Yoon Sun Och
Department of Pathology, Yonsei University College of Medicine, Severance Hospital, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea.
Blood Res. 2024 May 7;59(1):18. doi: 10.1007/s44313-024-00015-9.
Histiocytic and dendritic cell neoplasms comprise diverse tumors originating from the mononuclear phagocytic system, which includes monocytes, macrophages, and dendritic cells. The 5th edition of the World Health Organization (WHO) classification updating the categorization of these tumors, reflecting a deeper understanding of their pathogenesis.In this updated classification system, tumors are categorized as Langerhans cell and other dendritic cell neoplasms, histiocyte/macrophage neoplasms, and plasmacytoid dendritic cell neoplasms. Follicular dendritic cell neoplasms are classified as mesenchymal dendritic cell neoplasms within the stroma-derived neoplasms of lymphoid tissues.Each subtype of histiocytic and dendritic cell neoplasms exhibits distinct morphological characteristics. They also show a characteristic immunophenotypic profile marked by various markers such as CD1a, CD207/langerin, S100, CD68, CD163, CD4, CD123, CD21, CD23, CD35, and ALK, and hematolymphoid markers such as CD45 and CD43. In situ hybridization for EBV-encoded small RNA (EBER) identifies a particular subtype. Immunoprofiling plays a critical role in determining the cell of origin and identifying the specific subtype of tumors. There are frequent genomic alterations in these neoplasms, especially in the mitogen-activated protein kinase pathway, including BRAF (notably BRAF V600E), MAP2K1, KRAS, and NRAS mutations, and ALK gene translocation.This review aims to offer a comprehensive and updated overview of histiocytic and dendritic cell neoplasms, focusing on their ontogeny, morphological aspects, immunophenotypic profiles, and molecular genetics. This comprehensive approach is essential for accurately differentiating and classifying neoplasms according to the updated WHO classification.
组织细胞和树突状细胞肿瘤包括源自单核吞噬系统的多种肿瘤，该系统包括单核细胞、巨噬细胞和树突状细胞。世界卫生组织（WHO）第5版分类更新了这些肿瘤的分类，反映了对其发病机制的更深入理解。在这个更新的分类系统中，肿瘤被分类为朗格汉斯细胞和其他树突状细胞肿瘤、组织细胞/巨噬细胞肿瘤以及浆细胞样树突状细胞肿瘤。滤泡树突状细胞肿瘤在淋巴组织的基质来源肿瘤中被分类为间充质树突状细胞肿瘤。组织细胞和树突状细胞肿瘤的每个亚型都表现出独特的形态特征。它们还显示出以各种标志物如CD1a、CD207/朗格蛋白、S100、CD68、CD163、CD4、CD123、CD21、CD23、CD35和ALK以及血液淋巴标志物如CD45和CD43为特征的免疫表型谱。EBV编码的小RNA（EBER）原位杂交可识别特定亚型。免疫谱分析在确定肿瘤的起源细胞和识别肿瘤的特定亚型方面起着关键作用。这些肿瘤中经常存在基因组改变，尤其是在丝裂原活化蛋白激酶途径中，包括BRAF（特别是BRAF V600E）、MAP2K1、KRAS和NRAS突变以及ALK基因易位。本综述旨在提供组织细胞和树突状细胞肿瘤的全面且更新的概述，重点关注它们的个体发生、形态学方面、免疫表型谱和分子遗传学。这种全面的方法对于根据WHO更新的分类准确区分和分类肿瘤至关重要。
