sarcomas, but the clonal features of such group of tumor are still not clear. Here we investigated the clonal status of 33 samples including Langerhans cell histiocytosis (LCH), Langerhans cell sarcoma (LCS), follicular dendritic cell sarcoma (FDCS), interdigitating dendritic cell sarcoma (IDCS) and histiocytic sarcoma (HS). Among them, twenty-eight cases were sporadic without current or past lymphoma/leukemia. Three cases were found with a past history of T-cell lymphoma, one case was followed by extraosseous plasmacytoma, and one case was found with diffuse large B-cell lymphoma (DLBCL). Our results showed that there was a high frequency of clonal IG and T-cell receptor gene rearrangements in these cases. Notably, 4 cases of LCH and 2 cases of FDCS showed both B and T cell receptor gene rearrangements concurrently. One case of FDCS synchronous with DLBCL showed identical clonal IGH in both tumor populations and clonal TCRβ in FDCS alone. No matter if the presence of clonal receptor gene rearrangements was associated with the tumor origin or tumorigenesis, it might serve as a novel tumor marker for developing target therapy.
2008年世界卫生组织（WHO）发布的造血与淋巴组织组织细胞和树突状细胞肿瘤诊断标准不再要求不存在克隆性B细胞/T细胞受体基因重排。确实，在罕见的组织细胞和树突状细胞肿瘤病例中已鉴定出克隆性B细胞/T细胞受体基因重排，例如那些伴有淋巴瘤/白血病或之后发生淋巴瘤/白血病的病例，或一些散发性组织细胞/树突状细胞肉瘤，但这类肿瘤的克隆特征仍不清楚。在此，我们研究了33个样本的克隆状态，包括朗格汉斯细胞组织细胞增多症（LCH）、朗格汉斯细胞肉瘤（LCS）、滤泡树突状细胞肉瘤（FDCS）、交错突树突状细胞肉瘤（IDCS）和组织细胞肉瘤（HS）。其中，28例为散发性，无当前或既往淋巴瘤/白血病病史。3例有T细胞淋巴瘤病史，1例继发于骨外浆细胞瘤，1例伴有弥漫性大B细胞淋巴瘤（DLBCL）。我们的结果显示，这些病例中克隆性免疫球蛋白（IG）和T细胞受体基因重排的频率很高。值得注意的是，4例LCH和2例FDCS同时出现了B细胞和T细胞受体基因重排。1例与DLBCL同时发生的FDCS在两个肿瘤群体中均显示相同的克隆性免疫球蛋白重链（IGH），而仅在FDCS中显示克隆性T细胞受体β链（TCRβ）。无论克隆性受体基因重排的存在是否与肿瘤起源或肿瘤发生相关，它都可能成为开发靶向治疗的新型肿瘤标志物。
