Langerhans cell sarcoma in a patient with hairy cell leukemia: common clonal origin indicated by identical immunoglobulin gene rearrangements

Abstract

Histiocytic/dendritic cell sarcomas are rare tumors, a few of which have been reported in association with B-cell lymphoma/leukemia. Isolated reports have documented identical immunoglobulin gene rearrangements suggesting a common clonal origin for both the sarcoma and the B-cell neoplasm from individual patients. We report a case of a 75-year-old male with hairy cell leukemia who subsequently developed Langerhans cell sarcoma 1 year after his primary diagnosis of leukemia. The bone marrow biopsy containing hairy cell leukemia and skin biopsies of Langerhans cell sarcoma were evaluated by routine histology, immunohistochemistry, flow cytometric immunophenotyping and PCR-based gene rearrangement studies of the immunoglobulin heavy chain and kappa genes. The hairy cell leukemia showed characteristic morphologic, immunohistochemical and flow cytometric features. The Langerhans cell sarcoma showed pleomorphic cytology, a high mitotic rate and characteristic immunohistochemical staining for Langerin, S100 and CD1a. There was no evidence of B-cell differentiation or a background B-cell infiltrate based on the absence of immunoreactivity with antibodies to multiple B-cell markers. Identical immunoglobulin gene rearrangements were identified in both the hairy cell leukemia and Langerhans cell sarcoma specimens. Despite the phenotypic dissimilarity of the two neoplasms, identical immunoglobulin gene rearrangements indicate a common origin.

Langerhans Cell Sarcoma in the Cervical Lymph Node: A Case Report and Literature Review

“…In addition, LCS occurring after organ transplantation has been reported, such as following liver transplantation [5,6], likely related to long-term immunosuppression. Recently, LCS arising from other tumors, especially B cell leukemia, has been reported [7,8,9]. Based on our research, only 36 previous cases and 37 total cases including the present study have been reported in the English literature to date (table 1).…”

Cytological findings of langerhans cell sarcoma in a case of quintuple cancer

“…However, KRAS and BRAF gene mutations were not detected in our case. There have been several reports on LCS arising from other tumors of hematopoietic and lymphoid tissues 13, 14, 15, 16. However, there has been no report on LCS related to T‐cell/histiocyte‐rich large B‐cell lymphoma.…”

Langerhans cell sarcoma: a case report and review of the literature

“…Inflammatory cell infiltration, consisting of lymphocytes and eosinophil gran- Table I. Summary of the reported cases of Langerhans cell sarcoma [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35] ulocytes, was also found, and isolated mitotic figures were identified ( Fig. 3A and 3B).…”