Scholars@Duke
Corinne Mary Linardic

Corinne Mary Linardic

Associate Professor of Pediatrics
Pediatrics, Hematology-Oncology
linar001@mc.duke.edu
Box 102382, DUMC, Durham, NC 27710
LSRC Bldg Rm B361F, Research Drive, Durham, NC 27710

Selected Publications

Towards directed therapy for fusion-positive rhabdomyosarcoma.

Journal Article Pharmacol Ther · December 2025 Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. The fusion-positive variant of rhabdomyosarcoma has the dubious distinction of being one of the most difficult to cure childhood cancers. Although the gene fusions PAX3::FOXO1 and PAX7:: ... Full text Link to item Cite

Abstract A022-PR005: CDK8 Inhibition Releases the Muscle Differentiation Block in Fusion-Driven Alveolar Rhabdomyosarcoma

Conference Cancer Research · September 25, 2025 AbstractFunctional and chemical genomic approaches, such as the Broad Institute’s Cancer Dependency Map and PRISM (Profiling Relative Inhibition S ... Full text Cite

Prioritization of novel agents for further investigation in pediatric non-rhabdomyosarcoma soft tissue sarcomas: A report from the Children's Oncology Group.

Journal Article Eur J Cancer · August 26, 2025 Non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) is a group of over 70 tumors that occur across the age range and account for approximately 4 % of childhood cancers. Patients with metastatic or relapsed NRSTS have a dismal prognosis. The histologic, molecu ... Full text Link to item Cite

The impact of genetic ancestry on survival outcomes in pediatric rhabdomyosarcoma: A report from the Children's Oncology Group.

Journal Article HGG Adv · July 10, 2025 Emerging evidence suggests genetic ancestry may influence childhood cancer outcomes, but its impact on pediatric rhabdomyosarcoma (RMS) is unknown. We explored genetic ancestry's impact on survival among children with RMS. This multi-center observational c ... Full text Link to item Cite

Nonorbital, Nonparameningeal Head and Neck Rhabdomyosarcoma: A Report From the Children's Oncology Group.

Journal Article Pediatr Blood Cancer · June 2025 BACKGROUND: Rhabdomyosarcoma (RMS) is the most common pediatric head and neck soft-tissue sarcoma. Intergroup Rhabdomyosarcoma Study I-IV demonstrated that patients with alveolar RMS (ARMS), Group III disease, or clinically involved regional lymph nodes ha ... Full text Link to item Cite

Abstract 6707: Direct in vivo CRISPR screen identifies BAP1 and FAT1 as potent tumor suppressors in sarcomagenesis

Conference Cancer Research · April 21, 2025 AbstractUndifferentiated pleomorphic sarcoma (UPS) is among the most common soft tissue sarcomas (STS) in adults. For decades, little therapeutic progress has been made for STSs, including UPSs. Targeted the ... Full text Cite

Abstract 2810: Investigating the oncogenic function of the long noncoding RNA KCNQ1OT1 in rhabdomyosarcoma

Conference Cancer Research · April 21, 2025 AbstractRhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents, with survival for high-risk cases stagnant at less than 30%. Contemporary molecular classification of RMS is ... Full text Cite

Abstract A025: Direct in vivo CRISPR screen identifies BAP1 and FAT1 as potent tumor suppressors in sarcomagenesis

Conference Cancer Research · March 11, 2025 AbstractUndifferentiated pleomorphic sarcoma (UPS) is among the most common soft tissue sarcomas (STS) in adults. For decades, little therapeutic progress has been made for STSs, including UPSs. Targeted the ... Full text Cite

Roadmap for the next generation of Children's Oncology Group rhabdomyosarcoma trials.

Journal Article Cancer · November 15, 2024 Clinical trials conducted by the Intergroup Rhabdomyosarcoma (RMS) Study Group and the Children's Oncology Group have been pivotal to establishing current standards for diagnosis and therapy for RMS. Recent advancements in understanding the biology and cli ... Full text Link to item Cite

Spontaneous expression of the CIC::DUX4 fusion oncoprotein from a conditional allele potently drives sarcoma formation in genetically engineered mice.

Journal Article Oncogene · April 2024 CIC::DUX4 sarcoma (CDS) is a rare but highly aggressive undifferentiated small round cell sarcoma driven by a fusion between the tumor suppressor Capicua (CIC) and DUX4. Currently, there are no effective treatments and efforts to identify and translate bet ... Full text Link to item Cite

Abstract 1092: RUNX2 inhibition alters PAX3::FOXO1 driven fusion-positive rhabdomyosarcoma cell state and growth

Conference Cancer Research · March 22, 2024 AbstractInvestigation: Fusion-positive rhabdomyosarcoma (FP-RMS) is an aggressive pediatric cancer of skeletal muscle lineage. While the incidence of RMS is ~4.5 patients per million individuals aged &lt ... Full text Cite

Abstract 5960: Targeting CDK8: A translatable therapeutic approach for fusion-positive aRMS

Conference Cancer Research · March 22, 2024 AbstractAlveolar rhabdomyosarcoma (aRMS), characterized by poor overall survival and limited advancements in therapy over the past four decades, poses a great challenge in childhood cancer treatment. More th ... Full text Cite

Germline Genetic Testing and Survival Outcomes Among Children With Rhabdomyosarcoma: A Report From the Children's Oncology Group.

Journal Article JAMA Netw Open · March 4, 2024 IMPORTANCE: Determining the impact of germline cancer-predisposition variants (CPVs) on outcomes could inform novel approaches to testing and treating children with rhabdomyosarcoma. OBJECTIVE: To assess whether CPVs are associated with outcome among child ... Full text Link to item Cite

Expression of the CIC-DUX4 fusion oncoprotein mimics human CIC-rearranged sarcoma in genetically engineered mouse models.

Journal Article Res Sq · October 29, 2023 CIC-DUX4 sarcoma (CDS) is a rare but highly aggressive undifferentiated small round cell sarcoma driven by a fusion between the tumor suppressor Capicua (CIC) and DUX4. Currently, there are no effective treatments and efforts to identify and translate bett ... Full text Link to item Cite

Preclinical development of a chimeric antigen receptor T cell therapy targeting FGFR4 in rhabdomyosarcoma.

Journal Article Cell Rep Med · October 17, 2023 Pediatric patients with relapsed or refractory rhabdomyosarcoma (RMS) have dismal cure rates, and effective therapy is urgently needed. The oncogenic receptor tyrosine kinase fibroblast growth factor receptor 4 (FGFR4) is highly expressed in RMS and lowly ... Full text Link to item Cite

Expression of the CIC-DUX4 fusion oncoprotein mimics human CIC-rearranged sarcoma in genetically engineered mouse models.

Journal Article bioRxiv · September 28, 2023 CIC-DUX4 sarcoma (CDS) is a rare but highly aggressive undifferentiated small round cell sarcoma driven by a fusion between the tumor suppressor Capicua (CIC) and DUX4. Currently, there are no effective treatments and efforts to identify and translate bett ... Full text Link to item Cite

Children's Oncology Group's 2023 blueprint for research: Soft tissue sarcomas.

Journal Article Pediatr Blood Cancer · September 2023 In the United States, approximately 850-900 children and adolescents each year are diagnosed with soft tissue sarcomas (STS). STS are divided into rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma STS (NRSTS). RMS and NRSTS are risk stratified into low-, int ... Full text Link to item Cite

Abstract 3534: Interrogating the immune microenvironment of a novel mouse model of fusion positive rhabdomyosarcoma

Conference Cancer Research · April 4, 2023 AbstractImmunotherapies have been largely ineffective for pediatric soft tissue sarcomas, in particular for fusion-positive rhabdomyosarcoma (FP-RMS), characterized by the PAX-FOXO1 gene fusion. We are hampe ... Full text Cite

Abstract SY23-03: Rhabdomyosarcoma: New research tools for precision medicine

Conference Cancer Research · April 4, 2023 AbstractRhabdomyosarcoma (RMS) is a cancer of skeletal muscle histogenesis and the most common soft tissue sarcoma of childhood. Despite decades of basic and clinical research, survival for patients with hig ... Full text Cite

Predicting Molecular Subtype and Survival of Rhabdomyosarcoma Patients Using Deep Learning of H&E Images: A Report from the Children's Oncology Group.

Journal Article Clin Cancer Res · January 17, 2023 PURPOSE: Rhabdomyosarcoma (RMS) is an aggressive soft-tissue sarcoma, which primarily occurs in children and young adults. We previously reported specific genomic alterations in RMS, which strongly correlated with survival; however, predicting these mutati ... Full text Link to item Cite

Identification and targeting of a HES1-YAP1-CDKN1C functional interaction in fusion-negative rhabdomyosarcoma.

Journal Article Mol Oncol · October 2022 Rhabdomyosarcoma (RMS), a cancer characterized by features of skeletal muscle, is the most common soft-tissue sarcoma of childhood. With 5-year survival rates among high-risk groups at < 30%, new therapeutics are desperately needed. Previously, usin ... Full text Link to item Cite

Molecular testing of rhabdomyosarcoma in clinical trials to improve risk stratification and outcome: A consensus view from European paediatric Soft tissue sarcoma Study Group, Children's Oncology Group and Cooperative Weichteilsarkom-Studiengruppe.

Journal Article Eur J Cancer · September 2022 Rhabdomyosarcomas (RMSs) are the most common soft tissue sarcomas in children/adolescents less than 18 years of age with an annual incidence of 1-2/million. Inter/intra-tumour heterogeneity raise challenges in clinical, pathological and biological research ... Full text Link to item Cite

Clinical group and modified TNM stage for rhabdomyosarcoma: A review from the Children's Oncology Group.

Journal Article Pediatr Blood Cancer · June 2022 The Children's Oncology Group (COG) uses Clinical Group (CG) and modified Tumor Node Metastasis (TNM) stage to classify rhabdomyosarcoma (RMS). CG is based on surgicopathologic findings and is determined after the completion of initial surgical procedure(s ... Full text Link to item Cite

Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.

Journal Article J Clin Oncol · September 10, 2021 PURPOSE: Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. Despite aggressive therapy, the 5-year survival rate for patients with metastatic or recurrent disease remains poor, and beyond PAX-FOXO1 fusion status, no genomic markers are a ... Full text Link to item Cite

Expression of oncogenic HRAS in human Rh28 and RMS-YM rhabdomyosarcoma cells leads to oncogene-induced senescence.

Journal Article Sci Rep · August 13, 2021 Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. The two predominant histologic variants of RMS, embryonal and alveolar rhabdomyosarcoma (eRMS and aRMS, respectively), carry very different prognoses. While eRMS is associated with an ... Full text Link to item Cite

Epigenetic regulator BMI1 promotes alveolar rhabdomyosarcoma proliferation and constitutes a novel therapeutic target.

Journal Article Mol Oncol · August 2021 Rhabdomyosarcoma (RMS) is an aggressive pediatric soft tissue sarcoma. There are two main subtypes of RMS, alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma. ARMS typically encompasses fusion-positive rhabdomyosarcoma, which expresses either ... Full text Link to item Cite

Prioritization of Novel Agents for Patients with Rhabdomyosarcoma: A Report from the Children's Oncology Group (COG) New Agents for Rhabdomyosarcoma Task Force.

Journal Article J Clin Med · April 1, 2021 Rhabdomyosarcoma is the most common soft tissue sarcoma diagnosed in children and adolescents. Patients that are diagnosed with advanced or relapsed disease have exceptionally poor outcomes. The Children's Oncology Group (COG) convened a rhabdomyosarcoma n ... Full text Link to item Cite

Pathology of childhood rhabdomyosarcoma: A consensus opinion document from the Children's Oncology Group, European Paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

Journal Article Pediatr Blood Cancer · March 2021 The diagnosis and classification of rhabdomyosarcoma (RMS) has undergone several shifts over the last 30 years. While the main diagnostic categories remained the same, changes in the histologic criteria necessary for diagnosis, as well as varied reliance o ... Full text Link to item Cite

A method to culture human alveolar rhabdomyosarcoma cell lines as rhabdospheres demonstrates an enrichment in stemness and Notch signaling.

Journal Article Biol Open · February 9, 2021 The development of three-dimensional cell culture techniques has allowed cancer researchers to study the stemness properties of cancer cells in in vitro culture. However, a method to grow PAX3-FOXO1 fusion-positive rhabdomyosarcoma (FP-RMS), an aggressive ... Full text Link to item Cite

Experimental models

Chapter · January 1, 2021 To understand the pathophysiologic mechanisms of human bone and soft tissue sarcomas, and develop interventions to treat them, it became necessary to study sarcomas outside of the human body and deconstruct the events leading to full tumor formation. This ... Full text Cite

Abstract A08: Loss of noncanonical Hippo signaling in fusion-positive alveolar rhabdomyosarcoma increases invasiveness and a dedifferentiated phenotype associated with metastasis

Conference Molecular Cancer Research · August 1, 2020 AbstractA hallmark of alveolar rhabdomyosarcoma (ARMS) is the presence of a chromosomal translocation encoding the PAX3-FOXO1 fusion oncogene (FP-ARMS). Patients presenting with FP-ARMS represent the subset ... Full text Cite

Abstract A04: Genetic and pharmacologic inhibition of HES1 reduces YAP1 expression, impairing rhabdomyosarcoma cell growth

Conference Molecular Cancer Research · August 1, 2020 AbstractRhabdomyosarcoma (RMS) is a mesenchymal cancer with skeletal muscle histogenesis and the most common soft-tissue sarcoma of childhood. High-risk patient groups continue to have a poor survival (& ... Full text Cite

Abstract IA06: Dysregulated Hippo signaling in childhood rhabdomyosarcoma

Conference Molecular Cancer Research · August 1, 2020 AbstractRhabdomyosarcoma (RMS) is a mesenchymal cancer of skeletal muscle histogenesis and the most common soft tissue sarcoma of childhood. Survival for children with high-risk disease is less than 30% and ... Full text Cite

Response to Zhang et al.

Journal Article Genet Med · March 2020 Full text Link to item Cite

Detection of iron deficiency in children with Down syndrome.

Journal Article Genet Med · February 2020 PURPOSE: Current American Academy of Pediatrics guidelines for children with Down syndrome (DS) recommend a complete blood count (CBC) at birth and hemoglobin annually to screen for iron deficiency (ID) and ID anemia (IDA) in low-risk children. We aimed to ... Full text Link to item Cite

RASSF4 is required for skeletal muscle differentiation.

Journal Article Cell Biol Int · February 2020 RASSF4, a member of the classical RASSF family of scaffold proteins, is associated with alveolar rhabdomyosarcoma, an aggressive pediatric cancer of muscle histogenesis. However, the role of RASSF4 in normal myogenesis is unknown. We demonstrate here that ... Full text Link to item Cite

Dysregulated Hippo signaling in childhood rhabdomyosarcoma.

Conference MOLECULAR CANCER RESEARCH · 2020 Cite

A method to culture human alveolar rhabdomyosarcoma cell lines as rhabdospheres demonstrates an enrichment in stemness and notch signaling.

Journal Article Biol Open · January 1, 2020 The development of three-dimensional cell culture techniques has allowed cancer researchers to study the stemness properties of cancer cells in in vitro culture. However, a method to grow PAX3-FOXO1 fusion-positive rhabdomyosarcoma (FP-RMS) - an aggressive ... Full text Link to item Cite

Insights into pediatric rhabdomyosarcoma research: Challenges and goals.

Journal Article Pediatr Blood Cancer · October 2019 Overall survival rates for pediatric patients with high-risk or relapsed rhabdomyosarcoma (RMS) have not improved significantly since the 1980s. Recent studies have identified a number of targetable vulnerabilities in RMS, but these discoveries have infreq ... Full text Link to item Cite

Abstract 3670: Transcriptional co-activators TAZ/YAP are novel regulators of PAX3-FOXO1 transcriptional programing and fusion-positive rhabdomyosarcoma cancer cell stemness

Conference Cancer Research · July 1, 2019 AbstractIntroduction: Fusion Positive Rhabdomyosarcoma (FP-RMS), a soft tissue sarcoma of adolescents and young adults, is driven by the oncogenic transcription factor PAX3-FOXO1 (P3F). Although most pati ... Full text Cite

Relationship of DNA methylation to mutational changes and transcriptional organization in fusion-positive and fusion-negative rhabdomyosarcoma.

Journal Article Int J Cancer · June 1, 2019 Our previous study of DNA methylation in the pediatric soft tissue tumor rhabdomyosarcoma (RMS) demonstrated that fusion-positive (FP) and fusion-negative (FN) RMS tumors exhibit distinct DNA methylation patterns. To further examine the significance of DNA ... Full text Link to item Cite

Parenting a child with cancer: a couple-based approach.

Journal Article Transl Behav Med · May 16, 2019 Couples co-parenting a child with cancer face significant stressors that can adversely affect their couple relationship. How parents respond as a couple may affect the psychological adjustment of each parent and the child, as well as the ability of the fam ... Full text Link to item Cite

Proceedings of the fifth international RASopathies symposium: When development and cancer intersect.

Journal Article Am J Med Genet A · December 2018 This report summarizes and highlights the fifth International RASopathies Symposium: When Development and Cancer Intersect, held in Orlando, Florida in July 2017. The RASopathies comprise a recognizable pattern of malformation syndromes that are caused by ... Full text Link to item Cite

Loss of MST/Hippo Signaling in a Genetically Engineered Mouse Model of Fusion-Positive Rhabdomyosarcoma Accelerates Tumorigenesis.

Journal Article Cancer Res · October 1, 2018 A hallmark of fusion-positive alveolar rhabdomyosarcoma (aRMS) is the presence of a chromosomal translocation encoding the PAX3-FOXO1 fusion oncogene. Primary cell-based modeling experiments have shown that PAX3-FOXO1 is necessary, but not sufficient for a ... Full text Link to item Cite

The Transcriptional Coactivator TAZ Is a Potent Mediator of Alveolar Rhabdomyosarcoma Tumorigenesis.

Journal Article Clin Cancer Res · June 1, 2018 Purpose: Alveolar rhabdomyosarcoma (aRMS) is a childhood soft tissue sarcoma driven by the signature PAX3-FOXO1 (P3F) fusion gene. Five-year survival for aRMS is <50%, with no improvement in over 4 decades. Although the transcriptional coactivator TAZ is o ... Full text Link to item Cite

Soft Tissue Sarcoma Cancer Stem Cells: An Overview.

Journal Article Front Oncol · 2018 Soft tissue sarcomas (STSs) are an uncommon group of solid tumors that can arise throughout the human lifespan. Despite their commonality as non-bony cancers that develop from mesenchymal cell precursors, they are heterogeneous in their genetic profiles, h ... Full text Open Access Link to item Cite

A Novel Notch-YAP Circuit Drives Stemness and Tumorigenesis in Embryonal Rhabdomyosarcoma.

Journal Article Mol Cancer Res · December 2017 Rhabdomyosarcoma (RMS), a cancer characterized by skeletal muscle features, is the most common soft-tissue sarcoma of childhood. While low- and intermediate-risk groups have seen improved outcomes, high-risk patients still face a 5-year survival rate of <3 ... Full text Link to item Cite

Abstract 5830: A novel Notch-YAP circuit drives stemness and tumorigenesis in embryonal rhabdomyosarcoma

Conference Cancer Research · July 1, 2017 AbstractIntroduction: Rhabdomyosarcoma (RMS), a tumor characterized by skeletal muscle features, is the most common soft tissue sarcoma in children and adolescents. The standard of care treatment has not cha ... Full text Cite

The NOTCH1/SNAIL1/MEF2C Pathway Regulates Growth and Self-Renewal in Embryonal Rhabdomyosarcoma.

Journal Article Cell Rep · June 13, 2017 Tumor-propagating cells (TPCs) share self-renewal properties with normal stem cells and drive continued tumor growth. However, mechanisms regulating TPC self-renewal are largely unknown, especially in embryonal rhabdomyosarcoma (ERMS)-a common pediatric ca ... Full text Link to item Cite

Recent Insights into Notch Signaling in Embryonal Rhabdomyosarcoma.

Journal Article Curr Drug Targets · 2016 The Notch signaling pathway is an evolutionarily conserved developmental network critical for embryonic and postnatal regulation of tissue growth, homeostasis, and repair. Signaling is initiated when transmembrane Notch ligands bind to transmembrane Notch ... Full text Link to item Cite

Secreted Frizzled-Related Protein 3 (SFRP3) Is Required for Tumorigenesis of PAX3-FOXO1-Positive Alveolar Rhabdomyosarcoma.

Journal Article Clin Cancer Res · November 1, 2015 PURPOSE: Rhabdomyosarcoma (RMS) is a soft tissue sarcoma associated with the skeletal muscle lineage. Of the two predominant subtypes, known as embryonal (eRMS) and alveolar (aRMS), aRMS has the poorer prognosis, with a five-year survival rate of <50%. The ... Full text Link to item Cite

Histone Deacetylase Inhibitors Inhibit Rhabdomyosarcoma by Reactive Oxygen Species-Dependent Targeting of Specificity Protein Transcription Factors.

Journal Article Mol Cancer Ther · September 2015 The two major types of rhabdomyosarcoma (RMS) are predominantly diagnosed in children, namely embryonal (ERMS) and alveolar (ARMS) RMS, and patients are treated with cytotoxic drugs, which results in multiple toxic side effects later in life. Therefore, de ... Full text Link to item Cite

The third international meeting on genetic disorders in the RAS/MAPK pathway: towards a therapeutic approach.

Journal Article Am J Med Genet A · August 2015 "The Third International Meeting on Genetic Disorders in the RAS/MAPK Pathway: Towards a Therapeutic Approach" was held at the Renaissance Orlando at SeaWorld Hotel (August 2-4, 2013). Seventy-one physicians and scientists attended the meeting, and paralle ... Full text Link to item Cite

Role of the YAP Oncoprotein in Priming Ras-Driven Rhabdomyosarcoma.

Journal Article PLoS One · 2015 Rhabdomyosarcoma (RMS), a cancer characterized by features of skeletal muscle histogenesis, is the most common soft tissue sarcoma of childhood and adolescence. Survival for high-risk groups is less than 30% at 5 years. RMS also occurs during adulthood, wi ... Full text Link to item Cite

A Review: Molecular Aberrations within Hippo Signaling in Bone and Soft-Tissue Sarcomas.

Journal Article Front Oncol · 2015 The Hippo signaling pathway is an evolutionarily conserved developmental network vital for the regulation of organ size, tissue homeostasis, repair and regeneration, and cell fate. The Hippo pathway has also been shown to have tumor suppressor properties. ... Full text Open Access Link to item Cite

Rhabdomyosarcoma: current challenges and their implications for developing therapies.

Journal Article Cold Spring Harb Perspect Med · November 3, 2014 Rhabdomyosarcoma (RMS) represents a rare, heterogeneous group of mesodermal malignancies with skeletal muscle differentiation. One major subgroup of RMS tumors (so-called "fusion-positive" tumors) carries exclusive chromosomal translocations that join the ... Full text Link to item Cite

Alveolar rhabdomyosarcoma-associated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppression.

Journal Article J Clin Invest · January 2014 Alveolar rhabdomyosarcoma (aRMS) is an aggressive sarcoma of skeletal muscle characterized by expression of the paired box 3-forkhead box protein O1 (PAX3-FOXO1) fusion oncogene. Despite its discovery nearly two decades ago, the mechanisms by which PAX3-FO ... Full text Link to item Cite

Asparaginase-Induced Hypertriglyceridemia Presenting as Pseudohyponatremia during Leukemia Treatment.

Journal Article Case Rep Pediatr · 2014 Asparaginase is a chemotherapeutic agent used to induce disease remission in children with acute lymphoblastic leukemia (ALL). We describe the cases of two females with ALL who developed pseudohyponatremia as a presentation of hypertriglyceridemia followin ... Full text Link to item Cite

RAS and ROS in rhabdomyosarcoma.

Journal Article Cancer Cell · December 9, 2013 The 5-year survival for localized rhabdomyosarcoma is over 70%, but only 30% for patients presenting with metastatic disease. In this issue of Cancer Cell, Chen and colleagues performed whole-genome and RNA sequencing on human rhabdomyosarcoma and identifi ... Full text Link to item Cite

Distinct and overlapping sarcoma subtypes initiated from muscle stem and progenitor cells.

Journal Article Cell Rep · November 27, 2013 Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, whereas undifferentiated pleomorphic sarcoma (UPS) is one of the most common soft tissue sarcomas diagnosed in adults. To investigate the myogenic cell(s) of origin of these sarcoma ... Full text Link to item Cite

Inhibition of rhabdomyosarcoma cell and tumor growth by targeting specificity protein (Sp) transcription factors.

Journal Article Int J Cancer · February 15, 2013 Specificity protein (Sp) transcription factors Sp1, Sp3 and Sp4 are highly expressed in rhabdomyosarcoma (RMS) cells. In tissue arrays of RMS tumor cores from 71 patients, 80% of RMS patients expressed high levels of Sp1 protein, whereas low expression of ... Full text Link to item Cite

Human rhabdomyosarcoma cell lines for rhabdomyosarcoma research: utility and pitfalls.

Journal Article Front Oncol · 2013 Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence. Despite intergroup clinical trials conducted in Europe and North America, outcomes for high risk patients with this disease have not significantly improved in the l ... Full text Link to item Cite

FGFR4 blockade exerts distinct antitumorigenic effects in human embryonal versus alveolar rhabdomyosarcoma.

Journal Article Clin Cancer Res · July 15, 2012 Featured Publication PURPOSE: Rhabdomyosarcoma (RMS) is a malignancy with features of skeletal muscle, and the most common soft tissue sarcoma of childhood. Survival for high-risk groups is approximately 30% at 5 years and there are no durable therapies tailored to its genetic ... Full text Link to item Cite

In vivo imaging of tumor-propagating cells, regional tumor heterogeneity, and dynamic cell movements in embryonal rhabdomyosarcoma.

Journal Article Cancer Cell · May 15, 2012 Featured Publication Embryonal rhabdomyosarcoma (ERMS) is an aggressive pediatric sarcoma of muscle. Here, we show that ERMS-propagating potential is confined to myf5+ cells and can be visualized in live, fluorescent transgenic zebrafish. During early tumor growth, myf5+ ERMS ... Full text Link to item Cite

Embryonic signaling pathways and rhabdomyosarcoma: contributions to cancer development and opportunities for therapeutic targeting.

Journal Article Sarcoma · 2012 Featured Publication Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence, accounting for approximately 7% of childhood cancers. Current therapies include nonspecific cytotoxic chemotherapy regimens, radiation therapy, and surgery; however, thes ... Full text Link to item Cite

Inhibition of the Notch-Hey1 axis blocks embryonal rhabdomyosarcoma tumorigenesis.

Journal Article Clin Cancer Res · December 1, 2011 Featured Publication PURPOSE: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and remains refractory to combined-modality therapy in patients with high risk disease. In skeletal myogenesis, Notch signaling prevents muscle differentiation and promotes ... Full text Link to item Cite

Local therapy for rhabdomyosarcoma of the hands and feet: is amputation necessary? A report from the Children's Oncology Group.

Journal Article Int J Radiat Oncol Biol Phys · May 1, 2011 PURPOSE: To evaluate the outcome of children with rhabdomyosarcoma (RMS) of the hand or foot treated with surgery and/or local radiotherapy (RT). METHODS AND MATERIALS: Forty-eight patients with nonmetastatic RMS of the hand or foot were enrolled on Interg ... Full text Link to item Cite

Receptor tyrosine kinases as therapeutic targets in rhabdomyosarcoma.

Journal Article Sarcoma · 2011 Featured Publication Rhabdomyosarcomas (RMSs) are the most common soft tissue sarcomas of childhood and adolescence. To date, there are no effective treatments that target the genetic abnormalities in RMS, and current treatment options for high-risk groups are not adequate. Ov ... Full text Link to item Cite

Defining the cooperative genetic changes that temporally drive alveolar rhabdomyosarcoma.

Journal Article Cancer Res · December 1, 2008 Featured Publication Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence. Despite advances in therapy, patients with a histologic variant of RMS known as alveolar (aRMS) have a 5-year survival rate of <30%. aRMS tissues exhibit a number o ... Full text Link to item Cite

PAX3-FOXO1 fusion gene in rhabdomyosarcoma.

Journal Article Cancer Lett · October 18, 2008 Featured Publication Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence. The predominant histologic variants of this disease are termed embryonal (eRMS) and alveolar (aRMS), based on their appearance under light microscopy. Of the two, a ... Full text Link to item Cite

Genetic modeling of Ras-induced human rhabdomyosarcoma.

Journal Article Methods Enzymol · 2008 Featured Publication Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence. Historically, rhabdomyosarcoma has been studied by the manipulation of human cell lines derived from primary rhabdomyosarcoma tumor tissue adapted to grow in culture. Rec ... Full text Link to item Cite

The PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescence.

Journal Article Cancer Res · July 15, 2007 Featured Publication Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence. Despite advances in therapy, patients with a histologic variant of rhabdomyosarcoma known as alveolar rhabdomyosarcoma (ARMS) have a 5-year survival of <30%. ARMS is char ... Full text Link to item Cite

Genetic modeling of alveolar rhabdomyosarcoma

Journal Article PEDIATRIC BLOOD & CANCER · June 1, 2007 Link to item Cite

A network of genetic events sufficient to convert normal human cells to a tumorigenic state.

Journal Article Cancer Res · November 1, 2005 Featured Publication Although great progress has been made at identifying and characterizing individual genes involved in cancer, less is known about how the combination of such genes collaborate to form tumors in humans. To this end, we sought to genetically recreate tumorige ... Full text Link to item Cite

Genetic modeling of human rhabdomyosarcoma.

Journal Article Cancer Res · June 1, 2005 Featured Publication Rhabdomyosarcoma, a malignancy showing features of skeletal muscle differentiation, is the most common soft tissue sarcoma of childhood. The identification of distinct clinical presentation patterns, histologic tumor types, and risk groups suggests that rh ... Full text Link to item Cite

Rescue of an hTERT mutant defective in telomere elongation by fusion with hPot1.

Journal Article Mol Cell Biol · April 2004 The protein hPot1 shares homology with telomere-binding proteins in lower eukaryotes and associates with single-stranded telomeric DNA in vitro as well as colocalizing with telomere-binding proteins in vivo. We now show that hPot1 is coimmunoprecipitated w ... Full text Link to item Cite

Differential effects of rapamycin on mammalian target of rapamycin signaling functions in mammalian cells.

Journal Article Cancer Res · December 1, 2003 Rapamycin and its analogues have shown promising anticancer activities in preclinical and clinical studies. However, the mechanism whereby rapamycin inhibits signaling through the mammalian target of rapamycin (mTOR) remains poorly understood. Here, we sho ... Link to item Cite

Characterization of the cytotoxic activities of novel analogues of the antitumor agent, lavendamycin.

Journal Article Mol Cancer Ther · June 2003 Lavendamycin is a bacterially derived quinolinedione that displays significant antimicrobial and antitumor activities. However, preclinical development of lavendamycin as an anticancer agent was halted due to the poor aqueous solubility and relatively nons ... Link to item Cite

Acquired hypoprothrombinemia: effects of danazol treatment.

Journal Article Am J Hematol · December 1996 The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagu ... Full text Link to item Cite

Activation of the sphingomyelin cycle by brefeldin A: effects of brefeldin A on differentiation and implications for a role for ceramide in regulation of protein trafficking.

Journal Article Cell Growth Differ · June 1996 The sphingomyelin (SM) cycle is an emerging pathway of signal transduction that plays a role in the control of cell growth, cell differentiation, and apoptosis. During earlier investigation of SM pools hydrolyzed in the SM cycle, we examined the effects of ... Link to item Cite

Identification of a distinct pool of sphingomyelin involved in the sphingomyelin cycle.

Journal Article J Biol Chem · September 23, 1994 Sphingomyelin (SM) is a membrane phosphosphingolipid that has recently been identified as a key component of the SM cycle. In this signal transduction pathway, extracellular inducers such as tumor necrosis factor alpha cause hydrolysis of membrane SM, resu ... Link to item Cite

Identification of arachidonic acid as a mediator of sphingomyelin hydrolysis in response to tumor necrosis factor alpha.

Journal Article J Biol Chem · February 25, 1994 A sphingomyelin (SM)-signaling cycle has been described in human leukemia-derived HL-60 cells (Okazaki, T., Bell, R.M., and Hannun, Y.A. (1989) J. Biol. Chem. 264, 19076-19080). Activation of the cycle by tumor necrosis factor alpha (TNF alpha) occurs rapi ... Link to item Cite

Sphingolipid breakdown products: anti-proliferative and tumor-suppressor lipids.

Journal Article Biochim Biophys Acta · December 21, 1993 The sphingolipids are a family of lipids found ubiquitously in eukaryotic cell membranes. Within the last decade sphingolipids have emerged as active participants in the regulation of cell growth, differentiation, transformation, and cell-cell contact. A p ... Full text Link to item Cite

PROGRAMMED CELL-DEATH IS MEDIATED BY CERAMIDE

Journal Article CLINICAL RESEARCH · April 1, 1993 Link to item Cite

Programmed cell death induced by ceramide.

Journal Article Science · March 19, 1993 Sphingomyelin hydrolysis and ceramide generation have been implicated in a signal transduction pathway that mediates the effects of tumor necrosis factor-alpha (TNF-alpha) and other agents on cell growth and differentiation. In many leukemic cells, TNF-alp ... Full text Link to item Cite

Ceramide-mediated biology. Determination of structural and stereospecific requirements through the use of N-acyl-phenylaminoalcohol analogs.

Journal Article J Biol Chem · September 15, 1992 Ceramide is a postulated intracellular modulator of cell growth and differentiation (Okazaki, T., Bielawska, A., Bell, R.M., and Hannun, Y. A. (1990) J. Biol. Chem. 265, 15823-15831). In order to determine the structural and stereospecific requirements for ... Link to item Cite

Modulation of cell growth and differentiation by ceramide.

Journal Article FEBS Lett · July 28, 1992 Ceramide has been suggested as an intracellular modulator of cell growth and differentiation [Okazaki, T. et al. (1990) J. Biol. Chem. 265, 15823-15831]. In this study, parameters that modulate the effects of ceramide on HL-60 cell growth and differentiati ... Full text Link to item Cite

Brefeldin A promotes hydrolysis of sphingomyelin.

Journal Article J Biol Chem · July 25, 1992 The hydrolysis of sphingomyelin (SM) is a key reaction in the "sphingomyelin cycle," which plays a role in the regulation of cell proliferation and differentiation (Okazaki, T., Bell, R. M., and Hannun, Y. A. (1989) J. Biol. Chem. 264, 19076-19080). SM is ... Link to item Cite

Identification of sphingomyelin turnover as an effector mechanism for the action of tumor necrosis factor alpha and gamma-interferon. Specific role in cell differentiation.

Journal Article J Biol Chem · January 5, 1991 The biochemical signaling mechanisms involved in transducing the effects of tumor necrosis factor alpha (TNF alpha) and gamma-interferon (gamma-IFN) on leukemia cell differentiation are poorly defined. Recent studies established the existence of a sphingom ... Link to item Cite

Inhibition of the Anticoagulant Properties of Antithrombin III and Heparin by Fibrin

Journal Article Annals of the New York Academy of Sciences · January 1, 1989 Full text Cite

INHIBITION OF THE ANTICOAGULANT PROPERTIES OF ANTITHROMBIN-III AND HEPARIN BY FIBRIN

Journal Article ANNALS OF THE NEW YORK ACADEMY OF SCIENCES-SERIES · 1989 Cite