朗格汉斯细胞肉瘤是一种临床、生物学和预后异质性的“恶性”组织细胞增生症:系统回顾88例文献。

IF 3.1 3区 医学 Q1 PATHOLOGY
Annalisa Dezzani, Chiara Punziano, Emilio Berti, Arturo Bonometti
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引用次数: 0

摘要

恶性组织细胞病是一种罕见的组织细胞肿瘤,具有侵袭性的临床和组织病理学特征。其中一种,朗格汉斯细胞肉瘤(LCS),与朗格汉斯细胞组织细胞增生症有一些组织病理学特征,但其明显的恶性细胞学特征是其区别所在。关于LCS的文献大多局限于简短的报道和一些综述,而缺乏对其分类学的完整修订。本研究旨在填补LCS知识的空白,探索潜在的预后因素,并提出临床亚分类,以便更好地对患者进行分层,指导未来的治疗研究。按照PRISMA指南对文献进行了系统的综述。从每个纳入的患者中,收集了一套完整的临床和病理特征。描述性统计和关联统计以及生存分析使用R Studio进行。对88例患者进行队列分析,大多数为成年男性,多系统图像常涉及皮肤和淋巴结。pERK通路基因突变约占一半。总体预后较差,尽管与另一种血液肿瘤的关联对预后有显著的负面影响(p = 0.0017)。此外,在原发性病例中,将患者分为单系统与多系统观察到显著差异(p = 0.012)。尽管治疗方式高度异质性,但统计分析提供了根据疾病扩散治疗患者的相关性的见解(例如,仅用手术治疗局部肿块经常导致完全缓解,p = 0.0002)。本研究对LCS的分类学和预后因素进行了广泛的分析,强调了区分LCS与LCH和其他组织细胞病的重要性,以及采用统一的系统来定义疾病传播和指导治疗管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Langerhans cell sarcoma is a clinically, biologically, and prognostically heterogeneous "malignant" histiocytosis: a systematic review of 88 cases from the literature.

Malignant histiocytoses are rare histiocytic neoplasms that exhibit aggressive clinical and histopathological features. One of these entities, Langerhans cell sarcomas (LCS), shares some histopathological features with Langerhans cell histiocytosis but is distinguished by its overtly malignant cytologic features. The literature on LCS is mostly limited to short reports and a few reviews, while a complete revision of its nosology is lacking. This study aims to fill this gap in the knowledge on LCS, explore potential prognostic factors, and propose a clinical subclassification for better patient stratification, which could guide future treatment investigations. A systematic review of the literature was conducted following PRISMA guidelines. From each included patient, a complete set of clinical and pathological features was collected. Descriptive and association statistics, as well as survival analysis, were performed using R Studio. A cohort of 88 patients was analyzed, the majority being adult males with multisystem pictures often involving skin and lymph nodes. pERK pathway gene mutations were reported in around half. Overall prognosis was poor, even though the association with another hematological neoplasm displayed a significant negative prognostic impact (p = 0.0017). Moreover, in primary cases, a significant difference was observed dividing patients into single system vs multisystem (p = 0.012). Despite treatment modalities being highly heterogeneous, statistical analyses provided insights into the relevance of treating patients according to disease spread (e.g., treating localized masses with surgery alone leads to frequent complete remission, p = 0.0002). This study provides an extensive analysis of LCS nosology and prognostic factors, underscoring the importance of distinguishing LCS from LCH and other histiocytoses, as well as adopting a unified system to define disease spread and guide therapeutic management.

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来源期刊
Virchows Archiv
Virchows Archiv 医学-病理学
CiteScore
7.40
自引率
2.90%
发文量
204
审稿时长
4-8 weeks
期刊介绍: Manuscripts of original studies reinforcing the evidence base of modern diagnostic pathology, using immunocytochemical, molecular and ultrastructural techniques, will be welcomed. In addition, papers on critical evaluation of diagnostic criteria but also broadsheets and guidelines with a solid evidence base will be considered. Consideration will also be given to reports of work in other fields relevant to the understanding of human pathology as well as manuscripts on the application of new methods and techniques in pathology. Submission of purely experimental articles is discouraged but manuscripts on experimental work applicable to diagnostic pathology are welcomed. Biomarker studies are welcomed but need to abide by strict rules (e.g. REMARK) of adequate sample size and relevant marker choice. Single marker studies on limited patient series without validated application will as a rule not be considered. Case reports will only be considered when they provide substantial new information with an impact on understanding disease or diagnostic practice.
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