Aaron R Weiss, Andrea Ferrari, Leo Mascarenhas, Gianni Bisogno
{"title":"Current Approaches to the Treatment of Pediatric Soft Tissue Sarcomas: Rhabdomyosarcoma and Nonrhabdomyosarcoma Soft Tissue Sarcomas.","authors":"Aaron R Weiss, Andrea Ferrari, Leo Mascarenhas, Gianni Bisogno","doi":"10.1016/j.hoc.2025.04.004","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.004","url":null,"abstract":"<p><p>Soft tissue sarcomas (STS) are rare mesenchymal tumors representing up to 7% of all cancers in children. In the pediatric population, rhabdomyosarcoma (RMS) is the most common histology while the remainder is composed of several distinct histotypes collectively known as nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). RMS and NRSTS have individualized staging and treatment paradigms. Through cooperative group clinical trials, our understanding of STS and outcomes have improved. However, patients with distant metastases and who relapse continue to fare poorly. Global collaborations are necessary to make greater progress for this highest risk population.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082164","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M Jane Underdown, Sarah Kappa, Jonathan Metts, Thomas Cash
{"title":"Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas.","authors":"M Jane Underdown, Sarah Kappa, Jonathan Metts, Thomas Cash","doi":"10.1016/j.hoc.2025.04.003","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.003","url":null,"abstract":"<p><p>The classification of small round cell sarcomas encompasses a spectrum of diseases ranging from those that are more widely understood, such as Ewing sarcoma and desmoplastic small round cell tumor, to more newly characterized diseases such as capicua (CIC)-rearranged sarcomas, BCOR-altered sarcomas, and sarcomas with EWSR1-non-ETS fusions. This article provides background, unique molecular features, and current and emerging therapies for each round cell sarcoma subtype.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlo M Cicala, Sebastian Bauer, Michael C Heinrich, César Serrano
{"title":"Gastrointestinal Stromal Tumor: Current Approaches and Future Directions in the Treatment of Advanced Disease.","authors":"Carlo M Cicala, Sebastian Bauer, Michael C Heinrich, César Serrano","doi":"10.1016/j.hoc.2025.04.006","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.006","url":null,"abstract":"<p><p>This review discusses the current and future landscape of advanced gastrointestinal stromal tumor (GIST) treatment. It covers the role of tyrosine kinase inhibitors (TKIs), specifically imatinib, and further treatment options, such as sunitinib, regorafenib, and ripretinib, as well as avapritinib for platelet-derived growth factor receptor alpha D842V mutations. In addition, this review emphasizes individualized treatment strategies within multidisciplinary expert teams, including surgery and other locoregional therapies, together with the importance of mutation-guided approaches, particularly for wild-type GISTs. Finally, it explores the potential of next-generation KIT inhibitors, combination therapies, and other investigational approaches.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Evolving Concepts in the Management of Desmoid Tumors.","authors":"Sylvie Bonvalot, Aisha Miah, Bernd Kasper","doi":"10.1016/j.hoc.2025.04.009","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.009","url":null,"abstract":"<p><p>DT is a rare monoclonal fibroblastic neoplasm with an unpredictable biologic behavior. Over the past 15 y, the worldwide approach to desmoid tumor (DT) has shifted dramatically from surgery, when feasible, to frontline active surveillance in the majority of cases, to select patients who truly require treatment. Approximately 50% of cases demonstrate indolent disease, while the remaining patients may require active treatment. Surgery and radiotherapy still have limited indications, but 3 recent randomized trials have provided new insights into medical treatments. This review will highlight the evolution of global concepts and strategies.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandra Merlini, Angelo Paolo Dei Tos, Pan Pantziarka, Silvia Stacchiotti
{"title":"Approaches to Ultra-Rare Sarcomas: Definitions, Diagnostics, and Therapies.","authors":"Alessandra Merlini, Angelo Paolo Dei Tos, Pan Pantziarka, Silvia Stacchiotti","doi":"10.1016/j.hoc.2025.04.008","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.008","url":null,"abstract":"<p><p>Ultra-rare sarcomas are sarcomas with an incidence of 1 case per million inhabitants per year, in which rarity poses a barrier to conducting histotype-specific randomized studies. This manuscript provides practical information and reference for clinicians and researchers looking to develop an understanding of the complexity, worldwide discrepancies in treatment options, and management choices for these patients. We provide definitions, diagnostic challenges, and therapeutic approaches to ultra-rare sarcomas. We highlight existing discriminations faced by patients with ultra-rare sarcoma, differences in approval status for innovative agents, and future perspectives for sarcoma experts who aim at working for a change in the field.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Osteosarcoma: Diagnosis, Treatment, and Emerging Opportunities.","authors":"Michael W Bishop","doi":"10.1016/j.hoc.2025.04.005","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.04.005","url":null,"abstract":"<p><p>Osteosarcoma is the most common primary bone malignancy of childhood and adolescence. Despite advances in cures for many pediatric neoplasms, outcomes for osteosarcoma have not significantly changed for nearly 4 decades and treatment is similar for patients with both standard and higher risk disease. Recent progress has been demonstrated with evidence of activity of multitargeted tyrosine kinase inhibitors for patients with advanced osteosarcoma; further study is needed to evaluate their use for newly diagnosed patients. Novel approaches seek to exploit common cell surface antigens (LRRC15 and B7-H3) as well as aberrant DNA repair mechanisms and cell cycle checkpoints.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Role of Hematopoietic Cell Transplantation in Pediatric and Adult Hemophagocytic Lymphohistiocytosis—Remaining Unknowns and Challenges","authors":"Sarah Nikiforow MD, PhD , Christine N. Duncan MD","doi":"10.1016/j.hoc.2025.03.002","DOIUrl":"10.1016/j.hoc.2025.03.002","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 3","pages":"Pages 645-660"},"PeriodicalIF":2.5,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144056162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Adi Zoref-Lorenz MD, PhD , Joseph Rocco MD , Daniella M. Schwartz MD , Michael Jordan MD
{"title":"Recognizing and Managing Secondary Hemophagocytic Lymphohistiocytosis in Adults","authors":"Adi Zoref-Lorenz MD, PhD , Joseph Rocco MD , Daniella M. Schwartz MD , Michael Jordan MD","doi":"10.1016/j.hoc.2025.02.007","DOIUrl":"10.1016/j.hoc.2025.02.007","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 3","pages":"Pages 577-596"},"PeriodicalIF":2.5,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144033947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Molecular Pathogenesis of the Histiocytic and Dendritic Cell Neoplasms","authors":"Benjamin H. Durham MD","doi":"10.1016/j.hoc.2025.03.001","DOIUrl":"10.1016/j.hoc.2025.03.001","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 3","pages":"Pages 471-490"},"PeriodicalIF":2.5,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Barbara A. Degar MD , Jennifer T. Huang MD , Jacob Bledsoe MD
{"title":"Clinical Characteristics and Treatment of Histiocytic Disorders in Children","authors":"Barbara A. Degar MD , Jennifer T. Huang MD , Jacob Bledsoe MD","doi":"10.1016/j.hoc.2025.02.006","DOIUrl":"10.1016/j.hoc.2025.02.006","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 3","pages":"Pages 513-529"},"PeriodicalIF":2.5,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144058449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}