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Published on: June 13, 2019
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Diffuse Large B-Cell Lymphoma Transdifferentiating Into Histiocytic Sarcoma: Case Report + Systematic Review.
Patricia K Mansfield1, Sino Mehrmal2, M Yadira Hurley3,4
1Saint Louis University School of Medicine, Saint Louis University, St. Louis, Missouri, USA.
Journal of Cutaneous Pathology
|December 26, 2025
Summary
This study reports the first case of cutaneous histiocytic sarcoma (HS) in a patient with diffuse large B-cell lymphoma (DLBCL), linked by identical KRAS mutations. The findings suggest a rare transdifferentiation event from DLBCL to HS.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Diffuse large B-cell lymphoma (DLBCL) is an aggressive non-Hodgkin lymphoma.
- Cutaneous involvement in DLBCL can manifest as various skin lesions.
- Histiocytic sarcoma (HS) is a rare malignancy of myeloid or histiocytic origin.
Purpose of the Study:
- To report a unique case of cutaneous histiocytic sarcoma (HS) arising in a patient with a history of diffuse large B-cell lymphoma (DLBCL).
- To investigate the potential link between DLBCL and HS through genetic analysis.
- To review existing literature on cutaneous HS with underlying hematologic malignancies.
Main Methods:
- Clinical presentation and histopathologic examination of a patient with a rapidly growing skin tumor.
- Immunophenotypic characterization of tumor cells.
- Genetic analysis (KRAS mutation detection) of DLBCL and HS specimens.
- Systematic literature review of cutaneous HS cases.
Main Results:
- Histopathology confirmed cutaneous histiocytic sarcoma (HS) in a patient with prior diffuse large B-cell lymphoma (DLBCL).
- Identical p.G13D KRAS mutations were found in both the DLBCL and HS specimens, suggesting a common origin.
- Literature review identified only two prior cases of cutaneous HS with underlying hematologic transdifferentiation.
Conclusions:
- This case represents the first documented instance of systemic DLBCL with cutaneous involvement transdifferentiating into cutaneous HS, supported by identical KRAS mutations.
- The findings highlight a rare phenomenon of histiocytic transdifferentiation in the context of hematologic malignancy.
- Distinct clinical and pathological features underscore the unique nature of this case.

