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1. Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: expert recommendations from the World Sarcoma Network

Author

Demetri, G.D., Antonescu, C.R., Bjerkehagen, B., Bovée, J.V.M.G., Boye, K., Chacón, M., Dei Tos, A.P., Desai, J., Fletcher, J.A., Gelderblom, H., George, S., Gronchi, A., Haas, R.L., Hindi, N., Hohenberger, P., Joensuu, H., Jones, R.L., Judson, I., Kang, Y.-K., Kawai, A., Lazar, A.J., Le Cesne, A., Maestro, R., Maki, R.G., Martín, J., Patel, S., Penault-Llorca, F., Premanand Raut, C., Rutkowski, P., Safwat, A., Sbaraglia, M., Schaefer, I.-M., Shen, L., Serrano, C., Schöffski, P., Stacchiotti, S., Sundby Hall, K., Tap, W.D., Thomas, D.M., Trent, J., Valverde, C., van der Graaf, W.T.A., von Mehren, M., Wagner, A., Wardelmann, E., Naito, Y., Zalcberg, J., and Blay, J.-Y.

Published
2020
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4. Bone sarcomas: ESMO–PaedCan–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Casali, P.G., Bielack, S., Abecassis, N., Aro, H.T., Bauer, S., Biagini, R., Bonvalot, S., Boukovinas, I., Bovee, J V M G, Brennan, B., Brodowicz, T., Broto, J.M., Brugières, L., Buonadonna, A., De Álava, E., Dei Tos, A.P., Del Muro, X.G., Dileo, P., Dhooge, C., Eriksson, M., Fagioli, F., Fedenko, A., Ferraresi, V., Ferrari, A., Ferrari, S., Frezza, A.M., Gaspar, N., Gasperoni, S., Gelderblom, H., Gil, T., Grignani, G., Gronchi, A., Haas, R.L., Hassan, B., Hecker-Nolting, S., Hohenberger, P., Issels, R., Joensuu, H., Jones, R.L., Judson, I., Jutte, P., Kaal, S., Kager, L., Kasper, B., Kopeckova, K., Krákorová, D.A., Ladenstein, R., Le Cesne, A., Lugowska, I., Merimsky, O., Montemurro, M., Morland, B., Pantaleo, M.A., Piana, R., Picci, P., Piperno-Neumann, S., Pousa, A.L., Reichardt, P., Robinson, M.H., Rutkowski, P., Safwat, A.A., Schöffski, P., Sleijfer, S., Stacchiotti, S., Strauss, S.J., Sundby Hall, K., Unk, M., Van Coevorden, F., van der Graaf, W.T.A., Whelan, J., Wardelmann, E., Zaikova, O., and Blay, J.Y.

Published
2018
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5. Soft tissue and visceral sarcomas: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Casali, P.G., Abecassis, N., Bauer, S., Biagini, R., Bielack, S., Bonvalot, S., Boukovinas, I., Bovee, J V M G, Brodowicz, T., Broto, J.M., Buonadonna, A., De Álava, E., Dei Tos, A.P., Del Muro, X.G., Dileo, P., Eriksson, M., Fedenko, A., Ferraresi, V., Ferrari, A., Ferrari, S., Frezza, A.M., Gasperoni, S., Gelderblom, H., Gil, T., Grignani, G., Gronchi, A., Haas, R.L., Hannu, A., Hassan, B., Hohenberger, P., Issels, R., Joensuu, H., Jones, R.L., Judson, I., Jutte, P., Kaal, S., Kasper, B., Kopeckova, K., Krákorová, D.A., Le Cesne, A., Lugowska, I., Merimsky, O., Montemurro, M., Pantaleo, M.A., Piana, R., Picci, P., Piperno-Neumann, S., Pousa, A.L., Reichardt, P., Robinson, M.H., Rutkowski, P., Safwat, A.A., Schöffski, P., Sleijfer, S., Stacchiotti, S., Sundby Hall, K., Unk, M., Van Coevorden, F., Van der Graaf, W., Whelan, J., Wardelmann, E., Zaikova, O., and Blay, J.Y.

Published
2018
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6. Gastrointestinal stromal tumours: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Casali, P.G., Abecassis, N., Bauer, S., Biagini, R., Bielack, S., Bonvalot, S., Boukovinas, I., Bovee, J.V.M.G., Brodowicz, T., Broto, J.M., Buonadonna, A., De Álava, E., Dei Tos, A.P., Del Muro, X.G., Dileo, P., Eriksson, M., Fedenko, A., Ferraresi, V., Ferrari, A., Ferrari, S., Frezza, A.M., Gasperoni, S., Gelderblom, H., Gil, T., Grignani, G., Gronchi, A., Haas, R.L., Hannu, A., Hassan, B., Hohenberger, P., Issels, R., Joensuu, H., Jones, R.L., Judson, I., Jutte, P., Kaal, S., Kasper, B., Kopeckova, K., Krákorová, D.A., Le Cesne, A., Lugowska, I., Merimsky, O., Montemurro, M., Pantaleo, M.A., Piana, R., Picci, P., Piperno-Neumann, S., Pousa, A.L., Reichardt, P., Robinson, M.H., Rutkowski, P., Safwat, A.A., Schöffski, P., Sleijfer, S., Stacchiotti, S., Sundby Hall, K., Unk, M., Van Coevorden, F., Van der Graaf, W., Whelan, J., Wardelmann, E., Zaikova, O., and Blay, J.Y.

Published
2018
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9. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)

Author

Bauer, S., Blay, J.Y., van Coevorden, F., Dileo, P., Dürr, H.R., Fiore, M., Grünwald, V., Jones, R., Judson, I., Kettelhack, C., Kopeckova, K., Lazar, A., Lindner, L.H., Martin-Broto, J., Rutkowski, P., Stacchiotti, S., Stoeckle, E., Valverde, C., Verhoef, K., Wardelmann, E., Wartenberg, M., Kasper, B., Baumgarten, C., Garcia, J., Bonvalot, S., Haas, R., Haller, F., Hohenberger, P., Penel, N., Messiou, C., van der Graaf, W.T., and Gronchi, A.

Published
2017
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10. Heritable defects in telomere and mitotic function selectively predispose to sarcomas

Author

Ballinger, ML, Pattnaik, S, Mundra, PA, Zaheed, M, Rath, E, Priestley, P, Baber, J, Ray-Coquard, I, Isambert, N, Causeret, S, van der Graaf, WTA, Puri, A, Duffaud, F, Le Cesne, A, Seddon, B, Chandrasekar, C, Schiffman, JD, Brohl, AS, James, PA, Kurtz, J-E, Penel, N, Myklebost, O, Meza-Zepeda, LA, Pickett, H, Kansara, M, Waddell, N, Kondrashova, O, Pearson, J, Barbour, AP, Li, S, Nguyen, TL, Fatkin, D, Graham, RM, Giannoulatou, E, Green, MJ, Kaplan, W, Ravishankar, S, Copty, J, Powell, JE, Cuppen, E, van Eijk, K, Veldink, J, Ahn, J-H, Kim, JE, Randall, RL, Tucker, K, Judson, I, Sarin, R, Ludwig, T, Genin, E, Deleuze, J-F, Haber, M, Marshall, G, Cairns, MJ, Blay, J-Y, Thomas, DM, Ballinger, ML, Pattnaik, S, Mundra, PA, Zaheed, M, Rath, E, Priestley, P, Baber, J, Ray-Coquard, I, Isambert, N, Causeret, S, van der Graaf, WTA, Puri, A, Duffaud, F, Le Cesne, A, Seddon, B, Chandrasekar, C, Schiffman, JD, Brohl, AS, James, PA, Kurtz, J-E, Penel, N, Myklebost, O, Meza-Zepeda, LA, Pickett, H, Kansara, M, Waddell, N, Kondrashova, O, Pearson, J, Barbour, AP, Li, S, Nguyen, TL, Fatkin, D, Graham, RM, Giannoulatou, E, Green, MJ, Kaplan, W, Ravishankar, S, Copty, J, Powell, JE, Cuppen, E, van Eijk, K, Veldink, J, Ahn, J-H, Kim, JE, Randall, RL, Tucker, K, Judson, I, Sarin, R, Ludwig, T, Genin, E, Deleuze, J-F, Haber, M, Marshall, G, Cairns, MJ, Blay, J-Y, and Thomas, DM

Abstract

Cancer genetics has to date focused on epithelial malignancies, identifying multiple histotype-specific pathways underlying cancer susceptibility. Sarcomas are rare malignancies predominantly derived from embryonic mesoderm. To identify pathways specific to mesenchymal cancers, we performed whole-genome germline sequencing on 1644 sporadic cases and 3205 matched healthy elderly controls. Using an extreme phenotype design, a combined rare-variant burden and ontologic analysis identified two sarcoma-specific pathways involved in mitotic and telomere functions. Variants in centrosome genes are linked to malignant peripheral nerve sheath and gastrointestinal stromal tumors, whereas heritable defects in the shelterin complex link susceptibility to sarcoma, melanoma, and thyroid cancers. These studies indicate a specific role for heritable defects in mitotic and telomere biology in risk of sarcomas.

Published
2023

11. Impact of chemotherapy in uterine sarcoma (UtS): review of 13 clinical trials from the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) involving advanced/metastatic UtS compared to other soft tissue sarcoma (STS) patients treated with first line chemotherapy

Author

Ray-Coquard, I., Rizzo, E., Blay, J.Y., Casali, P., Judson, I., Hansen, A. Krarup, Lindner, L.H., dei Tos, A.P., Gelderblom, H., Marreaud, S., Litière, S., Rutkowski, P., Hohenberger, P., Gronchi, A., and van der Graaf, W.T.

Published
2016
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12. Outcome of uterine sarcoma patients treated with pazopanib: A retrospective analysis based on two European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) clinical trials 62043 and 62072

Author

Benson, C., Ray-Coquard, I., Sleijfer, S., Litière, S., Blay, J.-Y., Le Cesne, A., Papai, Z., Judson, I., Schöffski, P., Chawla, S., Gil, T., Piperno-Neumann, S., Marréaud, S., Dewji, M.R., and van der Graaf, W.T.A.

Published
2016
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15. 1491MO New benchmarks for designing clinical trials in advanced or metastatic liposarcoma (LPS) or synovial sarcoma (SS): An EORTC soft tissue and bone sarcoma group meta-analysis

Author

Kantidakis, G., primary, Litiere, S., additional, Neven, A., additional, Vinches, M., additional, Judson, I., additional, Blay, J-Y., additional, Wardelmann, E., additional, Stacchiotti, S., additional, D'Ambrosio, L., additional, Marreaud, S.I., additional, van der Graaf, W.T.A., additional, Kasper, B., additional, Fiocco, M., additional, and Gelderblom, H., additional

Published
2022
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27. Imaging vascular function for early stage clinical trials using dynamic contrast-enhanced magnetic resonance imaging

Author

Leach, M. O., Morgan, B., Tofts, P. S., Buckley, D. L., Huang, W., Horsfield, M. A., Chenevert, T. L., Collins, D. J., Jackson, A., Lomas, D., Whitcher, B., Clarke, L., Plummer, R., Judson, I., Jones, R., Alonzi, R., Brunner, T., Koh, D. M., Murphy, P., Waterton, J. C., Parker, G., Graves, M. J., Scheenen, T. W. J., Redpath, T. W., Orton, M., Karczmar, G., Huisman, H., Barentsz, J., Padhani, A., and on behalf of the Experimental Cancer Medicine Centres Imaging Network Steering Committee

Published
2012
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31. High-Dose Chemotherapy and Blood Autologous Stem-Cell Rescue Compared With Standard Chemotherapy in Localized High-Risk Ewing Sarcoma: Results of Euro-E.W.I.N.G.99 and Ewing-2008

Author

Whelan, J., Deley, M.C. le, Dirksen, U., Teuff, G. le, Brennan, B., Gaspar, N., Hawkins, D.S., Amler, S., Bauer, S., Bielack, S., Blay, J.Y., Burdach, S., Castex, M.P., Dilloo, D., Eggert, A., Gelderblom, H., Gentet, J.C., Hartmann, W., Hassenpflug, W.A., Hjorth, L., Jimenez, M., Klingebiel, T., Kontny, U., Kruseova, J., Ladenstein, R., Laurence, V., Lervat, C., Marec-Berard, P., Marreaud, S., Michon, J., Morland, B., Paulussen, M., Ranft, A., Reichardt, P., Berg, H. van den, Wheatley, K., Judson, I., Lewis, I., Craft, A., Juergens, H., Oberlin, O., Euro- E W I N G 99 Investigator, EWING-2008 Investigator, Paediatric Oncology, and CCA - Cancer Treatment and Quality of Life

Subjects
0301 basic medicine, Melphalan, Cancer Research, medicine.medical_specialty, Vincristine, medicine.medical_treatment, Medizin, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Original Reports, Medicine, Etoposide, Chemotherapy, Ifosfamide, business.industry, Hazard ratio, medicine.disease, 030104 developmental biology, Oncology, 030220 oncology & carcinogenesis, Sarcoma, business, Busulfan, medicine.drug
Abstract

Purpose For over 30 years, the place of consolidation high-dose chemotherapy in Ewing sarcoma (ES) has been controversial. A randomized study was conducted to determine whether consolidation high-dose chemotherapy improved survival in patients with localized ES at high risk for relapse. Methods Randomization between busulfan and melphalan (BuMel) or standard chemotherapy (vincristine, dactinomycin, and ifosfamide [VAI], seven courses) was offered to patients if they were younger than 50 years of age with poor histologic response (≥ 10% viable cells) after receiving vincristine, ifosfamide, doxorubicin, and etoposide (six courses); or had a tumor volume at diagnosis ≥ 200 mL if unresected, or initially resected, or resected after radiotherapy. A 15% improvement in 3-year event-free survival (EFS) was sought (hazard ratio [HR], 0.60). Results Between 2000 and 2015, 240 patients classified as high risk (median age, 17.1 years) were randomly assigned to VAI (n = 118) or BuMel (n = 122). Seventy-eight percent entered the trial because of poor histologic response after chemotherapy alone. Median follow-up was 7.8 years. In an intent-to-treat analysis, the risk of event was significantly decreased by BuMel compared with VAI: HR, 0.64 (95% CI, 0.43 to 0.95; P = .026); 3- and 8-year EFS were, respectively, 69.0% (95% CI, 60.2% to 76.6%) versus 56.7% (95% CI, 47.6% to 65.4%) and 60.7% (95% CI, 51.1% to 69.6%) versus 47.1% (95% CI, 37.7% to 56.8%). Overall survival (OS) also favored BuMel: HR, 0.63 (95% CI, 0.41 to 0.95; P = .028); 3- and 8-year OS were, respectively, 78.0% (95% CI, 69.6% to 84.5%) versus 72.2% (95% CI, 63.3% to 79.6%) and 64.5% (95% CI, 54.4% to 73.5%) versus 55.6% (95% CI, 45.8% to 65.1%). Results were consistent in the sensitivity analysis. Two patients died as a result of BuMel-related toxicity, one after standard chemotherapy. Significantly more BuMel patients experienced severe acute toxicities from this course of chemotherapy compared with multiple VAI courses. Conclusion BuMel improved EFS and OS when given after vincristine, ifosfamide, doxorubicin, and etoposide induction in localized ES with predefined high-risk factors. For this group of patients, BuMel may be an important addition to the standard of care.

Published
2018

32. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients

Author

Alman, B., Attia, S., Baumgarten, C., Benson, C., Blay, J.Y., Bonvalot, S., Breuing, J., Cardona, K., Casali, P.G., Coevorden, F. van, Colombo, C., Dei Tos, A.P., Dileo, P., Ferrari, A., Fiore, M., Frezza, A.M., Garcia, J., Gladdy, R., Gounder, M., Gronchi, A., Haas, R, Hackett, S., Haller, F., Hohenberger, P., Husson, O., Jones, R.L., Judson, I., Kasper, B., Kawai, A., Kogosov, V., Lazar, A., Maki, R., Mathes, T., Messiou, C., Navid, F., Nishida, Y., Palassini, E., Penel, N., Pollock, R., Pieper, D., Portnoy, M., Raut, C.P., Roets, E., Sandrucci, S., Sbaraglia, M., Stacchiotti, S., Thornton, K.A., Graaf, W.T.A. van der, Zande, K. van der, Houdt, W.J. van, Villalobos, V., Wagner, A.J., Wardelmann, E., Wartenberg, M., Watson, S., Weiss, A., Zafiropoulos, N., Alman, B., Attia, S., Baumgarten, C., Benson, C., Blay, J.Y., Bonvalot, S., Breuing, J., Cardona, K., Casali, P.G., Coevorden, F. van, Colombo, C., Dei Tos, A.P., Dileo, P., Ferrari, A., Fiore, M., Frezza, A.M., Garcia, J., Gladdy, R., Gounder, M., Gronchi, A., Haas, R, Hackett, S., Haller, F., Hohenberger, P., Husson, O., Jones, R.L., Judson, I., Kasper, B., Kawai, A., Kogosov, V., Lazar, A., Maki, R., Mathes, T., Messiou, C., Navid, F., Nishida, Y., Palassini, E., Penel, N., Pollock, R., Pieper, D., Portnoy, M., Raut, C.P., Roets, E., Sandrucci, S., Sbaraglia, M., Stacchiotti, S., Thornton, K.A., Graaf, W.T.A. van der, Zande, K. van der, Houdt, W.J. van, Villalobos, V., Wagner, A.J., Wardelmann, E., Wartenberg, M., Watson, S., Weiss, A., and Zafiropoulos, N.

Abstract

Contains fulltext : 217698.pdf (Publisher’s version ) (Open Access)

Published
2020

33. Metastatic Soft Tissue Sarcomas in Adolescents and Young Adults: A Specialist Center Experience

Author

Younger, E., Husson, O., Asare, B., Benson, C., Judson, I., Miah, A., Zaidi, S., Dunlop, A., Al-Muderis, O., Houdt, W.J. van, Jones, R.L., Graaf, W.T.A. van der, Younger, E., Husson, O., Asare, B., Benson, C., Judson, I., Miah, A., Zaidi, S., Dunlop, A., Al-Muderis, O., Houdt, W.J. van, Jones, R.L., and Graaf, W.T.A. van der

Abstract

Contains fulltext : 229630.pdf (publisher's version ) (Open Access), Purpose: Soft tissue sarcomas (STS) account for 8% of all cancers in adolescents and young adults (AYAs). Metastatic STS contribute significantly to disease-related mortality in this age group; however, data are limited due to under-representation in clinical trials. Methods: AYAs aged 18-39 years, diagnosed with metastatic STS between 1990 and 2012, were identified from The Royal Marsden Hospital database. Outcomes of interest were clinical characteristics, treatment patterns, overall survival (OS), and prognostic factors. Results: Overall, 455 patients were included. Median age at diagnosis of metastatic STS was 33 years (interquartile range [IQR] 27-37 years). The most common histological subtypes were leiomyosarcoma (n = 68, 15%), synovial sarcoma (n = 68, 15%), Ewing sarcoma (n = 44, 10%), and rhabdomyosarcoma (n = 35, 8%). Treatments included systemic therapy (n = 395, 87%; median 2 lines [IQR 1-3]; clinical trial n = 93, 22%), radiotherapy (n = 297, 66%), and metastasectomy (n = 191, 43%). Median duration between last chemotherapy regimen and death was 4.6 months (IQR 2-10). Median OS was 19.2 months (95% confidence interval [CI] 15.8-22.2); 5-year OS was 16%. Of common subtypes, patients with rhabdomyosarcoma had the worst OS (8.8 months; 95% CI 7.9-11.4). Adverse prognostic factors included male gender, synchronous metastases, bone or liver metastases, first-line polychemotherapy, and no metastasectomy. Conclusions: Outcomes were variable; patients with supposed chemosensitive subtypes had particularly poor survival. The diverse behavior of STS in AYAs highlights the need for subtype-specific clinical trials.

Published
2020

34. Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: expert recommendations from the World Sarcoma Network

Author

Demetri, GD, Antonescu, CR, Bjerkehagen, B, Bovée, JVMG, Boye, K, Chacón, M, Dei Tos, AP, Desai, J, Fletcher, JA, Gelderblom, H, George, S, Gronchi, A, Haas, RL, Hindi, N, Hohenberger, P, Joensuu, H, Jones, RL, Judson, I, Kang, YK, Kawai, A, Lazar, AJ, Le Cesne, A, Maestro, R, Maki, RG, Martín, J, Patel, S, Penault-Llorca, F, Premanand Raut, C, Rutkowski, P, Safwat, A, Sbaraglia, M, Schaefer, IM, Shen, L, Serrano, C, Schöffski, P, Stacchiotti, S, Sundby Hall, K, Tap, WD, Thomas, DM, Trent, J, Valverde, C, van der Graaf, WTA, von Mehren, M, Wagner, A, Wardelmann, E, Naito, Y, Zalcberg, J, Blay, JY, Demetri, GD, Antonescu, CR, Bjerkehagen, B, Bovée, JVMG, Boye, K, Chacón, M, Dei Tos, AP, Desai, J, Fletcher, JA, Gelderblom, H, George, S, Gronchi, A, Haas, RL, Hindi, N, Hohenberger, P, Joensuu, H, Jones, RL, Judson, I, Kang, YK, Kawai, A, Lazar, AJ, Le Cesne, A, Maestro, R, Maki, RG, Martín, J, Patel, S, Penault-Llorca, F, Premanand Raut, C, Rutkowski, P, Safwat, A, Sbaraglia, M, Schaefer, IM, Shen, L, Serrano, C, Schöffski, P, Stacchiotti, S, Sundby Hall, K, Tap, WD, Thomas, DM, Trent, J, Valverde, C, van der Graaf, WTA, von Mehren, M, Wagner, A, Wardelmann, E, Naito, Y, Zalcberg, J, and Blay, JY

Abstract

Sarcomas are a heterogeneous group of malignancies with mesenchymal lineage differentiation. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as tissue-agnostic oncogenic drivers has led to new personalized therapies for a subset of patients with sarcoma in the form of tropomyosin receptor kinase (TRK) inhibitors. NTRK gene rearrangements and fusion transcripts can be detected with different molecular pathology techniques, while TRK protein expression can be demonstrated with immunohistochemistry. The rarity and diagnostic complexity of NTRK gene fusions raise a number of questions and challenges for clinicians. To address these challenges, the World Sarcoma Network convened two meetings of expert adult oncologists and pathologists and subsequently developed this article to provide practical guidance on the management of patients with sarcoma harboring NTRK gene fusions. We propose a diagnostic strategy that considers disease stage and histologic and molecular subtypes to facilitate routine testing for TRK expression and subsequent testing for NTRK gene fusions.

Published
2020

35. Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: expert recommendations from the World Sarcoma Network

Author

Harvard University, Adelson Medical Research Foundation, National Institutes of Health (US), National Cancer Institute (US), Demetri, G. D., Antonescu, Cristina R., Bjerkehagen, B., Bovee, J. V. M. G., Boye, K., Chacón, Matias, Dei Tos, Angelo Paolo, Desai, Jayesh, Fletcher, Jonathan A., Gelderblom, Hans, George, Suzanne, Gronchi, Alesandro, Haas, R. L., Hindi, Nadia, Hohenberger, Peter, Joensuu, Heikki, Jones, Robin L., Judson, I., Kang, Y.-K., Kawai, Akira, Lazar, A. J., Cesne, Axel Le, Maestro, Roberta, Maki, Robert, Martín, J., Patel, S., Penault-Llorca, F., Premanand Raut, C., Rutkowski, Piotr, Safwat, A. A., Sbaraglia, Marta, Schaefer, I.-M., Shen, L., Serrano, C., Schöffski, P., Stacchiotti, Silvia, Sundby Hall, K., Tap, William D., Thomas, David M., Trent, J., Valverde, Claudia M., Graaf, W.T.A. van der, Mehren, M. von, Wagner, Andrew J., Wardelmann, Eva, Naito, Y., Zalcberg, J., Blay, Jean-Yves, Harvard University, Adelson Medical Research Foundation, National Institutes of Health (US), National Cancer Institute (US), Demetri, G. D., Antonescu, Cristina R., Bjerkehagen, B., Bovee, J. V. M. G., Boye, K., Chacón, Matias, Dei Tos, Angelo Paolo, Desai, Jayesh, Fletcher, Jonathan A., Gelderblom, Hans, George, Suzanne, Gronchi, Alesandro, Haas, R. L., Hindi, Nadia, Hohenberger, Peter, Joensuu, Heikki, Jones, Robin L., Judson, I., Kang, Y.-K., Kawai, Akira, Lazar, A. J., Cesne, Axel Le, Maestro, Roberta, Maki, Robert, Martín, J., Patel, S., Penault-Llorca, F., Premanand Raut, C., Rutkowski, Piotr, Safwat, A. A., Sbaraglia, Marta, Schaefer, I.-M., Shen, L., Serrano, C., Schöffski, P., Stacchiotti, Silvia, Sundby Hall, K., Tap, William D., Thomas, David M., Trent, J., Valverde, Claudia M., Graaf, W.T.A. van der, Mehren, M. von, Wagner, Andrew J., Wardelmann, Eva, Naito, Y., Zalcberg, J., and Blay, Jean-Yves

Abstract

Sarcomas are a heterogeneous group of malignancies with mesenchymal lineage differentiation. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as tissue-agnostic oncogenic drivers has led to new personalized therapies for a subset of patients with sarcoma in the form of tropomyosin receptor kinase (TRK) inhibitors. NTRK gene rearrangements and fusion transcripts can be detected with different molecular pathology techniques, while TRK protein expression can be demonstrated with immunohistochemistry. The rarity and diagnostic complexity of NTRK gene fusions raise a number of questions and challenges for clinicians. To address these challenges, the World Sarcoma Network convened two meetings of expert adult oncologists and pathologists and subsequently developed this article to provide practical guidance on the management of patients with sarcoma harboring NTRK gene fusions. We propose a diagnostic strategy that considers disease stage and histologic and molecular subtypes to facilitate routine testing for TRK expression and subsequent testing for NTRK gene fusions.

Published
2020

42. 1628MO A new benchmark for designing phase II trials for advanced or metastatic leiomyosarcoma (LMS) patients using progression free survival (PFS) as primary endpoint – an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) meta-analysis

Author

Kantidakis, G., primary, Litiere, S., additional, Neven, A., additional, Vinches, M., additional, Judson, I., additional, Schöffski, P., additional, Stacchiotti, S., additional, D’Ambrosio, L., additional, Marreaud, S.I., additional, Van Der Graaf, W.T.A., additional, Fiocco, M., additional, Kasper, B., additional, and Gelderblom, H., additional

Published
2020
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43. 1629MO First-line chemotherapy (CT) in advanced well-differentiated/dedifferentiated liposarcoma (WD/DD LPS): An EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis

Author

Stacchiotti, S., primary, Van der Graaf, W., additional, Doms, H., additional, Sanfilippo, R., additional, Marreaud, S.I., additional, Van Houdt, W., additional, Judson, I., additional, Kasper, B., additional, Litiere, S., additional, and Gelderblom, H., additional

Published
2020
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49. The impact of chemotherapy on survival of patients with extremity and trunk wall soft tissue sarcoma: revisiting the results of the EORTC-STBSG 62931 randomised trial

Author

Pasquali, S., Pizzamiglio, S., Touati, N., Litiere, S., Marreaud, S., Kasper, B., Gelderblom, H., Stacchiotti, S., Judson, I., Tos, A.P. dei, Verderio, P., Casali, P.G., Woll, P.J., Gronchi, A., and EORTC Soft Tissue Bone Sarcoma Grp

Subjects
0301 basic medicine, Oncology, Male, Cancer Research, medicine.medical_treatment, law.invention, 0302 clinical medicine, Randomized controlled trial, Interquartile range, law, Antineoplastic Combined Chemotherapy Protocols, Overall survival, Adjuvant, Randomised controlled trial, Soft tissue sarcoma, Ifosfamide, High risk, Torso, Sarcoma, Chemotherapy, Middle Aged, Prognosis, Neoadjuvant Therapy, Survival Rate, Chemotherapy, Adjuvant, 030220 oncology & carcinogenesis, Female, medicine.drug, Adult, medicine.medical_specialty, Adolescent, 03 medical and health sciences, Young Adult, Internal medicine, medicine, Humans, Doxorubicin, Aged, business.industry, Extremities, Nomogram, medicine.disease, Nomograms, 030104 developmental biology, business, Follow-Up Studies
Abstract

Background: This study was aimed at determining whether patients with high-risk soft tissue sarcoma (STS), as identified using the nomogram Sarculator, benefitted from adjuvant chemotherapy in the EORTC-STBSG 62931 randomised controlled trial (RCT), which failed to detect an impact for adjuvant doxorubicin plus ifosfamide (Adj) over observation (Obs).Methods: Patients with extremity and trunk wall STS in the EORTC-STBSG 62931 RCT were analysed (N = 290/351). Ten-year predicted probability of overall survival (pr-OS) was calculated using the prognostic nomogram Sarculator. Patients were grouped into three categories of predicted pr-OS: high (pr-OS>66%), intermediate (51

Published
2019

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