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18 results on '"Bielack, S."'

1. Gastrointestinal stromal tumours

Author

Casali, P.G., Blay, J.Y., Abecassis, N., Bajpai, J., Bauer, S., Biagini, R., Bielack, S., Bonvalot, S., Boukovinas, I., Bovee, J.V.M.G., Boye, K., Brodowicz, T., Buonadonna, A., Alava, E. de, Tos, A.P. dei, Muro, X.G. del, Dufresne, A., Eriksson, M., Fedenko, A., Ferraresi, V., Ferrari, A., Frezza, A.M., Gasperoni, S., Gelderblom, H., Gouin, F., Grignani, G., Haas, R., Hassan, A.B., Hindi, N., Hohenberger, P., Joensuu, H., Jones, R.L., Jungels, C., Jutte, P., Kasper, B., Kawai, A., Kopeckova, K., Krakorova, D.A., Cesne, A. le, Grange, F. le, Legius, E., Leithner, A., Lopez-Pousa, A., Martin-Broto, J., Merimsky, O., Messiou, C., Miah, A.B., Mir, O., Montemurro, M., Morosi, C., Palmerini, E., Pantaleo, M.A., Piana, R., Piperno-Neumann, S., Reichardt, P., Rutkowski, P., Safwat, A.A., Sangalli, C., Sbaraglia, M., Scheipl, S., Schoffski, P., Sleijfer, S., Strauss, D., Strauss, S.J., Hall, K.S., Trama, A., Unk, M., Sande, M.A.J. van de, Graaf, W.T.A. van der, Houdt, W.J. van, Frebourg, T., Gronchi, A., Stacchiotti, S., ESMO Guidelines Comm, EURACAN, GENTURIS, Casali P.G., Blay J.Y., Abecassis N., Bajpai J., Bauer S., Biagini R., Bielack S., Bonvalot S., Boukovinas I., Bovee J.V.M.G., Boye K., Brodowicz T., Buonadonna A., De Alava E., Dei Tos A.P., Del Muro X.G., Dufresne A., Eriksson M., Fedenko A., Ferraresi V., Ferrari A., Frezza A.M., Gasperoni S., Gelderblom H., Gouin F., Grignani G., Haas R., Hassan A.B., Hindi N., Hohenberger P., Joensuu H., Jones R.L., Jungels C., Jutte P., Kasper B., Kawai A., Kopeckova K., Krakorova D.A., Le Cesne A., Le Grange F., Legius E., Leithner A., Lopez-Pousa A., Martin-Broto J., Merimsky O., Messiou C., Miah A.B., Mir O., Montemurro M., Morosi C., Palmerini E., Pantaleo M.A., Piana R., Piperno-Neumann S., Reichardt P., Rutkowski P., Safwat A.A., Sangalli C., Sbaraglia M., Scheipl S., Schoffski P., Sleijfer S., Strauss D., Strauss S.J., Hall K.S., Trama A., Unk M., van de Sande M.A.J., van der Graaf W.T.A., van Houdt W.J., Frebourg T., Gronchi A., Stacchiotti S., Medical Oncology, ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Public Health Research (PHR), and Man, Biomaterials and Microbes (MBM)

Subjects
GIST, clinical practice guidelines, gastrointestinal stromal tumour, surgery, tyrosine kinase inhibitor, Oncology, Medizin, Hematology, clinical practice guideline
Abstract

Gastrointestinal stromal tumours (GISTs) are malignant mesenchymal tumours with a variable clinical behaviour, marked by differentiation towards the interstitial cells of Cajal.1 GISTs belong to the family of soft tissue sarcomas (STSs) but are treated separately due to their peculiar histogenesis, clinical behaviour and specific therapy. This European Society for Medical Oncology (ESMO)–European Reference Network for Rare Adult Solid Cancers (EURACAN)–European Reference Network for Genetic Tumour Risk Syndromes (GENTURIS) Clinical Practice Guideline (CPG) will cover GISTs while other STSs are covered in the ESMO–EURACAN–European Reference Network for Paediatric Oncology (ERN PaedCan)–GENTURIS STS CPG.

Published
2022

2. Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up

Author

Strauss, S.J., Frezza, A.M., Abecassis, N., Bajpai, J., Bauer, S., Biagini, R., Bielack, S., Blay, J.Y., Bolle, S., Bonvalot, S., Boukovinas, I., Bovee, J.V.M.G., Boye, K., Brennan, B., Brodowicz, T., Buonadonna, A., Alava, E. de, Tos, A.P. dei, Muro, X.G. del, Dufresne, A., Eriksson, M., Fagioli, F., Fedenko, A., Ferraresi, V., Ferrari, A., Gaspar, N., Gasperoni, S., Gelderblom, H., Gouin, F., Grignani, G., Gronchi, A., Haas, R., Hassan, A.B., Hecker-Nolting, S., Hindi, N., Hohenberger, P., Joensuu, H., Jones, R.L., Jungels, C., Jutte, P., Kager, L., Kasper, B., Kawai, A., Kopeckova, K., Krakorova, D.A., Cesne, A. le, Grange, F. le, Legius, E., Leithner, A., Pousa, A.L., Martin-Broto, J., Merimsky, O., Messiou, C., Miah, A.B., Mir, O., Montemurro, M., Morland, B., Morosi, C., Palmerini, E., Pantaleo, M.A., Piana, R., Piperno-Neumann, S., Reichardt, P., Rutkowski, P., Safwat, A.A., Sangalli, C., Sbaraglia, M., Scheipl, S., Schoffski, P., Sleijfer, S., Strauss, D., Hall, K.S., Trama, A., Unk, M., Sande, M.A.J. van de, Graaf, W.T.A. van der, Houdt, W.J. van, Frebourg, T., Ladenstein, R., Casali, P.G., Stacchiotti, S., ESMO Guidelines Comm, EURACAN, GENTURIS, ERN PaedCan, European Society for Medical Oncology, Strauss S.J., Frezza A.M., Abecassis N., Bajpai J., Bauer S., Biagini R., Bielack S., Blay J.Y., Bolle S., Bonvalot S., Boukovinas I., Bovee J.V.M.G., Boye K., Brennan B., Brodowicz T., Buonadonna A., de Alava E., Dei Tos A.P., Garcia del Muro X., Dufresne A., Eriksson M., Fagioli F., Fedenko A., Ferraresi V., Ferrari A., Gaspar N., Gasperoni S., Gelderblom H., Gouin F., Grignani G., Gronchi A., Haas R., Hassan A.B., Hecker-Nolting S., Hindi N., Hohenberger P., Joensuu H., Jones R.L., Jungels C., Jutte P., Kager L., Kasper B., Kawai A., Kopeckova K., Krakorova D.A., Le Cesne A., Le Grange F., Legius E., Leithner A., Lopez Pousa A., Martin-Broto J., Merimsky O., Messiou C., Miah A.B., Mir O., Montemurro M., Morland B., Morosi C., Palmerini E., Pantaleo M.A., Piana R., Piperno-Neumann S., Reichardt P., Rutkowski P., Safwat A.A., Sangalli C., Sbaraglia M., Scheipl S., Schoffski P., Sleijfer S., Strauss D., Sundby Hall K., Trama A., Unk M., van de Sande M.A.J., van der Graaf W.T.A., van Houdt W.J., Frebourg T., Ladenstein R., Casali P.G., Stacchiotti S., ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Public Health Research (PHR), and Man, Biomaterials and Microbes (MBM)

Subjects
medicine.medical_specialty, diagnosis, Medizin, bone sarcoma, Bone Neoplasm, Bone Sarcoma, Follow-Up Studie, 03 medical and health sciences, 0302 clinical medicine, follow-up, medicine, 030304 developmental biology, Osteosarcoma, 0303 health sciences, treatment, business.industry, Sarcoma, Hematology, Guideline, clinical practice guideline, management, 3. Good health, Clinical Practice, diagnosi, Oncology, Diagnosis treatment, 030220 oncology & carcinogenesis, Radiology, business, Human
Abstract

A. Kawai43, K. Kopeckova44, D. A. Krakorova45, A. Le Cesne46, F. Le Grange1, E. Legius47, A. Leithner48, A. Lopez Pousa49, J. Martin-Broto36, O. Merimsky50, C. Messiou51, A. B. Miah52, O. Mir53, M. Montemurro54, B. Morland55, C. Morosi56, E. Palmerini57, M. A. Pantaleo58, R. Piana59, S. Piperno-Neumann60, P. Reichardt61, P. Rutkowski62, A. A. Safwat63, C. Sangalli64, M. Sbaraglia19, S. Scheipl48, P. Schoffski65, S. Sleijfer66, D. Strauss67, K. Sundby Hall13, A. Trama68, M. Unk69, M. A. J. van de Sande70, W. T. A. van der Graaf66,71, W. J. van Houdt72, T. Frebourg73x, R. Ladenstein41z, P. G. Casali2,74z &

Published
2021

3. Soft tissue and visceral sarcomas

Author

Gronchi, A., Miah, A.B., DeiTos, A.P., Abecassis, N., Bajpai, J., Bauer, S., Biagini, R., Bielack, S., Blay, J.Y., Bolle, S., Bonvalot, S., Boukovinas, I., Bovee, J.V.M.G., Boye, K., Brennan, B., Brodowicz, T., Buonadonna, A., DeAlava, E., Muro, X.G. del, Dufresne, A., Eriksson, M., Fagioli, F., Fedenko, A., Ferraresi, V., Ferrari, A., Frezza, A.M., Gasperoni, S., Gelderblom, H., Gouin, F., Grignani, G., Haas, R., Hassan, A.B., Hecker-Nolting, S., Hindi, N., Hohenberger, P., Joensuu, H., Jones, R.L., Jungels, C., Jutte, P., Kager, L., Kasper, B., Kawai, A., Kopeckova, K., Krakorova, D.A., Cesne, A. le, LeGrange, F., Legius, E., Leithner, A., Lopez-Pousa, A., Martin-Broto, J., Merimsky, O., Messiou, C., Mir, O., Montemurro, M., Morland, B., Morosi, C., Palmerini, E., Pantaleo, M.A., Piana, R., Piperno-Neumann, S., Reichardt, P., Rutkowski, P., Safwat, A.A., Sangalli, C., Sbaraglia, M., Scheipl, S., Schoffski, P., Sleijfer, S., Strauss, D., Strauss, S., SundbyHall, K., Trama, A., Unk, M., VandeSande, M.A.J., VanderGraaf, W.T.A., Houdt, W.J. van, Frebourg, T., Casali, P.G., Stacchiotti, S., ESMO Guidelines Comm, EURACAN, GENTURIS, ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Public Health Research (PHR), Man, Biomaterials and Microbes (MBM), Gronchi A., Miah A.B., Dei Tos A.P., Abecassis N., Bajpai J., Bauer S., Biagini R., Bielack S., Blay J.Y., Bolle S., Bonvalot S., Boukovinas I., Bovee J.V.M.G., Boye K., Brennan B., Brodowicz T., Buonadonna A., De Alava E., Del Muro X.G., Dufresne A., Eriksson M., Fagioli F., Fedenko A., Ferraresi V., Ferrari A., Frezza A.M., Gasperoni S., Gelderblom H., Gouin F., Grignani G., Haas R., Hassan A.B., Hecker-Nolting S., Hindi N., Hohenberger P., Joensuu H., Jones R.L., Jungels C., Jutte P., Kager L., Kasper B., Kawai A., Kopeckova K., Krakorova D.A., Le Cesne A., Le Grange F., Legius E., Leithner A., Lopez-Pousa A., Martin-Broto J., Merimsky O., Messiou C., Mir O., Montemurro M., Morland B., Morosi C., Palmerini E., Pantaleo M.A., Piana R., Piperno-Neumann S., Reichardt P., Rutkowski P., Safwat A.A., Sangalli C., Sbaraglia M., Scheipl S., Schoffski P., Sleijfer S., Strauss D., Strauss S., Sundby Hall K., Trama A., Unk M., van de Sande M.A.J., van der Graaf W.T.A., van Houdt W.J., Frebourg T., Casali P.G., and Stacchiotti S.

Subjects
sarcoma, uterine sarcoma, treatment, diagnosis, desmoid, Medizin, Sarcoma, Soft Tissue Neoplasms, Hematology, Clinical Practice Guideline, Soft tissue sarcomas, retroperitoneal sarcoma, Retroperitoneal Sarcoma, soft tissue sarcomas, Follow-Up Studie, Desmoid, Uterine Sarcoma, follow-up, management, Oncology, soft tissue sarcoma, Human
Abstract

ispartof: ANNALS OF ONCOLOGY vol:32 issue:11 pages:1348-1365 ispartof: location:England status: published

Published
2021

4. 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

5. 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., Alava, E., Tos, A.P., 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., Krakorova, D.A., 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., Schoffski, P., Sleijfer, S., Stacchiotti, S., Hall, K.S., Unk, M., Coevorden, F., Graaf, W.D., Whelan, J., Wardelmann, E., Zaikova, O., Blay, J.Y., ESMO Guidelines Comm EURACAN, 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., Blay, J.Y., Man, Biomaterials and Microbes (MBM), Public Health Research (PHR), ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Heikki Joensuu / Principal Investigator, Department of Oncology, Clinicum, HUS Comprehensive Cancer Center, [Casali, P. G.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Frezza, A. M.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Gronchi, A.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Casali, P. G.] Univ Milan, Milan, Italy, [Frezza, A. M.] Univ Milan, Milan, Italy, [Gronchi, A.] Univ Milan, Milan, Italy, [Abecassis, N.] EPE, Inst Portugues Oncol Lisboa Francisco Gentil, Lisbon, Portugal, [Bauer, S.] Univ Hosp Essen, Essen, Germany, [Biagini, R.] Regina Elena Inst Canc Res, IFO, Musculoskeletal Tissue Bank, Dept Oncol Orthoped, Rome, Italy, [Bielack, S.] Olga Hosp, Klinikum Stuttgart, Stuttgart, Germany, [Bonvalot, S.] Inst Curie, Paris, France, [Piperno-Neumann, S.] Inst Curie, Paris, France, [Boukovinas, I.] NORDIX, Athens, Greece, [Bovee, J. V. M. G.] Leiden Univ, Med Ctr, Dept Pathol, Leiden, Netherlands, [Brodowicz, T.] Med Univ Wien, Vienna Gen Hosp AKH, Vienna, Austria, [Broto, J. M.] Hosp Univ Virgen Rocio, CIBERONC, Seville, Spain, [De Alava, E.] Hosp Univ Virgen Rocio, CIBERONC, Seville, Spain, [Buonadonna, A.] Ctr Riferimento Oncol Aviano, Aviano, Italy, [Dei Tos, A. P.] Osped Reg Treviso S Maria Ca Foncello, Treviso, Italy, [Del Muro, X. G.] HUB, ICO Hosp, Integrated Unit, Barcelona, Spain, [Dileo, P.] Univ Coll London Hosp, Sarcoma Unit, London, England, [Eriksson, M.] Skane Univ Hosp Lund, Lund, Sweden, [Fedenko, A.] NN Blokhin Russian Canc Res Ctr, Moscow, Russia, [Ferraresi, V.] Inst Sci Hosp Care, Regina Elena Natl Canc Inst, IRCCS, Rome, Italy, [Ferrari, A.] Fdn IRCCS Ist Nazl Tumori, Pediat Oncol Unit, Milan, Italy, [Ferrari, S.] Ist Ortoped Rizzoli, Bologna, Italy, [Picci, P.] Ist Ortoped Rizzoli, Bologna, Italy, [Gasperoni, S.] Univ Careggi Firenze, Azienda Osped, Florence, Italy, [Gelderblom, H.] Leiden Univ, Med Ctr, Dept Med Oncol, Leiden, Netherlands, [Gil, T.] Inst Jules Bordet, Brussels, Belgium, [Grignani, G.] IRCCS, FPO, Candiolo Canc Inst, Candiolo, Italy, [Haas, R. L.] Netherlands Canc Inst, Dept Radiotherapy, Amsterdam, Netherlands, [Haas, R. L.] Leiden Univ, Med Ctr, Dept Radiotherapy, Leiden, Netherlands, [Hannu, A.] Turku Univ Hosp, Turun Yliopistollinen Keskussairaala, Turlu, Finland, [Hassan, B.] Oxford Univ Hosp NHS Fdn Trust, Oxford, England, [Hohenberger, P.] Mannheim Univ, Med Ctr, Mannheim, Germany, [Kasper, B.] Mannheim Univ, Med Ctr, Mannheim, Germany, [Issels, R.] Ludwig Maximilians Univ Munchen, Univ Hosp, Dept Med 3, Munich, Germany, [Joensuu, H.] Univ Helsinki, Cent Hosp, HUCH, Helsinki, Finland, [Jones, R. L.] Royal Marsden Hosp, London, England, [Van der Graaf, W.] Royal Marsden Hosp, London, England, [Judson, I.] Inst Canc Res, London, England, [Jutte, P.] Univ Med Ctr Groningen, Groningen, Netherlands, [Kaal, S.] Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands, [Kopeckova, K.] Univ Hosp Motol, Prague, Czech Republic, [Krakorova, D. A.] Masaryk Mem Canc Inst, Brno, Czech Republic, [Le Cesne, A.] Gustave Roussy Canc Campus, Villejuif, France, [Lugowska, I.] Marie Sklodowska Curie Inst, Oncol Ctr, Warsaw, Poland, [Rutkowski, P.] Marie Sklodowska Curie Inst, Oncol Ctr, Warsaw, Poland, [Merimsky, O.] Tel Aviv Sourasky Med Ctr Ichilov, Tel Aviv, Israel, [Montemurro, M.] Univ Hosp Lausanne, Med Oncol, Lausanne, Switzerland, [Pantaleo, M. A.] Univ Bologna, Policlin S Orsola Malpighi, Azienda Osped, Bologna, Italy, [Piana, R.] Univ Cita Salute & Sci Torino, Azienda Osped, Turin, Italy, [Pousa, A. L.] Hosp Santa Creu & Sant Pau, Fundacio Gestio Sanitaria, Barcelona, Spain, [Reichardt, P.] Helios Klinikum Berlin Buch, Berlin, Germany, [Robinson, M. H.] Weston Pk Hosp NHS Trust, Dept Clin Oncol, YCRC, Sheffield, S Yorkshire, England, [Safwat, A. A.] Aarhus Univ Hosp, Aarhus, Denmark, [Schoffski, P.] Leuven Canc Inst, Leuven, Belgium, [Sleijfer, S.] Erasmus MC Canc Inst, Dept Med Oncol, Rotterdam, Netherlands, [Stacchiotti, S.] Ist Nazl Tumori, Fdn Ist Ricovero & Cura Carattere Sci, Milan, Italy, [Hall, K. Sundby] Norwegian Radium Hosp, Oslo Univ Hosp, Dept Oncol, Oslo, Norway, [Unk, M.] Inst Oncol Ljubljana, Ljubljana, Slovenia, [Van Coevorden, F.] Netherlands Canc Inst Antoni van Leeuwenhoek, Amsterdam, Netherlands, [Whelan, J.] Univ Coll Hosp, London, England, [Wardelmann, E.] Univ Klinikum Munster, Gerhard Domagk Inst Pathol, Munster, Germany, [Zaikova, O.] Norwegian Radium Hosp, Oslo Univ Hosp, Oslo, Norway, [Blay, J. Y.] Ctr Leon Bernard, Lyon, France, [Blay, J. Y.] UCBL1, Lyon, France, Amgen Dompe, AROG Bayer, Blueprint Medicines, Eli Lilly, Daiichi Sankyo Pharma, Epizyme, GlaxoSmithKline, Novartis, Pfizer, PharmaMar, Incyte, Lilly, Bayer, Janssen-Cilag, Eisai, Loxo Oncology, Nanobiotix, Bristol-Myers Squibb, Merck Sharp Dohme, Roche, Amgen, EuroSarc, CoBioRes, Exelixis, Plexxikon, Sixth Element Capital, Adaptaimmune, Amcure, AstraZeneca, Boehringer Ingelheim, Cristal Therapeutics, Daichii Sankyo Pharma, Genzyme, Ipsen, Medpace, Nektar, Philogen, Piqur Therapeutics, Swedish Orphan Biovitrium, Advenchen, Epizyme Inc., PharmarMar, Novartis Oncology, Milestone, Menarini, and Medical Oncology

Subjects
SURGERY, medicine.medical_treatment, Medizin, Aftercare, Medical Oncology, law.invention, DOSE IMATINIB, Endosonography, 0302 clinical medicine, Randomized controlled trial, law, Recurrence, Antineoplastic Combined Chemotherapy Protocols, Medicine, Digestive System Surgical Procedures, Societies, Medical, Adjuvant imatinib, NEOADJUVANT/ADJUVANT IMATINIB MESYLATE, GiST, Progression, Incidence, Stomach, Margins of Excision, KIT, Hematology, Gastrointestinal stromal tumours, 3. Good health, Clinical Practice, Europe, Intestines, Self-Help Groups, Treatment Outcome, Diagnosis treatment, Oncology, Chemotherapy, Adjuvant, 030220 oncology & carcinogenesis, 030211 gastroenterology & hepatology, ADJUVANT IMATINIB, Neoadjuvant/adjuvant imatinib mesylate, PHASE-II TRIAL, Mutations, Rare cancers Radboud Institute for Health Sciences [Radboudumc 9], GIST, Adult, Image-Guided Biopsy, medicine.medical_specialty, Gastrointestinal Stromal Tumors, education, 3122 Cancers, 03 medical and health sciences, Stomach surgery, Carney-stratakis, CARNEY-STRATAKIS, Humans, Gist, Neoplasm Staging, Chemotherapy, business.industry, MUTATIONS, General surgery, ta3121, RANDOMIZED-TRIAL, digestive system diseases, Imatinib mesylate, Phase-ii trial, 3121 General medicine, internal medicine and other clinical medicine, Surgery, Laparoscopy, Patient Participation, business, Dose imatinib, Tomography, X-Ray Computed
Abstract

Gastrointestinal stromal tumours (GISTs) are rare tumours, with an estimated unadjusted incidence of around 1/100 000/year. This only covers clinically relevant GISTs, since, if investigated, amuch higher number of lesions ≤ 1 cmin diameter (microGISTs) can be found at histopathological examination of stomach tissue in middle-aged and elderly individuals. There is a slight prevalence in males. The median age is around 60–65 years, with a wide range. Occurrence in children is very rare. Paediatric GIST represents a clinically and molecularly distinct subset, marked by female predominance, absence of KIT/platelet- derived growth factor alpha (PDGFRA) mutations, frequent mutations or silencing of the four genes that encode the subunits of the succinate dehydrogenase (SDH) enzyme complex, gastric multicentric location and possible lymph node metastases. Some syndromes are linked to GISTs: • The Carney triad syndrome, marked by gastric GISTs, paraganglioma and pulmonary chondromas (these may occur at different ages); • Carney–Stratakis syndrome, marked by a dyad of GIST and paraganglioma; and • Neurofibromatosis type 1(NF1), possibly leading to wild-type (WT), often multicentric GIST, predominantly located in the small bowel Families with germline autosomal dominant mutations of KIT are an extremely rare finding, presenting with multiple GISTs at an early age, possibly along with other associated features such as pigmented skin macules, urticaria pigmentosa and diffuse hyperplasia of the interstitial cells of Cajal in the gut wall.

Published
2018

6. 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., Alava, E. de, Tos, A.P.D., Muro, X.G. del, 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., Krakorova, D.A., Cesne, A. le, 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., Schoffski, P., Sleijfer, S., Stacchiotti, S., Hall, K.S., Unk, M., Coevorden, F. van, Graaf, W. van der, Whelan, J., Wardelmann, E., Zaikova, O., Blay, J.Y., ESMO Guidelines Comm EURACAN, Heikki Joensuu / Principal Investigator, Department of Oncology, Clinicum, Medical Oncology, Man, Biomaterials and Microbes (MBM), Public Health Research (PHR), ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), 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.

Subjects
0301 basic medicine, medicine.medical_specialty, medicine.medical_treatment, education, 3122 Cancers, Medizin, CASE-SERIES ANALYSIS, ENDOMETRIAL STROMAL SARCOMA, Preoperative care, RPS WORKING GROUP, 03 medical and health sciences, 0302 clinical medicine, Biopsy, medicine, DESMOID-TYPE FIBROMATOSIS, RETROPERITONEAL SARCOMA, Endometrial stromal sarcoma, Uterine sarcoma, medicine.diagnostic_test, business.industry, Soft tissue, NECROSIS-FACTOR-ALPHA, Hematology, medicine.disease, ISOLATED LIMB PERFUSION, ta3122, Surgery, Radiation therapy, 030104 developmental biology, Oncology, Diagnosis treatment, 030220 oncology & carcinogenesis, Sarcoma, Radiology, RARE CANCER NETWORK, business, RANDOMIZED PHASE-II, Rare cancers Radboud Institute for Health Sciences [Radboudumc 9], ADJUVANT RADIATION-THERAPY
Abstract

Soft tissue sarcomas (STSs) gather over 80 histological entities, with even more molecular subsets, characterised by a low to very low incidence in all populations. The majority of sarcomas arise from the soft tissue (close to 75%), with ~15% gastrointestinal stromal tumours (GISTs) and 10% bone sarcomas. These ESMO–EURACAN (European Society for Medical Oncology– European Reference Network for rare adult solid cancers) Clinical Practice Guidelines cover STSs, while GISTs are covered by dedicated ESMO–EURACAN Clinical Practice Guidelines. Kaposi’s sarcoma is not considered in the present document. Extraskeletal Ewing and Ewing-like sarcoma is covered by ESMO Clinical Practice Guidelines on bone sarcomas. In general, the same principles for these tumours in children apply to adults. This is also the case for embryonal and alveolar rhabdomyosarcomas, which are exceedingly rare in adults. On the other hand, pleomorphic rhabdomyosarcoma is viewed as a high-grade, adult-type STS. Extraskeletal osteosarcoma is also a high-grade STS, whose clinical resemblance with osteosarcoma of bone is doubtful (prospective collection of data is encouraged to generate evidence on the therapeutic implications of such a diagnosis). Adult STS pathological subtypes occurring in adolescents should be managed the same way as in adult patients, though the same histotypemight display clinical peculiarities when occurring at different ages.

Published
2018

7. Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Hogendoorn, Pc, ESMO/EUROBONET Working Group, Athanasou, N, Bielack, S, De Alava, E, Tos, Ap, Ferrari, S, Gelderblom, H, Grimer, R, Hall, Ks, Hassan, B, Jurgens, H, Paulussen, M, Rozeman, L, Taminiau, Ah, Whelan, J, Vanel, D., and Aglietta, Massimo

Subjects
Male, Leiomyosarcoma, medicine.medical_specialty, Breast Sarcoma, Adolescent, Biopsy, Bone Sarcoma, Risk Assessment, nonmetastatic ewings-sarcoma childrens-oncology-group study-group protocols core needle-biopsy prognostic-factors musculoskeletal tumors dedifferentiated chondrosarcoma neoadjuvant chemotherapy adjuvant chemotherapy cartilaginous tumors, bone sarcomas, Antigens, CD, Secondary Prevention, medicine, Humans, Neoplasm Metastasis, Child, Trabectedin, Neoplasm Staging, Osteosarcoma, L-Lactate Dehydrogenase, Radiotherapy, business.industry, Incidence, Soft tissue sarcoma, Ewing's sarcoma, Hematology, Periodic Acid-Schiff Reaction, Alkaline Phosphatase, Prognosis, medicine.disease, Combined Modality Therapy, Surgery, Europe, Treatment Outcome, Oncology, Chemotherapy, Adjuvant, Female, Sarcoma, Radiology, Chondrosarcoma, business, Follow-Up Studies, medicine.drug
Abstract

The following recommendations apply to adult-type soft tissue sarcomas arising from limbs and superficial trunk. Recommendations on retroperitoneal sarcomas, desmoid-type fibromatosis, uterine sarcomas head and neck sarcomas and breast sarcomas are provided separately at the end of the chapter with regard to those main aspects by which they differ from more frequent soft tissue sarcomas. In general, the main principles of diagnosis and treatment may well apply to all soft tissue sarcomas, including the rarest presentations [e.g. visceral sarcomas other than gastrointestinal stromal tumours (GISTs)], which therefore are not specifically covered. Specific histological types, however, may deserve specific approaches, not necessarily covered hereafter, given the scope of these Recommendations. Extraskeletal Ewing sarcoma as well as embryonal and alveolar rhabdomyosarcoma are covered by other ESMO Clinical Practice Guidelines, inasmuch as they need completely different approaches. The same applies to GIST. Kaposi's sarcoma is excluded from this chapter.

Published
2010

8. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Casali, P.G., Blay, J.Y., Bertuzzi, A., Bielack, S., Bjerkehagen, B., Bonvalot, S., Boukovinas, I., Bruzzi, P., Dei Tos, A.P., Dileo, P., Eriksson, M., Fedenko, A., Ferrari, A., Ferrari, S., Gelderblom, H., Grimer, R., Gronchi, A., Haas, R, Hall, K.S., Hohenberger, P., Issels, R., Joensuu, H., Judson, I., Cesne, A. le, Litiere, S., Martin-Broto, J., Merimsky, O., Montemurro, M., Morosi, C., Picci, P., Ray-Coquard, I., Reichardt, P., Rutkowski, P., Schlemmer, M., Stacchiotti, S., Torri, V., Trama, A., Coevorden, F. van, Graaf, W.T.A. van der, Vanel, D., Wardelmann, E., Colombo, C., Fiore, M., Mariani, L., Miceli, R., Pollock, R.E., Raut, C.P., Strauss, D., and Swallow, C.J.

Subjects
medicine.medical_specialty, Chemotherapy, Ifosfamide, Health Planning Guidelines, business.industry, medicine.medical_treatment, Soft tissue, Sarcoma, Hematology, Sunitinib malate, Prognosis, Combined Modality Therapy, Clinical Practice, Oncology, Diagnosis treatment, medicine, Humans, Radiology, business, Societies, Medical, medicine.drug, Rare cancers Radboud Institute for Health Sciences [Radboudumc 9], Follow-Up Studies, Neoplasm Staging
Abstract

Contains fulltext : 138992.pdf (Publisher’s version ) (Closed access)

Published
2014

10. Ewing's sarcoma of the bone: ESMO Clinical Recommendations for diagnosis, treatment and follow-up.

Author

Paulussen, M., Bielack, S., Jürgens, H., and Casali, P. G.

Subjects
*EWING'S sarcoma, *OSTEOSARCOMA, *BONE cancer, *ONCOLOGY, *DISEASE incidence, *DIAGNOSIS, *THERAPEUTICS, *SOCIETIES
Abstract

The article presents clinical recommendations for diagnosis, treatment and follow-up of Ewing's sarcoma of the bone from the European Society for Medical Oncology (ESMO). The incidence rate of the disease in white Caucasians is 0.3/100,000 per year. Diagnosis includes biopsy, which provides sufficient material for conventional histology. Information is also presented on staging and risk assessment and treatment plan.

Published
2009
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11. Osteosarcoma: ESMO Clinical Recommendations for diagnosis, treatment and follow-up.

Author

Bielack, S., Carrle, D., and Casali, P. G.

Subjects
*OSTEOSARCOMA, *ONCOLOGY, *DISEASE incidence, *BONE cancer treatment, *HEALTH risk assessment, *DIAGNOSIS, *SOCIETIES
Abstract

The article presents clinical recommendations for diagnosis, treatment and follow-up of osteosarcoma from the European Society for Medical Oncology (ESMO). Disease incidence is higher in adolescents and it usually arises in the metaphysis of a long extremity bone. Diagnosis involves an analysis of signs and symptoms, such as history of pain, but definitive diagnosis requires histological examination. Information is also presented on staging and risk assessment and treatment plan.

Published
2009
Full Text
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12. Ewing's sarcoma of the bone: ESMO Clinical Recommendations for diagnosis, treatment and follow-up.

Author

Paulussen, M., Bielack, S., Jrügens, H., and Jost, L.

Subjects
*EWING'S sarcoma, *BONE cancer, *MEDICAL societies, *BIOPSY, *CANCER treatment, *CANCER diagnosis, *PATHOLOGY, *ONCOLOGY
Abstract

The article presents the clinical recommendations of the European Society for Medical Oncology (ESMO) for the diagnosis, treatment and follow-up of patients with Ewing's sarcoma of the bone. Data on the age-related and population incidence of the disease are given. Diagnostic procedures may include biopsy and pathological tests by a pathologist with expertise in bone tumors. Components of the staging and risk assessment of the disease are discussed. Also noted are treatment options of localized disease, and metastatic and recurrent disease.

Published
2008
Full Text
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13. Osteosarcoma: ESMO Clinical Recommendations for diagnosis, treatment and follow-up.

Author

Bielack, S., Carrle, D., and Jost, L.

Subjects
*OSTEOSARCOMA, *MEDICAL societies, *BONE cancer, *CANCER diagnosis, *CANCER treatment, *ONCOLOGY
Abstract

The article presents the clinical recommendations of the European Society for Medical Oncology (ESMO) for the diagnosis, treatment and follow-up of patients with osteosarcoma. Data on age-adjusted incidence of the disease is given. Diagnostic procedures include histological examination of tumor material and pathology confirmation. Components of the staging and risk assessment of the disease are outlined. Also noted are treatment plans for localized tumors, metastatic disease and recurrent disease.

Published
2008
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14. Cancer burden in adolescents and young adults in Europe

Author

Trama Annalisa, Stark Daniel, Spasojević-Božović Ivana, Gaspar Nathalie, Peccatori Fedro, Toss Angela, Bernasconi Alice, Quarello Paola, Scheinemann Katrin, Jezdić Svetlana, Blondel Anne, Mountzios Giannis, Bielack Stefan, Saloustros Emmanouil, Ferrari A, Trama, A, Stark, D, Bozovic-Spasojevic, I, Gaspar, N, Peccatori, F, Toss, A, Bernasconi, A, Quarello, P, Scheinemann, K, Jezdic, S, Blondeel, A, Mountzios, G, Bielack, S, Saloustros, E, and Ferrari, A

Subjects
incidence, mortality, Cancer Research, Oncology, population-based cancer registry, adolescents and young adults, cancer, adolescents and young adult
Abstract

Background:Cancer epidemiology is unique in adolescents and young adults (AYAs; aged 15-39 years). The European Society for Medical Oncology/European Society for Paediatric Oncology (ESMO/SIOPE) AYA Working Group aims to describe the burden of cancers in AYAs in Europe and across European Union (EU) countries.Patients and methods:We used data available on the Global Cancer Observatory. We retrieved crude and age-standardised (World Standard Population) incidence and mortality rates. We reported about AYA cancer burden in Europe and between 28 EU member states. We described incidence and mortality for all cancers and for the 13 cancers most relevant to the AYA population.Results:Incidence and mortality varied widely between countries with the highest mortality observed in Eastern EU countries. Cancers of the female breast, thyroid and male testis were the most common cancers across countries followed by melanoma of skin and cancers of the cervix. Variations in cancer incidence rates across different populations may reflect different distribution of risk factors, variations in the implementation or uptake of screening as well as overdiagnosis. AYA cancer mortality disparities may be due to variation in early-stage diagnoses, different public education and awareness of cancer symptoms, different degrees of access or availability of treatment.Conclusions:Our results highlight the future health care needs and requirements for AYA-specialised services to ensure a homogeneous treatment across different countries as well as the urgency for preventive initiatives that can mitigate the increasing burden. &nbsp

Published
2023

15. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

Author

Peter Reichardt, C. Zimmermann, Emanuela Palmerini, Paul H. Huang, G. Oldani, R. Biagini, Silvia Stacchiotti, Akira Kawai, H. Leonard, A. P. Dei Tos, Andrew J. Wagner, Mikael Eriksson, Alessandro Gronchi, Kjetil Boye, Peter Hohenberger, X. Garcia del Muro, C. Errani, Annalisa Trama, Andreas Leithner, Maria Abbondanza Pantaleo, Dirk C. Strauss, Christopher D.M. Fletcher, F. le Grange, W.J. van Houdt, Judith V.M.G. Bovée, Rick L. Haas, J. Gutkovich, Heikki Joensuu, J. Martin Broto, François Gouin, A.M. Frezza, Elizabeth H. Baldini, A. Le Cesne, Akmal Safwat, Armelle Dufresne, Susanne Scheipl, W.T.A. van der Graaf, Shreyaskumar Patel, J. Wood, Robin L. Jones, M. Molinari, Stefan S. Bielack, K. Sundby Hall, Jean-Yves Blay, Nadia Hindi, Sandra J. Strauss, Sylvie Bonvalot, Christina Messiou, Virginia Ferraresi, V. Ravi, E. de Álava, Aisha Miah, Piotr Rutkowski, A. Lopez Pousa, William D. Tap, Sophie Piperno-Neumann, Rosalba Miceli, Augusto Caraceni, G. van Oortmerssen, Rebecca A. Gladdy, M. Wartenberg, A. Fedenko, Giovanni Grignani, Thomas Brodowicz, Michael Montemurro, Albiruni Ryan Abdul Razak, Dario Callegaro, Claudia Sangalli, Ioannis Boukovinas, Patrick Schöffski, M. Deoras-Sutliff, Chandrajit P. Raut, Jyoti Bajpai, C. Jungels, Andrea C. Ferrari, A. Tweddle, Hans Gelderblom, Brian P. Rubin, Nadia Zaffaroni, Paolo G. Casali, Carlo Morosi, M. A. J. van de Sande, P. Merriam, O. Merimsky, Cristina R. Antonescu, G. Sapisochin, Olivier Mir, Sebastian Bauer, Marta Sbaraglia, Bernd Kasper, Stacchiotti S., Miah A.B., Frezza A.M., Messiou C., Morosi C., Caraceni A., Antonescu C.R., Bajpai J., Baldini E., Bauer S., Biagini R., Bielack S., Blay J.Y., Bonvalot S., Boukovinas I., Bovee J.V.M.G., Boye K., Brodowicz T., Callegaro D., De Alava E., Deoras-Sutliff M., Dufresne A., Eriksson M., Errani C., Fedenko A., Ferraresi V., Ferrari A., Fletcher C.D.M., Garcia del Muro X., Gelderblom H., Gladdy R.A., Gouin F., Grignani G., Gutkovich J., Haas R., Hindi N., Hohenberger P., Huang P., Joensuu H., Jones R.L., Jungels C., Kasper B., Kawai A., Le Cesne A., Le Grange F., Leithner A., Leonard H., Lopez Pousa A., Martin Broto J., Merimsky O., Merriam P., Miceli R., Mir O., Molinari M., Montemurro M., Oldani G., Palmerini E., Pantaleo M.A., Patel S., Piperno-Neumann S., Raut C.P., Ravi V., Razak A.R.A., Reichardt P., Rubin B.P., Rutkowski P., Safwat A.A., Sangalli C., Sapisochin G., Sbaraglia M., Scheipl S., Schoffski P., Strauss D., Strauss S.J., Sundby Hall K., Tap W.D., Trama A., Tweddle A., van der Graaf W.T.A., Van De Sande M.A.J., Van Houdt W., van Oortmerssen G., Wagner A.J., Wartenberg M., Wood J., Zaffaroni N., Zimmermann C., Casali P.G., Dei Tos A.P., Gronchi A., University of Helsinki, Department of Oncology, Heikki Joensuu / Principal Investigator, and HUS Comprehensive Cancer Center

Subjects
Cancer Research, sarcoma, diagnosis, Medizin, Disease, HEPATIC EPITHELIOID HEMANGIOENDOTHELIOMA, Review, Medical Oncology, Patient advocacy, RISK STRATIFICATION, 0302 clinical medicine, PROPOSAL, CLINICAL CHARACTERISTICS, Medicine, guidelines, Child, 0303 health sciences, ddc:617, treatment, VASCULAR TUMORS, LIVER-TRANSPLANTATION, 3. Good health, IMAGING FINDINGS, diagnosi, epithelioid hemangioendothelioma, Oncology, OF-THE-LITERATURE, 030220 oncology & carcinogenesis, Hemangioendothelioma, Epithelioid, Sarcoma, guideline, management, Human, Adult, medicine.medical_specialty, Consensus, 3122 Cancers, Consensu, Patient Advocacy, Malignancy, Hepatic Epithelioid Hemangioendothelioma, 03 medical and health sciences, Humans, Intensive care medicine, Epithelioid hemangioendothelioma, 030304 developmental biology, business.industry, SOFT-TISSUE, medicine.disease, Rare cancer, Vascular Tumors, SARCOMAS, business
Abstract

Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication., Highlights • This consensus paper provides key recommendations on the management of epithelioid hemangioendothelioma (EHE). • Recommendations followed a consensus meeting between experts and a representative of the EHE advocacy group and SPAEN. • Authorship includes a multidisciplinary group of experts from different institutions from Europe, North America and Asia.

Published
2021

16. Outcome in dedifferentiated chondrosarcoma for patients treated with multimodal therapy: Results from the EUROpean Bone Over 40 Sarcoma Study

Author

Elisabetta Setola, Virginia Ferraresi, Peter Reichardt, Sigbjørn Smeland, Torsten Kessler, Rudolf Schwarz, Pierro Picci, Pietro Ruggieri, Ivar Hompland, Marco Gambarotti, Stefan S. Bielack, Eric L. Staals, Stefano Ferrari, Anne Flörcken, Lina Hansson, Otte Brosjö, Bodil Bjerkehagen, Thomas Kühne, Daniel Baumhoer, Emanuela Palmerini, Roberto Biagini, Alessandro Comandone, Matthias Kevric, Kirsten Sundby Hall, Gaetano Apice, Stefanie Hecker-Nolting, Rossella Bertulli, Paolo G. Casali, Gerda Hofmann-Wackersreuther, Mikael Eriksson, Claudia Blattmann, Davide Maria Donati, Fatime Krasniqi, Leo Kager, Björn N. Heydrich, Hompland I., Ferrari S., Bielack S., Palmerini E., Hall K.S., Picci P., Hecker-Nolting S., Donati D.M., Blattmann C., Bjerkehagen B., Staals E., Kager L., Gambarotti M., Kuhne T., Eriksson M., Ferraresi V., Kevric M., Biagini R., Baumhoer D., Brosjo O., Comandone A., Schwarz R., Bertulli R., Kessler T., Hansson L., Apice G., Heydrich B.-N., Setola E., Florcken A., Ruggieri P., Krasniqi F., Hofmann-Wackersreuther G., Casali P., Reichardt P., and Smeland S.

Subjects
0301 basic medicine, Oncology, Male, Cancer Research, Time Factors, Survival, medicine.medical_treatment, Systemic treatment, 0302 clinical medicine, Bone sarcoma, Antineoplastic Combined Chemotherapy Protocols, Chemotherapy, Complete surgical remission, Dedifferentiated chondrosarcoma, EURO-B.O.S.S, Sarcoma, Medicine, Prospective Studies, Ifosfamide, Multimodal therapy, Middle Aged, Chemotherapy regimen, Neoadjuvant Therapy, Europe, Chemotherapy, Adjuvant, 030220 oncology & carcinogenesis, Female, medicine.drug, Adult, medicine.medical_specialty, Chondrosarcoma, Bone Neoplasms, Disease-Free Survival, 03 medical and health sciences, Internal medicine, Humans, Doxorubicin, Aged, business.industry, Cancer, Cell Dedifferentiation, medicine.disease, 030104 developmental biology, Feasibility Studies, Methotrexate, business
Abstract

Introduction The role of chemotherapy for patients with dedifferentiated chondrosarcoma (DDCS) is still under discussion. Here, we present the outcome in patients with DDCS treated with intensive chemotherapy from the EUROpean Bone Over 40 Sarcoma Study. Materials and methods The chemotherapy regimen included doxorubicin, ifosfamide and cisplatin. Postoperative methotrexate was added in case of poor histological response. Toxicity was graded based on the National Cancer Institute expanded common toxicity criteria, version 2.0, and survival was analysed using Kaplan-Meier curves, log-rank tests and univariate Cox regression models. Results Fifty-seven patients with DDCS (localised, 34 [60%]; metastatic, 23 [40%]) aged 42–65 years were included. Surgical complete remission (SCR) was achieved in 36 (63%) patients. The median overall survival (OS) was 24 months (95% confidence interval, 22–25), and the 5-year OS was 39%. Patients with extremity localisation had a 5-year OS of 49% compared with 29% in patients with a central tumour (P = 0.08). Patients with localised disease had a 5-year OS of 46%, whereas patients with metastatic disease had a 5-year OS of 29% (P = 0.12). Patients in SCR had a 5-year OS of 49%, whereas patients not in SCR had a 5-year OS of 23% (P = 0.004). Chemotherapy toxicity was considerable but manageable. There was no treatment-related death, and 39 (70%) patients received ≥6 cycles of the planned nine chemotherapy cycles. Conclusions Adding intensive chemotherapy to surgery for treatment of DDCS is feasible and shows favourable survival data compared with previous reports. With the limitations of data from a non-controlled trial, we conclude that chemotherapy could be considered in the management of patients aged >40 years.

Published
2021

17. Bone sarcomas: ESMO-PaedCan-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up

Author

Laurence Brugières, K. Sundby Hall, Bruce Morland, Ioannis Boukovinas, Rolf D. Issels, Sebastian Bauer, D.A. Krakorova, Angela Buonadonna, E. de Álava, Nathalie Gaspar, P. Picci, Palma Dileo, Hans Gelderblom, Bernadette Brennan, Javier Martin Broto, Maria Abbondanza Pantaleo, Paul C Jutte, Ian Judson, Suzanne E. J. Kaal, B. Hassan, Ruth Ladenstein, Alexander Fedenko, Patrick Schöffski, Jean-Yves Blay, A. Le Cesne, Peter Hohenberger, Virginia Ferraresi, Sandra J. Strauss, Michael Montemurro, Robin L. Jones, Peter Reichardt, Stefan S. Bielack, Mikael Eriksson, Stefan Sleijfer, Akmal Safwat, Heikki Joensuu, Rick L. Haas, Bernd Kasper, Hannu T. Aro, Judith V.M.G. Bovée, Jeremy Whelan, A.M. Frezza, Antonio López Pousa, Stefanie Hecker-Nolting, Thomas Brodowicz, R. Biagini, Silvia Stacchiotti, S. Ferrari, Sylvie Bonvalot, S. Piperno-Neumann, Leo Kager, F. van Coevorden, Olga Zaikova, R. Piana, Catharina Dhooge, Piotr Rutkowski, M.H. Robinson, Ofer Merimsky, Katerina Kopeckova, X.G. del Muro, W.T.A. van der Graaf, Paolo G. Casali, N. Abecassis, Eva Wardelmann, Silvia Gasperoni, Giovanni Grignani, A. P. Dei Tos, Andrea Ferrari, Franca Fagioli, Alessandro Gronchi, Thierry Gil, Iwona Lugowska, M. Unk, Man, Biomaterials and Microbes (MBM), Public Health Research (PHR), ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Medical Oncology, 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.

Subjects
0301 basic medicine, Oncology, Biopsy, Medizin, Aftercare, CHILDRENS-ONCOLOGY-GROUP, Survivorship, Medical Oncology, 0302 clinical medicine, HIGH-DOSE CHEMOTHERAPY, Positron Emission Tomography Computed Tomography, Antineoplastic Combined Chemotherapy Protocols, GIANT-CELL TUMOR, Orthopedic Procedures, PRIMITIVE NEUROECTODERMAL TUMOR, Child, Societies, Medical, Osteosarcoma, SOFT-TISSUE TUMORS, Incidence (epidemiology), Incidence, Age Factors, Hematology, RANDOMIZED CONTROLLED-TRIAL, Magnetic Resonance Imaging, Neoadjuvant Therapy, Europe, Self-Help Groups, Treatment Outcome, 030220 oncology & carcinogenesis, Sarcoma, HIGH-GRADE OSTEOSARCOMA, Rare cancers Radboud Institute for Health Sciences [Radboudumc 9], Adult, medicine.medical_specialty, education, Bone Neoplasms, Bone Sarcoma, Bone and Bones, 03 medical and health sciences, POSITRON-EMISSION-TOMOGRAPHY, Internal medicine, medicine, Humans, Radionuclide Imaging, STUDY-GROUP PROTOCOLS, Neoplasm Staging, ta3126, NONMETASTATIC EWINGS-SARCOMA, business.industry, Cancer, medicine.disease, Long-Term Care, Cancer registry, 030104 developmental biology, Primitive neuroectodermal tumor, Radiotherapy, Adjuvant, Chondrosarcoma, Patient Participation, business
Abstract

Primary bone tumours are rare, accounting for < 0.2% of malignant neoplasms registered in the EUROCARE (European Cancer Registry based study on survival and care of cancer patients) database. Different bone tumour subtypes have distinct patterns of incidence, and each has no more than 0.3 incident cases per 100 000 per year. Osteosarcoma (OS) and Ewing sarcoma (ES) have a relatively high incidence in the second decade of life, whereas chondrosarcoma (CS) is more common in older age.

Published
2018

18. Osteosarcoma in very young children: experience of the Cooperative Osteosarcoma Study Group

Author

Gernot Jundt, Leo Kager, Susanna Lang, Nicole Bodmer, T. Klingebiel, Helmut Gadner, Stefan S. Bielack, Heribert Jürgens, Martin Dominkus, Andreas Zoubek, University of Zurich, and Bielack, S

Subjects
musculoskeletal diseases, Oncology, Male, Cancer Research, medicine.medical_specialty, Treatment outcome, 610 Medicine & health, Bone Neoplasms, Internal medicine, medicine, Humans, 1306 Cancer Research, Neoplasm Metastasis, neoplasms, Leg, Osteosarcoma, business.industry, medicine.disease, Treatment Outcome, 10036 Medical Clinic, Chemotherapy, Adjuvant, Child, Preschool, Physical therapy, Arm, 2730 Oncology, Female, business
Abstract

This study was conducted to investigate presentation, treatment, and outcome in very young children with osteosarcoma.The authors retrospectively analyzed the data of 2706 consecutive COSS patients with newly diagnosed osteosarcoma and identified 28 (1.0%) patients aged younger than 5 years at diagnosis. Demographic, diagnostic, tumor, treatment-related variables, response, and survival data were analyzed.Of the 28 preschoolers, 27 presented with high-grade central osteosarcoma of an extremity, and 1 had a secondary osteosarcoma of the orbit. This analysis focused on the 27 patients with extremity tumors. The size of the primary was large (≥one-third of the involved bone) in 20 of 27 patients. Primary metastases were detected in 4 of 27 children. All patients received multiagent chemotherapy, and 11 of 18 analyzed tumors responded well (90% necrosis) to neoadjuvant chemotherapy. Limb-sparing surgery was performed in 9 cases, ablative procedures were performed in 15, and, in 3 cases, no local surgery was performed. With a median follow-up of 4 years (6.2 years for survivors), 13 patients were alive. Four patients never achieved a complete remission, and 11 developed recurrences; 14 of these 15 patients died. Five-year overall and event-free survival probabilities were 51% (standard error of the mean [SE], 10%) and 48% (SE, 10%). Better survival was correlated with good response to chemotherapy and later time period of diagnosis.Osteosarcoma is extremely rare in preschool children. These patients often have large tumors that may require mutilating resections. Prognosis is in the range of that reported for older patients.

Published
2010

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