Concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma in a patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma

Abstract

The phenomenon of histiocytic/dendritic cell sarcomas arising through transformation of a pre‐existed lymphoproliferative disease is called transdifferentiation. Langerhans cell sarcoma transdifferentiating from chronic lymphocytic leukemia/small lymphocytic lymphoma is extremely rare and all the reported cases were localized in lymph nodes. We present a case of concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma, in which the chronic lymphocytic leukemia/small lymphocytic lymphoma preceded the development of the Langerhans cell sarcoma. A cutaneous lesion from a 63‐year‐old patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma was biopsied. The histologic examination revealed a mixture of two cell populations infiltrating diffusely the dermis. The first was composed of small lymphoid cells with somewhat monotonous appearance and mild nuclear atypia positive for PAX5, CD79a, CD20, CD23, CD5, and LEF1. The second was composed of large cells with abundant cytoplasm and pleomorphic nuclei. These cells were positive for CD1a, CD207, and S100 protein and exhibited a high mitotic rate and a high MIB‐1 immunostaining index. Therefore, two different entities, chronic lymphocytic leukemia/small lymphocytic lymphoma and Langerhans cell sarcoma, were detected in the same skin fragment. The patient died 3 years after initial diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma.

Chronic lymphocytic leukemia/small lymphocytic lymphoma complicated with skin Langerhans cell sarcoma: A case report

“…A primary history of CLL/SLL and long-term use of immunosuppressive agents may be the cause of skin LCS[ 2 , 4 - 6 , 11 - 13 ]. Previous studies showed that some CLL/SLL could transform into aggressive hematopoietic tumors of another lineage, such as LCS[ 2 , 4 - 6 , 11 - 13 ]. LCS clonally related to low-grade B-cell lymphomas and leukemias has been described[ 4 - 6 ].…”

“…Previous studies showed that some CLL/SLL could transform into aggressive hematopoietic tumors of another lineage, such as LCS[ 2 , 4 - 6 , 11 - 13 ]. LCS clonally related to low-grade B-cell lymphomas and leukemias has been described[ 4 - 6 ]. Among the previous studies, one reported that the two malignancies were detected in the same skin biopsy[ 4 ], and three other studies reported that the two malignancies were detected in the same lymph nodes[ 5 , 6 , 12 ].…”

“…Among the previous studies, one reported that the two malignancies were detected in the same skin biopsy[ 4 ], and three other studies reported that the two malignancies were detected in the same lymph nodes[ 5 , 6 , 12 ]. The mechanism is probably the transdifferentiation of the B cell lineage through direct transdifferentiation of neoplastic B cells into malignant histiocytes/DC, tumor lymphocytes dedifferentiate into early progenitor cells, and then progenitor cells redifferentiate into histiocytes/DCs[ 4 ]. It has been reported that the BRAF V600E mutation was found in patients with LCS and concurrent CLL/SLL[ 5 , 6 ].…”

“…LCS and CLL/SLL can occur in the same diseased tissues, such as lymph nodes or skin[ 4 - 6 ]. We report a patient with a history of CLL/SLL diagnosed with cutaneous LCS by skin biopsy, but there was no CLL/SLL in the cutaneous lesion of LCS.…”

The clinicopathological features of epithelioid undifferentiated sarcoma with TFE3 amplification: one case report and literature review

“…Studies have shown increased risks of second malignancies after CLL. Langerhans cell sarcoma 29 , myeloid sarcoma 30 , and histiocytic/dendritic cell sarcomas 31 would transdifferentiate from CLL. Skin neoplasm was also reported preceding the diagnosis CLL, including malignant melanoma 32 , Kaposi sarcoma [33][34][35] , Merkel cell carcinoma, malignant brous histiocytoma, dermato brosarcoma protuberans, sebaceous carcinoma 36 , and leiomyosarcoma 37 .…”