文章：

淋巴组织间充质树突状细胞肿瘤的当前视角：起源见解、分类更新及临床病理特征

Current Perspectives on Mesenchymal Dendritic Cell Neoplasms of Lymphoid Tissue: Insights into Ontogeny, Updates on Classification, and Clinicopathologic Characteristics

原文发布日期：19 June 2025

DOI: 10.3390/cancers17122055

类型: Article

开放获取: 是

英文摘要：

Mesenchymal dendritic cell neoplasms represent a distinct category of hematologic malignancies that challenge traditional classifications of histiocytic and classical dendritic/Langerhans cell neoplasms. Historically grouped under the broader umbrella of dendritic cell neoplasms, these entities differ significantly in their ontogeny, histopathologic features, molecular alterations, and clinical behavior. They are categorized into three main subtypes including follicular dendritic cell sarcoma, fibroblastic reticular cell tumor, and EBV-positive inflammatory follicular dendritic cell sarcoma/fibroblastic reticular cell tumor. They originate from mesenchymal stromal cells, and genetic alterations activating the NF- κβ pathway are frequent in follicular dendritic cell sarcomas. Immunophenotypic characterization is critical to distinguish these from other hematologic malignancies including histiocytic and classical dendritic/Langerhans cell neoplasms and other solid (non-hematopoietic) cancers. This review recapitulates current knowledge on existing classifications, details their diverse ontogeny from classical dendritic cell neoplasms, and provides insights into their clinicopathologic characteristics to improve diagnostic accuracy. We detail two case studies that demonstrate the challenges involved in the histopathologic diagnosis of these rare tumors, necessitating a comprehensive workup. Integrating developmental biology into practical diagnostic algorithms is essential to improve recognition and classification of these underdiagnosed neoplasms, ultimately guiding timely management.

摘要翻译： 

间充质树突状细胞肿瘤代表一类独特的血液系统恶性肿瘤，其存在挑战了传统组织细胞与经典树突状/朗格汉斯细胞肿瘤的分类体系。历史上这类肿瘤被归入广义的树突状细胞肿瘤范畴，但它们在细胞起源、组织病理学特征、分子改变及临床行为方面存在显著差异。主要分为三个亚型：滤泡树突状细胞肉瘤、成纤维细胞性网状细胞肿瘤以及EB病毒阳性炎性滤泡树突状细胞肉瘤/成纤维细胞性网状细胞肿瘤。此类肿瘤起源于间充质基质细胞，其中滤泡树突状细胞肉瘤常出现激活NF-κB信号通路的基因改变。免疫表型特征分析对于区分此类肿瘤与组织细胞/经典树突状/朗格汉斯细胞肿瘤及其他实体性（非造血系统）肿瘤至关重要。本文综述了现有分类体系的最新认知，详细阐述了其与经典树突状细胞肿瘤在发育起源上的差异，并深入剖析其临床病理特征以提高诊断准确性。我们通过两例病例研究具体展示了这类罕见肿瘤在组织病理诊断中面临的挑战，强调需进行全面的诊断评估。将发育生物学知识整合到实际诊断流程中，对于提高这类诊断不足肿瘤的识别与分类水平具有关键意义，最终为及时制定临床治疗方案提供指导。

原文链接：

Current Perspectives on Mesenchymal Dendritic Cell Neoplasms of Lymphoid Tissue: Insights into Ontogeny, Updates on Classification, and Clinicopathologic Characteristics