Abstract
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.
Highlights
Histiocytic and dendritic cell neoplasms are rare among the tumors of hemapoietic and lymphoid tissues, and only account for less than 1% of hematolymphoid tumors presenting in lymph nodes [1]
Histiocytic and dendritic cell neoplasms are consisted of a group of tumors including histiocytic sarcoma (HS), Langerhans cell histiocytosis/Langerhans cell sarcoma (LCH/LCS), interdigitating dendritic cell sarcoma (IDCS) and follicular dendritic cell sarcoma (FDCS)
In order to get further information about the molecular characteristics of histiocytic and dendritic cell neoplasms and to learn more about the nature of the correlation between such group of tumors and lymphoma/ leukemia, we investigated the clonal status of 33 samples that included LCH, LCS, FDCS, IDCS, and HS
Summary
Histiocytic and dendritic cell neoplasms are rare among the tumors of hemapoietic and lymphoid tissues, and only account for less than 1% of hematolymphoid tumors presenting in lymph nodes [1]. In order to get further information about the molecular characteristics of histiocytic and dendritic cell neoplasms and to learn more about the nature of the correlation between such group of tumors and lymphoma/ leukemia, we investigated the clonal status of 33 samples that included LCH, LCS, FDCS, IDCS, and HS. Twenty eight of these cases were sporadic without a history or concurrent with lymphoma/leukemia, while three cases were with a past history of T-cell lymphoma, one case was followed extraosseous plasmacytoma, and one case was concomitant with diffuse large B-cell lymphoma (DLBCL)
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