Article
Pathology
Karen J. Fritchie, Baptiste Ameline, Vanghelita Andrei, Christopher Griffith, Akeesha A. Shah, Josephine K. Dermawan, Matteo Trucco, Thomas Budd, Judith J. Thangaiah, Jeremy Molligan, Rumeal D. Whaley, Kelly Magliocca, Elizabeth Azzato, Annemieke van Zante, Vickie Jo, Bin Xu, Justin A. Bishop, Lisa Rooper, Daniel Baumhoer
Summary: This study explored the relationship between Ewing sarcoma and ALES using DNA methylation profiling. The results showed that ALES has a distinct methylation signature from conventional Ewing sarcoma. This finding strengthens the understanding that ALES should be considered a separate entity from Ewing sarcoma.
Article
Oncology
Matthew T. T. Houdek, Mark J. J. Heidenreich, Safia K. K. Ahmed, Wendy Allen-Rhoades, Brittany L. L. Siontis, Steven I. I. Robinson, Ivy A. A. Petersen, Peter S. S. Rose
Summary: The study evaluated the treatment experience of EES patients and found that chemotherapy and surgery can achieve excellent local control.
JOURNAL OF SURGICAL ONCOLOGY
(2023)
Article
Oncology
Brittany L. Murphy, Min Yi, Angelica M. Gutierrez Barrera, Debu Tripathy, Kelly K. Hunt, Banu K. Arun
Summary: This study found that more than 25% of breast cancer patients who underwent germline panel testing were diagnosed with other primary malignancies (OPMs) during the short-term follow-up period, and the diagnosis of an OPM was associated with reduced overall survival (OS).
ANNALS OF SURGICAL ONCOLOGY
(2023)
Article
Oncology
Brittany L. Murphy, Min Yi, Angelica M. Gutierrez Barrera, Debu Tripathy, Kelly K. Hunt, Banu K. Arun
Summary: Women with a history of breast cancer are more likely to be diagnosed with other primary malignancies. This study found that over 25% of patients who underwent germline testing for breast cancer were diagnosed with other primary malignancies in the short-term follow-up period, and this diagnosis was associated with reduced overall survival.
ANNALS OF SURGICAL ONCOLOGY
(2023)
Article
Oncology
Logan G. Spector, Aubrey K. Hubbard, Brandon J. Diessner, Mitchell J. Machiela, Beau Webber, Joshua D. Schiffman
Summary: Ewing sarcoma is the second most common primary bone tumor in children and adolescents, with few known epidemiological or genetic risk factors. Studies show significant variation in ES incidence across different geographic regions, indicating potential ancestral influence on disease risk.
INTERNATIONAL JOURNAL OF CANCER
(2021)
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Junhao Wu, Ya Liu, Weidong Gong, Taiping Liao, Chunyin Zhang
Summary: Subcutaneous Ewing sarcoma is rare and often presents as a mass. Diagnosis is typically confirmed through PET/CT scans and histopathological tests.
CLINICAL NUCLEAR MEDICINE
(2021)
Review
Oncology
Ajay Gupta, Richard F. Riedel, Chirag Shah, Scott C. Borinstein, Michael S. Isakoff, Rashmi Chugh, Jeremy M. Rosenblum, Erin S. Murphy, Shauna R. Campbell, Catherine M. Albert, Stacey Zahler, Stefanie M. Thomas, Matteo Trucco
Summary: Ewing sarcoma is a common malignant tumor in adolescents and young adults. This review focuses on the experience of the National Ewing Sarcoma Tumor Board and aims to provide guidelines and recommendations for the upfront management of Ewing sarcoma patients.
Article
Oncology
Bree R. Eaton, Line Claude, Daniel J. Indelicato, Ralph Vatner, Brian Yeh, Rudolf Schwarz, Nadia Laack
Summary: Ewing sarcoma requires complex multidisciplinary management, with standard treatment involving a combination of chemotherapy, surgery, and radiation followed by consolidation local treatment. Data support the efficacy of these treatment modalities.
PEDIATRIC BLOOD & CANCER
(2021)
Review
Oncology
Daria Fayzullina, Sergey Tsibulnikov, Mikhail Stempen, Brett A. Schroeder, Naveen Kumar, Rajesh Kumar Kharwar, Arbind Acharya, Peter Timashev, Ilya Ulasov
Summary: Ewing sarcoma is a rare malignant bone tumor with a high recurrence rate. This study identifies new therapeutic targets, particularly the EWSR1/FLI1 fusion protein, and proposes experimental therapy targeting multiple signaling pathways for improved patient survival.
Article
Oncology
David Boyce-Fappiano, B. Ashleigh Guadagnolo, Ravin Ratan, Wei-Lien Wang, Michael J. Wagner, Shreyaskumar Patel, John A. Livingston, Patrick P. Lin, Kevin Diao, Devarati Mitra, Ahsan Farooqi, Alexander J. Lazar, Christina L. Roland, Andrew J. Bishop
Summary: This study reviewed the treatment experience of 60 patients with localized extraskeletal Ewing sarcoma and found that combined-modality local therapy did not provide a local control advantage compared to single-modality local therapy. Single-modality local therapy may be sufficient for local control in select patients with favorable disease features.
Review
Cell Biology
Maryne Dupuy, Francois Lamoureux, Mathilde Mullard, Anais Postec, Laura Regnier, Marc Baud'huin, Steven Georges, Benedicte Brounais-Le Royer, Benjamin Ory, Francoise Redini, Franck Verrecchia
Summary: Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children, adolescents, and young adults in Europe, with a survival rate of 70% for localized forms using conventional treatment. However, resistance to chemotherapy and pulmonary metastases greatly reduce the survival rate. ES is characterized by a chromosomal translocation that leads to the fusion protein EWS-FLI1, which plays a crucial role in the development of ES. This review provides an overview of ES from a clinical and biological perspective.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Article
Biology
Elena Vasileva, Mikako Warren, Timothy J. Triche, James F. Amatruda
Summary: Ewing sarcoma is a malignant tumor that mainly affects children, adolescents, and young adults. The lack of a reliable genetic animal model has hindered the study of tumor cell and microenvironment interactions. By introducing human EWSR1-FLI1 gene into zebrafish, a new genetic model of Ewing sarcoma has been developed, which exhibits high penetrance and rapid tumor formation, providing a valuable tool for investigating the disease. Additionally, the study reveals the important role of the extracellular matrix in Ewing sarcoma tumor growth and suggests that targeting proteoglycan metabolism could be a potential novel therapy.
Article
Pediatrics
Chuanxi Zheng, Yong Zhou, Yi Luo, Hongying Zhang, Chongqi Tu, Li Min
Summary: A rare case of primary extra-skeletal Ewing sarcoma (EES) originating in the genitals was reported, resulting in the patient's death from sepsis and lung metastasis complications. Despite the rarity of EES, it can occur in any soft tissue site, highlighting the significance of early diagnosis and timely treatment for a favorable prognosis.
FRONTIERS IN PEDIATRICS
(2021)
Article
Oncology
Philip Heesen, Andreas Ranft, Vivek Bhadri, Benedicte Brichard, Stephane Collaud, Sona Cyprova, Hans Eich, Torben Ek, Hans Gelderblom, Jendrik Hardes, Lianne Haveman, Susanne Jabar, Wolfgang Hartmann, Dimosthenis Andreou, Peter Hauser, Josephine Kersting, Heribert Juergens, Jukka Kanerva, Thomas Kuehne, Anna Raciborska, Jelena Rascon, Arne Streitbuerger, Beate Timmermann, Yasmin Uhlenbruch, Uta Dirksen
Summary: This study analyzed the association between local therapy and event-free survival (EFS), overall survival (OS), and local recurrence (LR) in patients with Ewing sarcoma. The results showed that in certain situations, combination therapy with radiotherapy and surgery can improve the prognosis of patients. This provides important evidence for the personalized treatment of Ewing sarcoma.
EUROPEAN JOURNAL OF CANCER
(2023)
Review
Medicine, General & Internal
Stefan K. Zoellner, James E. Amatruda, Sebastian Bauer, Stephane Collaud, Enrique de Alava, Steven G. DuBois, Jendrik Hardes, Wolfgang Hartmann, Heinrich Kovar, Markus Metzler, David S. Shulman, Arne Streitbuerger, Beate Timmermann, Jeffrey A. Toretsky, Yasmin Uhlenbruch, Volker Vieth, Thomas G. P. Gruenewald, Uta Dirksen
Summary: Ewing sarcoma is a highly aggressive bone and soft-tissue cancer with a genetically simple yet specific therapeutic target. Current standard treatment involving systemic therapy and local treatment provides a realistic chance of cure for the majority of patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Oncology
Alexander Drilon, Vivek Subbiah, Oliver Gautschi, Pascale Tomasini, Filippo de Braud, Benjamin J. Solomon, Daniel Shao-Weng Tan, Guzman Alonso, Juergen Wolf, Keunchil Park, Koichi Goto, Victoria Soldatenkova, Sylwia Szymczak, Scott S. Barker, Tarun Puri, Aimee Bence Lin, Herbert Loong, Benjamin Besse
Summary: In this study, selpercatinib demonstrated durable and robust responses in both previously treated and treatment-naive patients with RET fusion-positive NSCLC, including intracranial activity.
JOURNAL OF CLINICAL ONCOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Vivek Subbiah
Summary: Technological advances are transforming evidence generation in medicine, but the clinical trial landscape must adapt to keep pace. Wearable technologies, data science, and machine learning offer a glimpse into the future of evidence-based medicine. Despite advances, clinical translations in major areas of medicine are lagging. The COVID-19 pandemic exposed limitations but also spurred positive changes like new trial designs and a shift towards a patient-centric evidence-generation system.
Review
Oncology
Blessie Elizabeth Nelson, Jacob J. Adashek, Steven H. Lin, Vivek Subbiah
Summary: With the development of immune-based therapies, the abscopal phenomenon has gained significant attention. Collaboration among radiation oncologists, oncologists and immunologists has increased, leading to exploration of various methods to induce the abscopal effect. However, further prospective studies and data analysis are needed to determine the appropriate candidates and cancer types.
Review
Oncology
Carmen Belli, Matteo Repetto, Santosh Anand, Camillo Porta, Vivek Subbiah, Giuseppe Curigliano
Summary: Phosphoinositide 3-kinases (PI3Ks) play a crucial role in tumorigenesis, particularly the p110 alpha subunit (PIK3CA) with activating mutations found in various tumors. Current PI3K inhibitors have limited efficacy due to on-target toxicity, inhibiting both wild-type and mutant PI3Ks. However, the development of mutant and isoform selective PIK3CA inhibitors has surged interest in targeting PIK3CA in solid tumors. This review summarizes the understanding of PI3K alterations, treatment strategies, and emerging resistance mechanisms and the role of mutant selective PIK3CA inhibitors.
BRITISH JOURNAL OF CANCER
(2023)
Editorial Material
Oncology
Gilberto Morgan, Vivek Subbiah
Summary: Social media platforms like Facebook and Twitter have transformed rare disease research, connecting patients with rare cancers. A recent study from the Germ Cell Tumor Survivor Sisters Facebook group shows the value of patient groups in supporting research and caring for people with the disease. These studies represent the beginning of empowered patient-driven research for rare diseases through social media.
Article
Oncology
Filip Janku, Milind M. Javle, Shiraj Sen, Shubham Pant, Lindsay G. Bramwell, Vivek Subbiah, Tracey Way, David S. Wages, Catherine A. Wheeler, Takeaki Suzuki, Kazunori Saeki, Ruth Ann Subach, Timothy Madden, Gary Maier, Mary J. Johansen, Kin Cheung, Gerald S. Falchook
Summary: FF-10502-01, a nucleoside similar to gemcitabine but with different effects, has shown promising activity in preclinical gemcitabine-resistant tumor models. In a first-in-human trial, FF-10502-01 was found to be well-tolerated with manageable side effects and demonstrated efficacy in patients with biliary tract cancer.
Editorial Material
Oncology
Aakash Desai, Neha K. Reddy, Vivek Subbiah
Summary: The advent of precision medicine has significantly impacted oncologic biomarker research, drug discovery, drug development, and patient outcomes. Precision oncology involves genomic profiling of tumors to identify actionable genetic abnormalities. Advances in clinical next-generation sequencing and the availability of targeted therapies have quickly been integrated into clinical practice.
Editorial Material
Oncology
Aakash Desai, Vivek Subbiah
Summary: Genome driven precision oncology has revolutionized the treatment of various cancers, but there are access barriers. A recent study demonstrated the effectiveness of a global outreach program via social media, providing free tumor genomic testing with a focus on rare cancers. For more details, refer to the article by Doe-Tetteh et al., p. 2445.
CLINICAL CANCER RESEARCH
(2023)
Article
Oncology
Vivek Subbiah, Sant P. Chawla, Anthony P. Conley, Breelyn A. Wilky, Anthony Tolcher, Nehal J. Lakhani, David Berz, Vasily Andrianov, William Crago, Monica Holcomb, Abrahim Hussain, Carson Veldstra, James Kalabus, Brianne 'Neill, Lane Senne, Emily Rowell, Analeah B. Heidt, Katelyn M. Willis, Brendan P. Eckelman
Summary: The study presented the preclinical characterization and clinical findings of INBRX-109 in patients with unresectable/metastatic chondrosarcoma, demonstrating its antitumor activity and safety profile.
CLINICAL CANCER RESEARCH
(2023)
Article
Oncology
Mohamed A. Gouda, Lars Buschhorn, Andreas Schneeweiss, Adam Wahida, Vivek Subbiah
Summary: The current approaches for cancer drug development are not meeting the accelerated need for a fast and efficient method of evaluating drugs in the personalized medicine era. N-of-1 studies offer a potential solution by shifting from a drug-centric to a patient-centric model, but several considerations need to be addressed before their widespread application. This article reviews the concept of N-of-1 trials and provides real-world examples of their use in developmental therapeutics. N-of-1 trials present an exceptional opportunity for expediting cancer drug development in the precision oncology era.
Article
Oncology
Blessie Elizabeth Nelson, Neha K. Reddy, Jason T. Huse, Behrang Amini, Mirella Nardo, Mohamed Gouda, Shiao-Pei Weathers, Vivek Subbiah
Summary: The identification of BRAF V600 mutation and the development of combined BRAF and MEK targeting agents have made significant impact on tissue-agnostic precision oncology therapies. However, resistance can occur and it is important to identify potential resistance mechanisms. We report a case of recurrent glioblastoma that initially responded to treatment but later developed resistance through histological transformation and acquisition of additional mutations.
NPJ PRECISION ONCOLOGY
(2023)
Article
Medicine, Research & Experimental
Blessie Elizabeth Nelson, Jacob J. Adashek, Steven H. Lin, Vivek Subbiah
Summary: With the emergence of immunotherapy, interest in the abscopal effect has been renewed. This phenomenon, although once considered elusive, is now increasingly reported. Further exploration using a multimodality approach and unconventional modalities is urgently needed.
Editorial Material
Oncology
Justin T. Moyers, Vivek Subbiah
Summary: Genome-driven precision oncology has significantly changed the approach to different types of cancer treatment by personalizing it based on individual's gene information. A recent study has confirmed the benefits of neurotrophic receptor tyrosine kinase (NTRK) inhibitors in treating sarcomas with NTRK fusions. This study further emphasizes the importance of genomics in identifying effective treatments for rare targets such as NTRK fusions.
Editorial Material
Oncology
Jie Wu, Vivek Subbiah
Summary: This study found that ESR1-TAF and ERα LBD mutants upregulated the expression of kinases such as FLT4, RET, JAK1, and IGF1R. Inhibition of RET with pralsetinib suppressed cell proliferation and xenograft growth in breast cancer. This research deepens our understanding of RET as an ESR1 target gene and suggests the potential use of pralsetinib to target wildtype RET in ER-positive breast cancer.
Review
Cell Biology
Vivek Subbiah, Srdan Verstovsek
Summary: This article summarizes the most common toxicities associated with FGFR inhibitors and provides practical advice for managing them. Hyperphosphatemia, ocular adverse events, dermatologic AEs, and gastrointestinal AEs can all be managed with appropriate measures and treatments.
CELL REPORTS MEDICINE
(2023)