An Unusual Journey From Lymphoma to Sarcoma: Presumed Transdifferentiation of Diffuse Large B‐Cell Lymphoma to Follicular Dendritic Cell Sarcoma—A Case Study

Diffuse large B‐cell lymphoma (DLBCL) is the most prevalent form of non‐Hodgkin lymphoma, capable of occurring in lymph nodes as well as various extranodal locations. In contrast, follicular dendritic cell sarcoma (FDCS) is a rare malignancy of low‐to‐intermediate grade that originates from stroma‐derived cells typically located in germinal centers. While histiocytic and dendritic cell neoplasms have been reported following B‐cell lymphomas, cases consistent with presumed transdifferentiation from DLBCL to FDCS remain exceedingly rare. We report a 42‐year‐old woman with a history of refractory germinal center subtype DLBCL who underwent multiple lines of chemoimmunotherapy and achieved a documented complete metabolic response (CMR). However, detailed follow‐up records during the subsequent interval were not fully available. Approximately two years later, she developed a rapidly enlarging cervical mass. Histopathological and immunohistochemical evaluation confirmed FDCS. Although the clinicopathologic findings were consistent with presumed lineage switching, molecular confirmation of clonality was not available. Despite surgical resection and initiation of gemcitabine‐based chemotherapy, the patient succumbed to a pulmonary embolism. This case highlights the diagnostic challenges of FDCS in heavily treated lymphoma patients and underscores the importance of molecular diagnostic testing to confirm clonal relationships. Given the limited evidence regarding optimal management of FDCS, a multidisciplinary approach remains essential.

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Publication Details

Journal
Case Reports in Hematology
Published
2026-01-01
DOI
https://doi.org/10.1155/crh/6130986
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

An Unusual Journey From Lymphoma to Sarcoma: Presumed Transdifferentiation of Diffuse Large B‐Cell Lymphoma to Follicular Dendritic Cell Sarcoma—A Case Study

Hana Saffar, F. Michael Ameli, Aysan Nozheh, Maryam Lotfi et al.
Case Reports in Hematology
Histiocytic Disorders and Treatments
article

An Unusual Journey From Lymphoma to Sarcoma: Presumed Transdifferentiation of Diffuse Large B‐Cell Lymphoma to Follicular Dendritic Cell Sarcoma—A Case Study

Hana Saffar, F. Michael Ameli, Aysan Nozheh, Maryam Lotfi, Amirabbas Sadrian
article en

Abstract

Diffuse large B‐cell lymphoma (DLBCL) is the most prevalent form of non‐Hodgkin lymphoma, capable of occurring in lymph nodes as well as various extranodal locations. In contrast, follicular dendritic cell sarcoma (FDCS) is a rare malignancy of low‐to‐intermediate grade that originates from stroma‐derived cells typically located in germinal centers. While histiocytic and dendritic cell neoplasms have been reported following B‐cell lymphomas, cases consistent with presumed transdifferentiation from DLBCL to FDCS remain exceedingly rare. We report a 42‐year‐old woman with a history of refractory germinal center subtype DLBCL who underwent multiple lines of chemoimmunotherapy and achieved a documented complete metabolic response (CMR). However, detailed follow‐up records during the subsequent interval were not fully available. Approximately two years later, she developed a rapidly enlarging cervical mass. Histopathological and immunohistochemical evaluation confirmed FDCS. Although the clinicopathologic findings were consistent with presumed lineage switching, molecular confirmation of clonality was not available. Despite surgical resection and initiation of gemcitabine‐based chemotherapy, the patient succumbed to a pulmonary embolism. This case highlights the diagnostic challenges of FDCS in heavily treated lymphoma patients and underscores the importance of molecular diagnostic testing to confirm clonal relationships. Given the limited evidence regarding optimal management of FDCS, a multidisciplinary approach remains essential.

Case Reports in HematologyVol. 2026(1)
Imam Khomeini Hospital (IR), Tehran University of Medical Sciences (IR)
Gender equality
Openalex Percentile: Top 3%
Histiocytic Disorders and Treatments
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