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Cancers
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Ewing Sarcoma
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Ewing Sarcoma

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: closed (31 March 2020) | Viewed by 38832

Special Issue Editors

Prof. Dr. Kazutaka Kikuta

E-Mail Website
Guest Editor
Department of Musculoskeletal Oncology and Orthopaedic Surgery, Tochigi Cancer Center, Tochigi 320-0834, Japan
Interests: sarcoma; rare cancer; biobank; patient derived cancer model
Special Issues, Collections and Topics in MDPI journals
Prof. Tadashi Kondo

E-Mail Website
Guest Editor
Division of Pharmacoproteomics, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan
Interests: rare cancer research; sarcoma; proteogenomics; applications of patient-derived cancer model
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Ewing sarcoma is the most common sarcoma from childhood to adolescence. Treatment methods that have shown improvement in clinical outcomes of Ewing sarcoma include advanced chemotherapy, as well as multidisciplinary treatment that combines surgery and radiotherapy. Ewing sarcoma is classified into localized and metastatic types. While outcomes are good for patients with localized Ewing sarcoma, prognosis is poor for patients with metastatic Ewing sarcoma. Moreover, it remains challenging to optimize therapies for patients who have metastatic Ewing sarcoma.

Ewing sarcoma results from simple sarcoma-specific genetic alterations that produce TET/FET and ETS family member fusion proteins. Genomic analyses have been reported for Ewing sarcoma using whole exon sequencing; wide genome sequencing to identify drug targets has also been reported. The results have shown that Ewing sarcoma exhibits a low incidence of single nucleotide variation and that the frequency of mutations in the STAG2 gene, which exhibits the most frequent mutations other than ETS family member fusion genes, is only 8–17%. The frequency of p53 mutations in Ewing sarcoma is ≤10%, and many of the mutations are detected in tissue after treatment; this suggests that, in contrast to osteosarcoma, p53 mutations are extremely unlikely to be initial events in Ewing sarcoma. Clinically, overlapping STAG2 and TP53 mutations are associated with poor prognosis in patients with Ewing sarcoma. However, genome analysis studies have not revealed any factors that could serve as targets for new treatment for Ewing sarcoma, as it appears to be driven entirely by a fusion gene.

Multiple studies have been published regarding the fusion oncoprotein and the products of other genetic alterations that significantly impact Ewing sarcoma tumorigenesis; however, they are not yet utilized in clinical practice. This Special Issue, “Ewing Sarcoma” will highlight the diverse challenges encountered in understanding the pathogenesis of Ewing sarcoma, as well as in its clinical management. Invited articles will cover a wide range of topics, including but not restricted to the following: (1) update on the clinical management of Ewing sarcoma, focusing on therapies for patients with metastases; (2) applications of biomarkers related to therapeutic targets for patients with metastases; (3) update on the molecular tumorigenesis mechanism related to metastasis of Ewing sarcoma; and (4) role of personalized genomics in identifying therapeutic targets in metastatic Ewing sarcoma.

Prof. Dr. Kazutaka Kikuta
Prof. Tadashi Kondo
Guest Editors

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Keywords

  • Ewing Sarcoma
  • Fusion genes
  • Clinical management
  • Biomarkers
  • Molecular metastatic mechanism
  • Personalized genomics

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Published Papers (9 papers)

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Research

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15 pages, 904 KiB  
Article
Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: The MD Anderson Cancer Center Experience
by Nidale Tarek, Rabih Said, Clark R. Andersen, Tina S. Suki, Jessica Foglesong, Cynthia E. Herzog, Nizar M. Tannir, Shreyaskumar Patel, Ravin Ratan, Joseph A. Ludwig and Najat C. Daw
Cancers 2020, 12(10), 2927; https://doi.org/10.3390/cancers12102927 - 11 Oct 2020
Cited by 15 | Viewed by 3031
Abstract
Limited information exists on the clinical behavior of the Ewing sarcoma family of tumors (ESFT) of the kidney. We reviewed the records of 30 patients (aged 8–69 years) with ESFT of the kidney seen at our institution between 1990 and 2013. We analyzed [...] Read more.
Limited information exists on the clinical behavior of the Ewing sarcoma family of tumors (ESFT) of the kidney. We reviewed the records of 30 patients (aged 8–69 years) with ESFT of the kidney seen at our institution between 1990 and 2013. We analyzed the event-free survival (EFS) and overall survival (OS) for associations with patient demographics, disease group, tumor size, tumor thrombus, and treatment. Six patients (20%) had tumors confined to the kidney (Group I), seven (23.3%) had local tumor extension beyond the kidney (Group II), and 17 (56.7%) had distant metastasis at diagnosis (Group III). Twenty-five (83.3%) patients underwent radical (19 upfront, five delayed) or partial (one upfront) nephrectomy, 25 (83.3%) chemotherapy and four (13.3%) radiotherapy. The 4-year EFS and OS were 43% (95% CI, 26–61%) and 63% (95% CI, 46–81%), respectively. EFS and OS were significantly associated with disease group and chemotherapy (p < 0.039). The presence of tumor thrombus in renal vein and/or inferior vena cava was associated with worse EFS (p = 0.053). Patients with disease confined to the kidney treated with nephrectomy and adjuvant chemotherapy have favorable outcomes. Local tumor extension beyond the kidney, tumor thrombus, and distant metastasis are unfavorable factors that warrant intensification or novel approaches of therapy. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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18 pages, 1254 KiB  
Article
IGF-1R/mTOR Targeted Therapy for Ewing Sarcoma: A Meta-Analysis of Five IGF-1R-Related Trials Matched to Proteomic and Radiologic Predictive Biomarkers
by Hesham M. Amin, Ajaykumar C. Morani, Najat C. Daw, Salah-Eddine Lamhamedi-Cherradi, Vivek Subbiah, Brian A. Menegaz, Deeksha Vishwamitra, Ghazaleh Eskandari, Bhawana George, Robert S. Benjamin, Shreyaskumar Patel, Juhee Song, Alexander J. Lazar, Wei-Lien Wang, Razelle Kurzrock, Alberto Pappo, Peter M. Anderson, Gary K. Schwartz, Dejka Araujo, Branko Cuglievan, Ravin Ratan, David McCall, Sana Mohiuddin, John A. Livingston, Eric R. Molina, Aung Naing and Joseph A. Ludwigadd Show full author list remove Hide full author list
Cancers 2020, 12(7), 1768; https://doi.org/10.3390/cancers12071768 - 2 Jul 2020
Cited by 22 | Viewed by 4204
Abstract
Background : Ten to fourteen percent of Ewing sarcoma (ES) study participants treated nationwide with IGF-1 receptor (IGF-1R)-targeted antibodies achieved tumor regression. Despite this success, low response rates and short response durations (approximately 7-weeks) have slowed the development of this therapy. Methods: [...] Read more.
Background : Ten to fourteen percent of Ewing sarcoma (ES) study participants treated nationwide with IGF-1 receptor (IGF-1R)-targeted antibodies achieved tumor regression. Despite this success, low response rates and short response durations (approximately 7-weeks) have slowed the development of this therapy. Methods: We performed a meta-analysis of five phase-1b/2 ES-oriented trials that evaluated the anticancer activity of IGF-1R antibodies +/− mTOR inhibitors (mTORi). Our meta-analysis provided a head-to-head comparison of the clinical benefits of IGF-1R antibodies vs. the IGF-1R/mTOR-targeted combination. Available pretreatment clinical samples were semi-quantitatively scored using immunohistochemistry to detect proteins in the IGF-1R/PI3K/AKT/mTOR pathway linked to clinical response. Early PET/CT imaging, obtained within the first 2 weeks (median 10 days), were examined to determine if reduced FDG avidity was predictive of progression-free survival (PFS). Results: Among 56 ES patients treated at MD Anderson Cancer Center (MDACC) with IGF-1R antibodies, our analysis revealed a significant ~two-fold improvement in PFS that favored a combination of IGF-1R/mTORi therapy (1.6 vs. 3.3-months, p = 0.042). Low pIGF-1R in the pretreatment specimens was associated with treatment response. Reduced total-lesion glycolysis more accurately predicted the IGF-1R response than other previously reported radiological biomarkers. Conclusion: Synergistic drug combinations, and newly identified proteomic or radiological biomarkers of IGF-1R response, may be incorporated into future IGF-1R-related trials to improve the response rate in ES patients. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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19 pages, 7688 KiB  
Article
Reconstruction of Ewing Sarcoma Developmental Context from Mass-Scale Transcriptomics Reveals Characteristics of EWSR1-FLI1 Permissibility
by Henry E. Miller, Aparna Gorthi, Nicklas Bassani, Liesl A. Lawrence, Brian S. Iskra and Alexander J. R. Bishop
Cancers 2020, 12(4), 948; https://doi.org/10.3390/cancers12040948 - 11 Apr 2020
Cited by 19 | Viewed by 8071
Abstract
Ewing sarcoma is an aggressive pediatric cancer of enigmatic cellular origins typically resulting from a single translocation event t (11; 22) (q24; q12). The resulting fusion gene, EWSR1-FLI1, is toxic or unstable in most primary tissues. Consequently, attempts to model Ewing sarcomagenesis [...] Read more.
Ewing sarcoma is an aggressive pediatric cancer of enigmatic cellular origins typically resulting from a single translocation event t (11; 22) (q24; q12). The resulting fusion gene, EWSR1-FLI1, is toxic or unstable in most primary tissues. Consequently, attempts to model Ewing sarcomagenesis have proven unsuccessful thus far, highlighting the need to identify the cellular features which permit stable EWSR1-FLI1 expression. By re-analyzing publicly available RNA-Sequencing data with manifold learning techniques, we uncovered a group of Ewing-like tissues belonging to a developmental trajectory between pluripotent, neuroectodermal, and mesodermal cell states. Furthermore, we demonstrated that EWSR1-FLI1 expression levels control the activation of these developmental trajectories within Ewing sarcoma cells. Subsequent analysis and experimental validation demonstrated that the capability to resolve R-loops and mitigate replication stress are probable prerequisites for stable EWSR1-FLI1 expression in primary tissues. Taken together, our results demonstrate how EWSR1-FLI1 hijacks developmental gene programs and advances our understanding of Ewing sarcomagenesis. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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18 pages, 3830 KiB  
Article
The Receptor Tyrosine Kinase RON and Its Isoforms as Therapeutic Targets in Ewing Sarcoma
by Philipp Berning, Carolin Hennemann, Claudia Tulotta, Christiane Schaefer, Birgit Lechtape, Marc Hotfilder, Yassmine El Gourari, Heribert Jürgens, Ewa Snaar-Jagalska, Georg Hempel, Uta Dirksen and Jenny Potratz
Cancers 2020, 12(4), 904; https://doi.org/10.3390/cancers12040904 - 7 Apr 2020
Cited by 3 | Viewed by 3507
Abstract
The receptor tyrosine kinase (RTK) RON is linked to an aggressive metastatic phenotype of carcinomas. While gaining interest as a therapeutic target, RON remains unstudied in sarcomas. In Ewing sarcoma, we identified RON among RTKs conferring resistance to insulin-like growth factor-1 receptor (IGF1R) [...] Read more.
The receptor tyrosine kinase (RTK) RON is linked to an aggressive metastatic phenotype of carcinomas. While gaining interest as a therapeutic target, RON remains unstudied in sarcomas. In Ewing sarcoma, we identified RON among RTKs conferring resistance to insulin-like growth factor-1 receptor (IGF1R) targeting. Therefore, we explored RON in pediatric sarcoma cell lines and an embryonic Tg(kdrl:mCherry) zebrafish model, using an shRNA-based approach. To examine RON–IGF1R crosstalk, we employed the clinical-grade monoclonal antibody IMC-RON8, alone and together with the IGF1R-antibody IMC-A12. RON silencing demonstrated functions in vitro and in vivo, particularly within micrometastatic cellular capacities. Signaling studies revealed a unidirectional IGF1-mediated cross-activation of RON. Yet, IMC-A12 failed to sensitize cells to IMC-RON8, suggesting additional mechanisms of RON activation. Here, RT-PCR revealed that childhood sarcomas express short-form RON, an isoform resistant to antibody-mediated targeting. Interestingly, in contrast to carcinomas, treatment with DNA methyltransferase inhibitor did not diminish but increased short-form RON expression. Thus, this first report supports a role for RON in the metastatic progression of Ewing sarcoma. While principal molecular functions appear transferrable between carcinomas, Ewing sarcoma and possibly more common sarcoma subtypes, RON highlights that specific regulations of cellular networks and isoforms require better understanding to successfully transfer targeting strategies. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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17 pages, 3582 KiB  
Article
High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma
by Martin F. Orth, Tilman L.B. Hölting, Marlene Dallmayer, Fabienne S. Wehweck, Tanja Paul, Julian Musa, Michaela C. Baldauf, Didier Surdez, Olivier Delattre, Maximilian M. L. Knott, Laura Romero-Pérez, Merve Kasan, Florencia Cidre-Aranaz, Julia S. Gerke, Shunya Ohmura, Jing Li, Aruna Marchetto, Anton G. Henssen, Özlem Özen, Shintaro Sugita, Tadashi Hasegawa, Takayuki Kanaseki, Stefanie Bertram, Uta Dirksen, Wolfgang Hartmann, Thomas Kirchner and Thomas G.P. Grünewaldadd Show full author list remove Hide full author list
Cancers 2020, 12(3), 644; https://doi.org/10.3390/cancers12030644 - 10 Mar 2020
Cited by 16 | Viewed by 4633
Abstract
Ewing sarcoma (EwS) is an aggressive cancer displaying an undifferentiated small-round-cell histomorphology that can be easily confused with a broad spectrum of differential diagnoses. Using comparative transcriptomics and immunohistochemistry (IHC), we previously identified BCL11B and GLG1 as potential specific auxiliary IHC markers for [...] Read more.
Ewing sarcoma (EwS) is an aggressive cancer displaying an undifferentiated small-round-cell histomorphology that can be easily confused with a broad spectrum of differential diagnoses. Using comparative transcriptomics and immunohistochemistry (IHC), we previously identified BCL11B and GLG1 as potential specific auxiliary IHC markers for EWSR1-FLI1-positive EwS. Herein, we aimed at validating the specificity of both markers in a far larger and independent cohort of EwS (including EWSR1-ERG-positive cases) and differential diagnoses. Furthermore, we evaluated their intra-tumoral expression heterogeneity. Thus, we stained tissue microarrays from 133 molecularly confirmed EwS cases and 320 samples from morphological mimics, as well as a series of patient-derived xenograft (PDX) models for BCL11B, GLG1, and CD99, and systematically assessed the immunoreactivity and optimal cut-offs for each marker. These analyses demonstrated that high BCL11B and/or GLG1 immunoreactivity in CD99-positive cases had a specificity of 97.5% and an accuracy of 87.4% for diagnosing EwS solely by IHC, and that the markers were expressed by EWSR1-ERG-positive EwS. Only little intra-tumoral heterogeneity in immunoreactivity was observed for differential diagnoses. These results indicate that BCL11B and GLG1 may help as specific auxiliary IHC markers in diagnosing EwS in conjunction with CD99, especially if confirmatory molecular diagnostics are not available. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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17 pages, 3428 KiB  
Article
Combined Inhibition of Epigenetic Readers and Transcription Initiation Targets the EWS-ETS Transcriptional Program in Ewing Sarcoma
by Günther H.S. Richter, Tim Hensel, Oxana Schmidt, Vadim Saratov, Kristina von Heyking, Fiona Becker-Dettling, Carolin Prexler, Hsi-Yu Yen, Katja Steiger, Simone Fulda, Uta Dirksen, Wilko Weichert, Shudong Wang, Stefan Burdach and Beat W. Schäfer
Cancers 2020, 12(2), 304; https://doi.org/10.3390/cancers12020304 - 28 Jan 2020
Cited by 14 | Viewed by 4526
Abstract
Background: Previously, we used inhibitors blocking BET bromodomain binding proteins (BRDs) in Ewing sarcoma (EwS) and observed that long term treatment resulted in the development of resistance. Here, we analyze the possible interaction of BRD4 with cyclin-dependent kinase (CDK) 9. Methods: Co-immunoprecipitation experiments [...] Read more.
Background: Previously, we used inhibitors blocking BET bromodomain binding proteins (BRDs) in Ewing sarcoma (EwS) and observed that long term treatment resulted in the development of resistance. Here, we analyze the possible interaction of BRD4 with cyclin-dependent kinase (CDK) 9. Methods: Co-immunoprecipitation experiments (CoIP) to characterize BRD4 interaction and functional consequences of inhibiting transcriptional elongation were assessed using drugs targeting of BRD4 or CDK9, either alone or in combination. Results: CoIP revealed an interaction of BRD4 with EWS-FLI1 and CDK9 in EwS. Treatment of EwS cells with CDKI-73, a specific CDK9 inhibitor (CDK9i), induced a rapid downregulation of EWS-FLI1 expression and block of contact-dependent growth. CDKI-73 induced apoptosis in EwS, as depicted by cleavage of Caspase 7 (CASP7), PARP and increased CASP3 activity, similar to JQ1. Microarray analysis following CDKI-73 treatment uncovered a transcriptional program that was only partially comparable to BRD inhibition. Strikingly, combined treatment of EwS with BRD- and CDK9-inhibitors re-sensitized cells, and was overall more effective than individual drugs not only in vitro but also in a preclinical mouse model in vivo. Conclusion: Treatment with BRD inhibitors in combination with CDK9i offers a new treatment option that significantly blocks the pathognomonic EWS-ETS transcriptional program and malignant phenotype of EwS. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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13 pages, 6099 KiB  
Article
Sensitive and Specific Detection of Ewing Sarcoma Minimal Residual Disease in Ovarian and Testicular Tissues in an In Vitro Model
by Laure Chaput, Victoria Grèze, Pascale Halle, Nina Radosevic-Robin, Bruno Pereira, Lauren Véronèse, Hervé Lejeune, Philippe Durand, Guillaume Martin, Sandra Sanfilippo, Michel Canis, Justyna Kanold, Andrei Tchirkov and Florence Brugnon
Cancers 2019, 11(11), 1807; https://doi.org/10.3390/cancers11111807 - 17 Nov 2019
Cited by 4 | Viewed by 2886
Abstract
Ewing sarcoma (EWS) is a common pediatric solid tumor with high metastatic potential. Due to toxic effects of treatments on reproductive functions, the cryopreservation of ovarian tissue (OT) or testicular tissue (TT) is recommended to preserve fertility. However, the risk of reintroducing residual [...] Read more.
Ewing sarcoma (EWS) is a common pediatric solid tumor with high metastatic potential. Due to toxic effects of treatments on reproductive functions, the cryopreservation of ovarian tissue (OT) or testicular tissue (TT) is recommended to preserve fertility. However, the risk of reintroducing residual metastatic tumor cells should be evaluated before fertility restoration. Our goal was to validate a sensitive and specific approach for EWS minimal residual disease (MRD) detection in frozen germinal tissues. Thawed OT (n = 12) and TT (n = 14) were contaminated with tumor RD-ES cells (10, 100, and 1000 cells) and EWS-FLI1 tumor-specific transcript was quantified with RT-qPCR. All contaminated samples were found to be positive, with a strong correlation between RD-ES cell numbers and EWS-FLI1 levels in OT (r = 0.93) and TT (r = 0.96) (p < 0.001). No transcript was detected in uncontaminated control samples. The invasive potential of Ewing cells was evaluated using co-culture techniques. After co-culturing, tumor cells were detected in OT/TT with histology, FISH, and RT-qPCR. In addition, four OT and four TT samples from children with metastatic EWS were tested, and no MRD was found using RT-qPCR and histology. We demonstrated the high sensitivity and specificity of RT-qPCR to detect EWS MRD in OT/TT samples. Clinical trial: NCT 02400970. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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Review

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14 pages, 970 KiB  
Review
Primary and Metastatic Intracranial Ewing Sarcoma at Diagnosis: Retrospective International Study and Systematic Review
by Lianne M. Haveman, Andreas Ranft, Henk van den Berg, Stephanie Klco-Brosius, Ruth Ladenstein, Michael Paulussen, Heribert Juergens, Uta Dirksen and Johannes H.M. Merks
Cancers 2020, 12(6), 1675; https://doi.org/10.3390/cancers12061675 - 24 Jun 2020
Cited by 11 | Viewed by 4241
Abstract
Intracranial Ewing sarcoma (EwS) is rare and publications on primary or metastatic intracranial EwS are minimal. The aim of this study was to describe incidence, clinical behavior, treatment, and factors associated with outcome in patients with primary intracranial EwS or patients with a [...] Read more.
Intracranial Ewing sarcoma (EwS) is rare and publications on primary or metastatic intracranial EwS are minimal. The aim of this study was to describe incidence, clinical behavior, treatment, and factors associated with outcome in patients with primary intracranial EwS or patients with a primary extracranial EwS and cerebral metastases at diagnosis. We reviewed all patients with primary or with metastatic intracranial EwS at diagnosis registered in the International Clinical Trial Euro-E.W.I.N.G.99 (EE99). In total, 17 of 1435 patients (1.2%) presented with primary intracranial EwS; 3 of them had metastatic disease. Four patients (0.3%) with primary extracranial EwS presented with intracranial metastatic lesions. The 3-year event-free survival (EFS) was 64% and overall survival (OS) was 70% in patients with a primary intracranial EwS. Local control in patients with primary intracranial EwS consisted of surgery (6%), radiotherapy (RT) (18%), or both modalities (76%). Univariate analysis showed that patients < 15 years of age had significantly better outcome (EFS: 72%; OS: 76%) compared to those aged above 15 years (EFS: 13%; OS: 25%). In conclusion, primary intracranial EwS and extracranial EwS with cerebral metastases at diagnosis is rare, yet survival is comparable with local and metastatic EwS elsewhere in the body. Age and stage of disease are important prognostic factors. Besides chemotherapeutic treatment, local control with surgical resection combined with RT is recommended whenever feasible. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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Other

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11 pages, 263 KiB  
Commentary
Current Status and Perspectives of Patient-Derived Models for Ewing’s Sarcoma
by Tadashi Kondo
Cancers 2020, 12(9), 2520; https://doi.org/10.3390/cancers12092520 - 4 Sep 2020
Cited by 4 | Viewed by 2436
Abstract
Patient-derived cancer models, including cell lines, organoids, and xenografts, are indispensable tools in cancer research. These models, which recapitulate molecular features of original tumors, allow studies on the biological significance of cancer-associated genes, antitumor effects of novel agents, and molecular mechanisms underlying clinical [...] Read more.
Patient-derived cancer models, including cell lines, organoids, and xenografts, are indispensable tools in cancer research. These models, which recapitulate molecular features of original tumors, allow studies on the biological significance of cancer-associated genes, antitumor effects of novel agents, and molecular mechanisms underlying clinical behaviors of tumors. Moreover, the predictive utility of patient-derived cancer models is expected to facilitate drug development and precision medicine. Ewing’s sarcoma is a highly aggressive mesenchymal tumor with a high metastasis rate. Previous studies demonstrated the utility of cell lines and xenografts in Ewing’s sarcoma research and clinical studies. However, the number of Ewing’s sarcoma models available from public biobanks is limited; this creates an obstacle for research on Ewing’s sarcoma. Novel Ewing’s sarcoma models are needed to establish their utility, further our understanding of the molecular mechanisms, and help develop effective therapeutic strategies. In this review, the current status of patient-derived cancer models is overviewed, and future prospects of model development are discussed from the perspective of Ewing’s sarcoma research. It should be of interest to researchers and clinicians who work on patient-derived cancer models. Full article
(This article belongs to the Special Issue Ewing Sarcoma)
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