Dendritic Cell Sarcoma Characteristics and Clinical Management
Summary
Dendritic cell sarcomas are rare neoplasms arising from antigen-presenting dendritic cells, most commonly the follicular subtype located within lymphoid germinal centres. They exhibit a broad histological spectrum, ranging from spindle-cell to epithelioid morphologies, often accompanied by an inflammatory milieu. Diagnosis hinges on careful histopathological evaluation and immunophenotyping, with markers such as CD21, CD23 and CD35 establishing follicular dendritic cell lineage. Clinically, these tumours present as slow-growing masses in nodal or extranodal sites but carry a considerable risk of local recurrence and distant metastasis. Surgical excision remains the cornerstone of treatment for localized disease, while adjuvant radiotherapy or chemotherapy is considered in high-risk cases. Emerging evidence supports the incorporation of immune checkpoint inhibitors and targeted therapies for advanced or refractory presentations. Multidisciplinary management, integrating radiological assessment, molecular profiling and tailored systemic therapies, is essential to improve outcomes. Ongoing research aims to refine diagnostic algorithms, identify prognostic biomarkers and develop standardised protocols for immunotherapeutic interventions, underscoring the global imperative to enhance patient care and extend survival in this enigmatic malignancy.
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Dendritic Cell Sarcoma Characteristics and Clinical Management publication trend
The graph below shows the total number of articles in dendritic cell sarcoma characteristics and clinical management across all publications each year (not limited to Nature Index journals).
Technical terms
Dendritic Cell Sarcoma: A malignant tumour originating from dendritic antigen-presenting cells, encompassing various subtypes based on lineage and location.
Follicular Dendritic Cell Sarcoma (FDCS): A neoplasm arising from follicular dendritic cells within lymphoid tissues, identified by spindle- to ovoid-cell morphology and CD21/CD23/CD35 expression.
Inflammatory Pseudotumour-Like FDCS (IPT-like FDCS): An EBV-associated variant characterised by a prominent lymphoplasmacytic infiltrate and pseudotumorous growth pattern.
Immunohistochemistry: A technique employing antigen-specific antibodies to detect proteins in tissue sections, essential for tumour classification.
PD-1 Inhibitor: An immune checkpoint blocker that augments T-cell-mediated antitumour responses by inhibiting the programmed cell death-1 pathway.
References
- Favorable response to PD-1 inhibitor plus chemotherapy as first-line treatment for metastatic follicular dendritic cell sarcoma of the spleen: a case report. Frontiers in Immunology (2023).
- EBV-positive inflammatory follicular dendritic cell sarcoma of the colon with clonal immunoglobulin gene rearrangement: A case report and literature review. Heliyon (2024).
- Aggressive Variant of Hepatic Epstein–Barr Virus-Associated Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma with PD-L1 and SSTR2a Expression. Diagnostics (2023).
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