Synchronous case of follicular lymphoma and Langerhans cell sarcoma in the same lymph node

Abstract

Langerhans cell sarcoma (LCS) is a very rare histiocytic and dendritic cell neoplasm originating from Langerhans cells. There are case reports of histiocytic and dendritic cell neoplasms synchronously or sequentially observed in patients with malignant lymphoma. We present a case in which LCS and follicular lymphoma (FL) grade 3a were observed within the same lymph node. A 66‐year‐old male visited our hospital with a general malaise. Pleural effusion and systemic lymph adenopathy were detected. Biopsy of an inguinal lymph node was performed. The lymph node had regions with follicular structure and regions with acidophilic cytoplasm and large proliferating atypical cells. The tumor cells in the regions with follicular structure showed positivity for CD20 and BCL2 consistent with an FL grade 3a diagnosis. The tumor cells in the regions without follicular structure showed positivity for CD1a and S‐100 and were consistent with an LCS diagnosis. Both tumor cells showed the positivity of BCL6 split by FISH. PCR detected IgH clonality in DNA collected from each region, and direct sequence analysis of cells from both tumors detected almost identical amino acid sequences. This finding is important for future research on the development of very rare histiocytic and dendritic cell neoplasms.

Co-occurence of follicular lymphoma and Langerhans cell histiocytosis with primary parotid gland involvement: a case report

“…In 2018, a case of FL and HCL development in the same lymph node was reported by Shimono J. et al. 1 Both tumor cells showed positivity of BCL6, IgH clonality and some identical amino acid sequences in direct sequence analysis of DNA. That case is important in demonstrating both association and developmental origin of those two distinct entities.…”

“…Follicular lymphoma (FL) is one of the most common types of Non-Hodgkin Lymphoma (NHL) which originates from B-cells. 1 Generally it has an indolent clinical course, although histological transformation to aggressive lymphoma may occur in 25-35 % of cases, and therafter the prognosis has been reported to be poor. Transformation to histiocytic sarcoma may also be seen but very rarely.…”

“…To date, the co-existence in the same lymph node of LCH with FL has been reported in one case and with mantle cell lymphoma in another case. 1 , 5 However, there is no case in which LCH and FL have been determined in the same parotid gland (PG). The case is here reported of a patient with concomitant FL grade II and LCH which developed in the same PG.…”

Exploring the current status and trends of research on Langerhans Cell Sarcoma: A bibliometric analysis

“…The mechanism of this particular etiology is unclear and is still a matter of debate. Studies conducted within the last decade have also revealed that the development of LCS may not only be restricted to the histiocyte lineage but may also arise from hematopoietic tumors such as hairy cell leukemia, chronic lymphocytic leukemia/small lymphocytic lymphoma, and marginal-zone B-cell lymphoma [35][36][37][38][39][40][41][42][43][44][45].…”