[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-375740-105":59,"doc-detail-375740-en":131},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":124,"head_meta":126,"extra_data":128,"updated_unix":130},105,"en","histiocytic-neoplasms-a-brief-review-and-differential-diagnosis","Histiocytic neoplasms - a brief review and differential diagnosis","","Histiocytic neoplasms encompass juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, ALK-positive histiocytosis, and histiocytic sarcoma, as defined in the 5th edition of the WHO Classification of Haematolymphoid Tumours. Although these entities show distinctive clinicopathologic and typical histologic features, proliferation of histiocytic cells can create morphological overlap, requiring careful differentiation from non-histiocytic and non-neoplastic conditions. The review summarizes clinical findings, molecular features, histopathology, and immunophenotypes while addressing differential diagnosis.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/histiocytic-neoplasms-a-brief-review-and-differential-diagnosis/375740/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/histiocytic-neoplasms-a-brief-review-and-differential-diagnosis/375740.png","ImageObject",300,407,{"name":92,"@type":93},"Genevieve","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-24",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What histiocytic neoplasms are covered in this review?","Question",{"text":113,"@type":114},"The review covers juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, ALK-positive histiocytosis, and histiocytic sarcoma within the WHO classification framework.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"Why is differential diagnosis challenging for histiocytic neoplasms?",{"text":118,"@type":114},"Morphological overlap can occur because many entities share proliferation of histiocytic cells, making separation from non-histiocytic and non-neoplastic conditions necessary.",{"name":120,"@type":111,"acceptedAnswer":121},"What does the review provide to support diagnosis and differentiation?",{"text":122,"@type":114},"It summarizes clinical findings, molecular features, histopathology, and immunophenotypes, then discusses differential diagnosis across the listed entities.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},375740,1790616774,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":52,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},1374391974585,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","Journal of clinical and experimental hematopathology Vol. 64 No. 3, 156-165, 2024  \nReview Article  \nHistiocytic neoplasms: a brief review and differential diagnosis  \nRin Yamada, Yoshihiro Komohara  \nHistiocytic neoplasms (HNs) include juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, ALKpositive histiocytosis, and histiocytic sarcoma in the 5th edition of the World Health Organization Classification of Haematolymphoid Tumours. These entities are clinicopathologically distinctive, and typical histological findings have been established. However, the common feature of a proliferation of histiocytic cells often leads to morphological overlap among HNs, and also necessitates a differential diagnosis from several non-HNs or non-neoplastic conditions. In this review, we provide a brief summary of the clinical findings, molecular features, histopathologies, and immunophenotypes of HNs, as well as to discuss their differential diagnosis.  \nKeywords: juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, ALK-positive histiocytosis, histiocytic sarcoma  \nINTRODUCTION  \nHistiocytic/dendritic cell neoplasms are tumors that originate from or show differentiation towards monocytes, macrophages, and dendritic cells. The 5th edition of the World Health Organization (WHO) Classification of Haematolymphoid Tumours categorizes them into plasmacytoid dendritic cell neoplasms, Langerhans cell and other dendritic cell neoplasms, and histiocytic neoplasms (HNs) . 1 HNs include juvenile xanthogranuloma (JXG), ErdheimChester disease (ECD), Rosai-Dorfman disease (RDD), ALKpositive histiocytosis (APH), and histiocytic sarcoma (HS) .1 These entities are clinicopathologically distinctive, and typical histological findings have been established. However, the common feature of a proliferation of histiocytic cells often leads to morphological overlap among HNs, and also necessitates a differential diagnosis from several non-HNs or non-neoplastic conditions. The aim of this review is to provide a brief summary of the clinical findings, molecular features, histopathologies, and immunophenotypes of HNs, as well as to discuss their differential diagnosis.  \nJuvenile xanthogranuloma  \nJXG is the most common HN and is characterized by the proliferation of histiocytes displaying varying degrees of lipidization or xanthomatization associated with maturation over time.2,3 The median age at diagnosis is 13 years, with a  \npeak incidence in the first year of life and a slight male predilection (a male-to-female ratio of 1.3–1.4), while cases in older people are also seen.2–4 Neurofibromatosis 1 patients have a higher incidence of JXG compared to the general population.5 Approximately 70% of cases exhibit driver alterations in various genes related to the mitogen-activated protein kinase (MAPK) pathway or receptor tyrosine kinase (RTK) .6 Among these, mutations in MAP2K1, KRAS/NRAS, or CSF1R, as well as NTRK1 fusion, are relatively prevalent, each occurring at a frequency of about 10% . The most common presentation is a solitary cutaneous lesion with a predilection for the head, neck, and trunk.3,4,7 Multiple cutaneous or extracutaneous lesions can also occur, but systemic involvement is less than 1% .8 Common sites of extracutaneous lesions include the soft tissue, ocular region, liver, spleen, lung, kidney, and central nervous system (CNS) .4,7 Cutaneous lesions often undergo spontaneous involution, and the prognosis is generally favorable. However, patients with intracranial lesions can experience serious permanent sequelae, and a few patients with systemic JXG die of the disease.3,9  \nMacroscopically, a cutaneous lesion, typically 5-to-10 mm in diameter, appears erythematous to yellowish, correlating with the degree of lipidization. 10 Microscopically, three patterns with a time-dependent course are recognized: early JXG (EJXG), classic JXG (CJXG), and late transitional JXG (TJXG) .3,4 EJXG is characterized by a compact s","cbCaibJ72kUJRqrG","https://ap.wps.com/l/cbCaibJ72kUJRqrG","pdf",1701695,"English","# Introduction\n# Juvenile xanthogranuloma\n# Erdheim-Chester disease","[{\"question\":\"What histiocytic neoplasms are covered in this review?\",\"answer\":\"The review covers juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, ALK-positive histiocytosis, and histiocytic sarcoma within the WHO classification framework.\"},{\"question\":\"Why is differential diagnosis challenging for histiocytic neoplasms?\",\"answer\":\"Morphological overlap can occur because many entities share proliferation of histiocytic cells, making separation from non-histiocytic and non-neoplastic conditions necessary.\"},{\"question\":\"What does the review provide to support diagnosis and differentiation?\",\"answer\":\"It summarizes clinical findings, molecular features, histopathology, and immunophenotypes, then discusses differential diagnosis across the listed entities.\"}]","Histiocytic neoplasms - a brief review and differential diagnosis | PDF",1790215970,25]