What question did this study set out to answer?

This research aims to clarify the clinical outcomes, molecular features, and diagnostic challenges of rare dendritic and clear cell sarcomas.

February 19, 2026Open Access

Clear Cell and Histiocytic/Dendritic Cell Sarcomas: Clinical Outcomes, Molecular Features, and Diagnostic Pitfalls

GTGabriel TinocoThe Ohio State University MHMarium Husain DLDavid A. LiebnerThe Ohio State University Comprehensive Cancer Center – Arthur G. James Cancer Hospital and Richard J. Solove Research Institute

Key Points

This research aims to clarify the clinical outcomes, molecular features, and diagnostic challenges of rare dendritic and clear cell sarcomas.
Conducted a retrospective cohort study at The Ohio State University Comprehensive Cancer Center from 2010 to 2022
Analyzed demographics, treatment modalities, clinical outcomes, and molecular profiles
Recorded time to progression (TTP) and progression rates based on treatment types
Performed a structured literature review for context
Total of 10 patients with dendritic cell sarcoma and 5 with clear cell sarcoma were evaluable
62.5% of dendritic cell sarcoma patients progressed during or shortly after systemic therapies
75% of clear cell sarcoma patients progressed during or shortly after treatment
Overall mortality was 40% in dendritic cell sarcoma and 60% in clear cell sarcoma patients
TP53 mutations were found in 4 of 7 tested dendritic cell sarcoma cases

Abstract

Background: Dendritic and histiocytic cell sarcoma (DHCS) and clear cell sarcoma (CCS) are ultra-rare soft-tissue sarcomas characterized by diagnostic ambiguity, limited treatment guidelines, and poor outcomes. Their rarity has restricted the development of evidence-based management strategies, leaving clinical decisions reliant on small case series and institutional experience. DHCS typically presents without a unifying molecular driver and is often misclassified without comprehensive immunophenotyping. CCS is defined by EWSR1-ATF1/CREB1 fusions but exhibits low responsiveness to conventional chemotherapy. There remains a clear need to clarify natural history, therapeutic responses, and molecular characteristics in both. Methods: We conducted a retrospective cohort study of adult patients with histologically confirmed DHCS or CCS seen at The Ohio State University Comprehensive Cancer Center between 2010 and 2022. Demographics, treatment modalities, clinical outcomes, and molecular profiles were extracted and analyzed descriptively. Time to progression (TTP) and progression rates by treatment modality were recorded. A structured literature review was conducted to provide context for the findings. Results: Outcomes are descriptive and cohort-specific, reflecting institutional experience rather than generalizable estimates. Total of 10 patients with DHCS and 5 with CCS were evaluable. Most DHCS patients presented with metastatic disease. Among DHCS patients who received systemic therapies, five of eight (62.5%) experienced progression during or shortly after treatment. Among CCS patients who received systemic therapies, three of four (75%) progressed during or shortly after treatment. Overall mortality occurred in 4 of 10 DHCS patients (40%) and 3 of 5 CCS patients (60%). TP53 mutations were identified in four of seven next-generation sequencing (NGS)-tested DHCS cases, and PD-L1 positivity was detected in five of seven tested DHCS cases and one of five tested CCS cases. Conclusions: Despite multimodal treatment, this referral-based cohort of patients with ultra-rare DHCS and CCS showed high rates of progression and mortality. Our findings underscore the urgent need for multi-institutional collaboration and biomarker-driven clinical trials to guide management of these ultra-rare sarcoma subtypes.

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