Review
Oncology
Veronica Aran, Sylvie Devalle, Walter Meohas, Manoela Heringer, Anabela Cunha Caruso, Diego Pinheiro Aguiar, Maria Eugenia Leite Duarte, Vivaldo Moura Neto
Summary: Bone sarcomas, rare but deadly tumors, have diverse treatment approaches based on tumor staging. Despite no significant changes in clinical outcomes over the past 30 years, early identification of molecular biomarkers may improve disease prognosis.
CRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY
(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)
Article
Oncology
Santiago A. Lozano-Calderon, Jose Ignacio Albergo, Olivier Q. Groot, Nelson A. Merchan, Jad M. El Abiad, Vanessa Salinas, Luis Carlos Gomez Mier, Camilo Soto Montoya, Marco L. Ferrone, John E. Ready, Francisco J. Linares, Adam S. Levin, Manuel Peleteiro Pensado, Jose Juan Pozo Kreilinger, Irene Barrientos Ruiz, Eduardo J. Ortiz-Cruz, Mark C. Gebhardt, Gregory M. Cote, Edwin Choy, Dimitrios Spentzos, Yin P. Hung, Vikram Deshpande, Ivan A. Chebib, Robert Allan McCulloch, German Farfalli, Luis Aponte Tinao, Carol D. Morris, Gunnlaugur Petur Nielsen, Megan E. Anderson, Lee M. Jeys
Summary: Complete tumor necrosis induced by chemotherapy significantly improves overall survival, recurrence-free survival, metastasis-free survival, and event-free survival in patients with Ewing sarcoma, emphasizing the importance of achieving a complete response in neoadjuvant treatment.
Article
Medicine, General & Internal
Stefania Kokkali, Anastasios Kyriazoglou, Elpida Mangou, Panagiota Economopoulou, Michail Panousieris, Amanda Psyrri, Alexandros Ardavanis, Nikolaos Vassos, Ioannis Boukovinas
Summary: Retrospective analysis showed that cabozantinib has antitumor activity in adult patients with advanced bone sarcoma and ES/primitive neuroectodermal tumors, but no significant objective response rate was observed. Common adverse events in clinical practice included fatigue, anorexia, hypertransaminasemia, weight loss, and diarrhea.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Cell Biology
Tarek Assi, Sarah Watson, Bachar Samra, Elie Rassy, Axel Le Cesne, Antoine Italiano, Olivier Mir
Summary: Osteosarcoma is the most common primary tumor of the bones in young adults, with poor prognosis in relapsed or metastatic cases. However, advancements in targeted therapy, particularly focusing on angiogenesis, have shown promise in enhancing anti-tumor activity in osteosarcoma patients.
Article
Pharmacology & Pharmacy
Zunaira Shoaib, Timothy M. Fan, Joseph M. K. Irudayaraj
Summary: Osteosarcoma, a common primary bone tumor, is influenced by mechanobiology in its pathogenesis. Mechanobiology-driven therapies can curb tumor progression by altering the physical microenvironment or inhibiting proteins associated with metastasis.
BRITISH JOURNAL OF PHARMACOLOGY
(2022)
Review
Oncology
Valentina Evdokimova, Hendrik Gassmann, Laszlo Radvanyi, Stefan E. G. Burdach
Summary: This review discusses the progress and challenges of immunotherapy in pediatric sarcomas, focusing on Ewing sarcoma and osteosarcoma. It explores the mechanisms of immune escape and immunosuppression employed by these tumors, as well as potential novel directions for research and therapy.
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
Orthopedics
Susan Hesni, Daniel Lindsay, Paul O'Donnell, Asif Saifuddin
Summary: Extra-skeletal osteosarcoma is a rare soft tissue sarcoma that presents diagnostic challenges. This review outlines the demographics, clinical presentation, imaging features, differential diagnosis, management, and outcomes of the disease, serving as a reference for radiologists and clinicians involved in the care of patients with soft tissue tumors and tumor-like lesions.
SKELETAL RADIOLOGY
(2023)
Article
Multidisciplinary Sciences
Liyun Luo, Zhijie Zhang, Ni Qiu, Li Ling, Xiaoting Jia, Ying Song, Hongsheng Li, Jiansheng Li, Hui Lyu, Hao Liu, Zhimin He, Bolin Liu, Guopei Zheng
Summary: Resistance to Herceptin in HER2-positive breast cancer is a major challenge, with aberrant activation of the IGF2/IRS1 signaling pathway due to disruption of the negative feedback loop between FOXO3a and miRNAs. Detecting increased levels of IGF2 and IRS1 in patients with poor response to Herceptin-containing regimens provides insights for identifying predictive biomarkers and strategies to overcome resistance to Herceptin.
NATURE COMMUNICATIONS
(2021)
Article
Oncology
Akihito Nagano, Masanori Tsugita, Yutaka Nishimoto, Haruhiko Akiyama, Akira Kawai
Summary: In the study of patients with primary bone sarcomas other than osteosarcoma, Ewing sarcoma, and chondrosarcoma, it was found that surgery was an independent predictor of overall survival, while distant metastases at presentation were significant predictors of both overall and progression-free survival. There was no significant difference in outcomes between patients treated with surgery alone and those treated with surgery plus chemotherapy.
JAPANESE JOURNAL OF CLINICAL ONCOLOGY
(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.
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.
Review
Cell Biology
Manideep C. Pachva, Horton Lai, Andy Jia, Melanie Rouleau, Poul H. Sorensen
Summary: Ewing sarcoma is a highly aggressive cancer, with poor prognosis for patients with metastatic disease. Intercellular communication within the tumor microenvironment is crucial for cancer cells to establish immunosuppressive and cancer-permissive environments. Additional research on this communication mechanism may lead to new therapeutic strategies.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Oncology
Emmy D. G. Fleuren, Myrella Vlenterie, Winette T. A. van der Graaf
Summary: Osteosarcoma (OS) and Ewing sarcoma (ES) are two common types of primary bone cancer that mainly affect young people. Despite aggressive treatment, survival rates have not significantly improved in the past four decades. Clinical efficacy has been observed with some mono-Receptor Tyrosine Kinase (RTK) inhibitors, but only in small subsets of patients. Newer generation multi-RTK inhibitors have shown efficacy in larger groups of OS or ES patients. However, registration for these indications has not been obtained, making it difficult to implement these drugs in routine patient care.
FRONTIERS IN ONCOLOGY
(2023)
Article
Oncology
Richard Lock, Hernan Carol, John M. Maris, E. Anders Kolb, Richard Gorlick, C. Patrick Reynolds, Min H. Kang, Stephen T. Keir, Jianrong Wu, Andrei Purmal, Andrei Gudkov, Dias Kurmashev, Raushan T. Kurmasheva, Peter J. Houghton, Malcolm A. Smith
PEDIATRIC BLOOD & CANCER
(2017)
Article
Oncology
Raushan T. Kurmasheva, Richard Gorlick, E. Anders Kolb, Stephen T. Keir, John M. Maris, Richard B. Lock, Hernan Carol, Min Kang, C. Patrick Reynolds, Jianrong Wu, Peter J. Houghton, Malcolm A. Smith
PEDIATRIC BLOOD & CANCER
(2017)
Review
Oncology
Douglas J. Harrison, David S. Geller, Jonathan D. Gill, Valerae O. Lewis, Richard Gorlick
EXPERT REVIEW OF ANTICANCER THERAPY
(2018)
Article
Oncology
Roelof Koster, Orestis A. Panagiotou, William A. Wheeler, Eric Karlins, Julie M. Gastier-Foster, Silvia Regina Caminada de Toledo, Antonio S. Petrilli, Adrienne M. Flanagan, Roberto Tirabosco, Irene L. Andrulis, Jay S. Wunder, Nalan Gokgoz, Ana Patino-Garcia, Fernando Lecanda, Massimo Serra, Claudia Hattinger, Piero Picci, Katia Scotlandi, David M. Thomas, Mandy L. Ballinger, Richard Gorlick, Donald A. Barkauskas, Logan G. Spector, Margaret Tucker, D. Hicks Belynda, Meredith Yeager, Robert N. Hoover, Sholom Wacholder, Stephen J. Chanock, Sharon A. Savage, Lisa Mirabello
INTERNATIONAL JOURNAL OF CANCER
(2018)
Article
Oncology
Chong Zhu, Kristofor A. Olson, Michael Roth, David S. Geller, Richard G. Gorlick, Jonathan Gill, Nadia N. Laack, R. L. Randall
JOURNAL OF SURGICAL ONCOLOGY
(2018)
Article
Oncology
Eliana Shaul, Michael Roth, Yungtai Lo, David S. Geller, Bang Hoang, Rui Yang, David Malkin, Richard Gorlick, Jonathan Gill
Article
Multidisciplinary Sciences
Jason Glover, Tsz-Kwong Man, Donald A. Barkauskas, David Hall, Tanya Tello, Mary Beth Sullivan, Richard Gorlick, Katherine Janeway, Holcombe Grier, Ching Lau, Jeffrey A. Toretsky, Scott C. Borinstein, Chand Khanna, Timothy M. Fan
Review
Oncology
Jonathan Gill, Richard Gorlick
Summary: Osteosarcoma, the most common primary bone cancer in children and young adults, presents challenges in treatment due to its rarity and molecular heterogeneity. Collaborative efforts in biological discoveries, technologies, and therapeutic approaches are expected to advance osteosarcoma treatment and improve patient outcomes. The increasing feasibility of molecular profiling, along with the creation of robust model systems and large tissue banks, has enhanced understanding of osteosarcoma biology.
NATURE REVIEWS CLINICAL ONCOLOGY
(2021)
Article
Oncology
Anderson B. Collier, Mark D. Krailo, Ha M. Dang, Steven G. DuBois, Douglas S. Hawkins, Mark L. Bernstein, Lisa R. Bomgaars, Damon R. Reed, Richard G. Gorlick, Katherine A. Janeway
Summary: Analysis of seven Children's Oncology Group phase 2 trials for patients with relapsed/progressive solid tumors showed a 6-month event-free survival (EFS) of 12.7% for relapsed/progressive Ewing sarcoma patients, indicating poor outcomes. Despite docetaxel achieving its protocol-specified radiographic response rate, its EFS was similar to other agents, suggesting that a higher radiographic response rate may not lead to superior disease control. This EFS benchmark could be used as an additional endpoint in trials for recurrent Ewing sarcoma.
PEDIATRIC BLOOD & CANCER
(2021)
Article
Cell Biology
Xin Zhou, Allyson Beilter, Zhaohui Xu, Ruli Gao, Shunbin Xiong, Adriana Paulucci-Holthauzen, Guillermina Lozano, Benoit de Crombrugghe, Richard Gorlick
Summary: This study identified chondrocytes as an additional source for mesenchymal progenitor cells (MPCs) and showed that Wnt/ss-catenin pathway discretely regulates the development from chondrocytes to C-MPC and subsequently from C-MPC to osteoblasts. Furthermore, the study unveiled a previously unrecognized functional link between ss-catenin and p53, with p53 playing a negative role in MPC osteogenic differentiation induced by Wnt/ss-catenin signaling.
CELL DEATH & DISEASE
(2021)
Article
Oncology
Qing Yang, Jiemiao Hu, Zhiliang Jia, Qi Wang, Jing Wang, Long Hoang Dao, Wendong Zhang, Sheng Zhang, Xueqing Xia, Richard Gorlick, Shulin Li
Summary: This study proposes a new cell therapy for treating osteosarcoma. By introducing a specific protein into peripheral blood mononuclear cells, tumor growth can be effectively inhibited without observable toxicity.
CLINICAL CANCER RESEARCH
(2022)
Correction
Medicine, General & Internal
Hannah C. Beird, Stefan S. Bielack, Adrienne M. Flanagan, Jonathan Gill, Dominique Heymann, Katherine A. Janeway, J. Andrew Livingston, Ryan D. Roberts, Sandra J. Strauss, Richard Gorlick
NATURE REVIEWS DISEASE PRIMERS
(2022)
Article
Medicine, General & Internal
Hannah C. Beird, Stefan S. Bielack, Adrianne M. Flanagan, Jonathan Gill, Dominique Heymann, Katherine A. Janeway, J. Andrew Livingston, Ryan D. Roberts, Sandra J. Strauss, Richard Gorlick
Summary: Osteosarcoma is the most common primary malignant bone tumor with a bimodal incidence peaking at 18 and 60 years of age. It is more common in males and is driven by genetic factors related to bone formation leading to malignant progression and metastasis. Screening is currently focused on high-risk groups, and the prognosis for patients with metastatic disease remains poor.
NATURE REVIEWS DISEASE PRIMERS
(2022)
Article
Biochemistry & Molecular Biology
Jiayi Sun, Wing-Yuk Chow, Gufeng Xu, M. John Hicks, Manjula Nakka, Jianhe Shen, Patrick Kwok Shing M. Ng, Aaron Taylor, Alexander E. Yu, Jason A. Farrar, Donald Barkauskas, Richard M. Gorlick, Jaime M. Guidry Auvil, Daniela Gerhard, Paul Meltzer, Rudy Guerra, Tsz-Kwong C. Man, Ching Lau, TARGET Osteosarcoma Consortium
Summary: Osteosarcoma is a primary malignant bone tumor that commonly metastasizes to the lungs. Altered FAS expression due to DNA methylation has been explored as a mechanism in cancer cells. Analysis revealed high variability in methylation of CpG sites that correlated with FAS mRNA expression. Treatment with demethylating agent 5-azacytidine restored FAS protein expression and reduced metastasis in osteosarcoma cells.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Oncology
Lara E. Davis, Katherine A. Janeway, Aaron R. Weiss, Yen-Lin E. Chen, Thomas J. Scharschmidt, Mark Krailo, Julia L. Glade Bender, Lisa M. Kopp, Shreyaskumar R. Patel, Gary K. Schwartz, L. Elise Horvath, Douglas S. Hawkins, Meredith K. Chuk, Denise K. Reinke, Richard G. Gorlick, R. Lor Randall