{"title":"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。","authors":"Annalisa Dezzani, Chiara Punziano, Emilio Berti, Arturo Bonometti","doi":"10.1007/s00428-025-04230-2","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":23514,"journal":{"name":"Virchows Archiv","volume":" ","pages":""},"PeriodicalIF":3.1000,"publicationDate":"2025-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Langerhans cell sarcoma is a clinically, biologically, and prognostically heterogeneous \\\"malignant\\\" histiocytosis: a systematic review of 88 cases from the literature.\",\"authors\":\"Annalisa Dezzani, Chiara Punziano, Emilio Berti, Arturo Bonometti\",\"doi\":\"10.1007/s00428-025-04230-2\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":23514,\"journal\":{\"name\":\"Virchows Archiv\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":3.1000,\"publicationDate\":\"2025-08-23\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Virchows Archiv\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00428-025-04230-2\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"PATHOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Virchows Archiv","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00428-025-04230-2","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PATHOLOGY","Score":null,"Total":0}
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.
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.