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29 results on '"Baldi, Giacomo G."'

2. A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl)

Author

Palmerini, Emanuela, Gambarotti, Marco, Italiano, Antoine, Nathenson, Michael J., Ratan, Ravin, Dileo, Palma, Provenzano, Salvatore, Jones, Robin L., DuBois, Steven G., Martin-Broto, Javier, de Alava, Enrique, Baldi, Giacomo G., Grignani, Giovanni, Ferraresi, Virginia, Brunello, Antonella, Paoluzzi, Luca, Bertulli, Rossella, Hindi, Nadia, Montemurro, Michael, Rothermundt, Christian, Cocchi, Stefania, Salguero-Aranda, Carmen, Donati, Davide, Martin, Juan D., Abdelhamid Ahmed, Amr H., Mazzocca, Alessandro, Carretta, Elisa, Cesari, Marilena, Pierini, Michela, Righi, Alberto, Sbaraglia, Marta, Laginestra, Maria A., Scotlandi, Katia, Dei Tos, Angelo P., Ibrahim, Toni, Stacchiotti, Silvia, and Vincenzi, Bruno

Published
2023
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3. The observational EURACAN prospective clinical registry dedicated to epithelioid hemangioendothelioma: The protocol of an international and collaborative effort on an ultra-rare entity.

Author

Frezza, Anna Maria, Leonard, Hugh, Aggerholm-Pedersen, Ninna, Badalamenti, Giuseppe, Baili, Paolo, Baldi, Giacomo G., Bauer, Sebastian, Bazzurri, Serena, Benzonelli, Irene, Bertuzzi, Alexia, Blay, Jean-Yves, Bianchi, Giuseppe, Bonfarnuzzo, Simone, Bouvier, Christophe, Boye, Kyetil, Martin Broto, Javier, Brunello, Antonella, Campanacci, Domenico, Casali, Paolo G., and Cicala, Carlo

Subjects
DISEASE relapse, CANCER relapse, OVERALL survival, PROPORTIONAL hazards models, MEDICAL records, NATURAL history
Abstract

Introduction: Epithelioid hemangioendothelioma (EHE) is an ultra-rare sarcoma, marked by distinctive molecular and pathological features and with a variable clinical behavior. Its natural history is still partially understood, reliable prognostic and predictive factors are lacking and many questions are still open on the optimal management. In the context of EURACAN, a prospective registry specifically dedicated to EHE was developed and launched with the aim of providing, through high-quality prospective data collection, a better understanding of this disease. Study design: Registry-based cohort study including only new cases of patients with a pathological and molecularly confirmed diagnosis of EHE Objectives: To improve the understanding of EHE natural history, validate and identify new prognostic and predictive factors, clarify the activity and efficacy of currently available treatment options, describe treatment pattern. Methods: Settings and participants: It is an hospital-based registry established in centers with expertise in EHE including adult patients with a new pathological and molecularly confirmed diagnosis of EHE starting from the 1st December 2023. The characteristics of each patient in the facility who meets the above-mentioned inclusion criteria will be collected prospectively and longitudinally with follow-up at cancer progression and / or cancer relapse or patient death. It is a secondary use of data which will be collected from the clinical records. The data collected for the registry will not entail further examinations or admissions to the facility and/or additional appointments to those normally provided for routine patient follow-up. Variables: Full details on patients and disease features, treatment and outcome will be collected, according to common clinical practice guidelines developed and shared with all the contributing centers. In addition, data on potential confounders (e.g. comorbidity; functional status etc.) will also be collected. Statistical methods: The data analyses will include descriptive statistics and analytical analyses. Multivariable Cox's proportional hazards model and Hazard ratios (HR) for all-cause or cause-specific mortality will be used to determine independent predictors of overall survival, recurrence and progression. Results: The registry has been joined by 21 sarcoma reference centers across EU and UK, covering 10 countries. Patients' recruitment started in December 2023. The estimated completion date is December 2033 upon agreement on the achievement of all the registry objectives. The already established collaboration and participation of EHE patient's associations involved in the project will help in promoting the registry and fostering accrual. [ABSTRACT FROM AUTHOR]

Published
2024
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4. Corrigendum to ‘Chemotherapy in patients with localized angiosarcoma of any site: A retrospective european study’ [Eur J Cancer 171 (2022) 183–192]

Author

Conforti, Fabio, primary, Gronchi, Alessandro, additional, Penel, Nicholas, additional, Jones, Robin L., additional, Broto, Javier M., additional, Sala, Isabella, additional, Bagnardi, Vincenzo, additional, Napolitano, Andrea, additional, Pala, Laura, additional, Pennacchioli, Elisabetta, additional, Catania, Chiara, additional, Queirolo, Paola, additional, Grigani, Giovanni, additional, Merlini, Alessandra, additional, Stacchiotti, Silvia, additional, Comandone, Alessandro, additional, Vincenzi, Bruno, additional, Quagliuolo, Vittorio, additional, Bertuzzi, Alexia, additional, Boglione, Antonella, additional, Palassini, Elena, additional, Baldi, Giacomo G., additional, Blay, Jean-Yves, additional, Ryckewaert, Thomas, additional, Toulmonde, Maud, additional, Italiano, Antoine, additional, Le Cesne, Axel, additional, Ray-Coquard, Isabelle, additional, Cruz, Josefina, additional, Hernández-León, Carmen N., additional, Trufero, Javier M., additional, da Silva Moura, David, additional, Muñiz, Nadia H., additional, and De Pas, Tommaso, additional

Published
2023
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5. A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl)

Author

Fondazione Carisbo, Palmerini, Emanuela, Gambarotti, Marco, Italiano, Antoine, Nathenson, Michael J., Ratan, Ravin, Dileo, Palma, Provenzano, Salvatore, Jones, Robin L., DuBois, Steven G., Martín-Broto, Javier, Álava, Enrique de, Baldi, Giacomo G., Grignani, Giovanni, Ferraresi, Virginia, Brunello, Antonella, Paoluzzi, Luca, Bertulli, Rossella, Hindi, Nadia, Montemurro, Michael, Rothermundt, Christian, Cocchi, Stefania, Salguero-Aranda, Carmen, Donati, Davide María, Martin, Juan D., Abdelhamid Ahmed, Amr H., Mazzocca, Alessandro, Carretta, Elisa, Cesari, Marilena, Pierini, Michela, Righi, Alberto, Sbaraglia, Marta, Laginestra, Maria A., Scotlandi, Katia, Dei Tos, Angelo Paolo, Ibrahim, Toni, Stacchiotti, Silvia, Vincenzi, Bruno, Fondazione Carisbo, Palmerini, Emanuela, Gambarotti, Marco, Italiano, Antoine, Nathenson, Michael J., Ratan, Ravin, Dileo, Palma, Provenzano, Salvatore, Jones, Robin L., DuBois, Steven G., Martín-Broto, Javier, Álava, Enrique de, Baldi, Giacomo G., Grignani, Giovanni, Ferraresi, Virginia, Brunello, Antonella, Paoluzzi, Luca, Bertulli, Rossella, Hindi, Nadia, Montemurro, Michael, Rothermundt, Christian, Cocchi, Stefania, Salguero-Aranda, Carmen, Donati, Davide María, Martin, Juan D., Abdelhamid Ahmed, Amr H., Mazzocca, Alessandro, Carretta, Elisa, Cesari, Marilena, Pierini, Michela, Righi, Alberto, Sbaraglia, Marta, Laginestra, Maria A., Scotlandi, Katia, Dei Tos, Angelo Paolo, Ibrahim, Toni, Stacchiotti, Silvia, and Vincenzi, Bruno

Abstract

[Background] Undifferentiated small round cell sarcomas (URCSs) represent a diagnostic challenge, and their optimal treatment is unknown. We aimed to define the clinical characteristics, treatment, and outcome of URCS patients., [Methods] URCS patients treated from 1983 to 2019 at 21 worldwide sarcoma reference centres were retrospectively identified. Based on molecular assessment, cases were classified as follows: (1) CIC-rearranged round cell sarcomas, (2) BCOR::CCNB3-rearranged round cell sarcomas, (3) unclassified URCSs. Treatment, prognostic factors and outcome were reviewed., [Results] In total, 148 patients were identified [88/148 (60%) CIC-rearranged sarcoma (median age 32 years, range 7–78), 33/148 (22%) BCOR::CCNB3-rearranged (median age 17 years, range 5–91), and 27/148 (18%) unclassified URCSs (median age 37 years, range 4–70)]. One hundred-one (68.2%) cases presented with localised disease; 47 (31.8%) had metastases at diagnosis. Male prevalence, younger age, bone primary site, and a low rate of synchronous metastases were observed in BCOR::CCNB3-rearranged cases. Local treatment was surgery in 67/148 (45%) patients, and surgery + radiotherapy in 52/148 (35%). Chemotherapy was given to 122/148 (82%) patients. At a 42.7-month median follow-up, the 3-year overall survival (OS) was 92.2% (95% CI 71.5–98.0) in BCOR::CCNB3 patients, 39.6% (95% CI 27.7–51.3) in CIC-rearranged sarcomas, and 78.7% in unclassified URCSs (95% CI 56.1–90.6; p < 0.0001)., [Conclusions] This study is the largest conducted in URCS and confirms major differences in outcomes between URCS subtypes. A full molecular assessment should be undertaken when a diagnosis of URCS is suspected. Prospective studies are needed to better define the optimal treatment strategy in each URCS subtype.

Published
2023

6. Retrospective observational studies in ultra-rare sarcomas: A consensus paper from the Connective Tissue Oncology Society (CTOS) community of experts on the minimum requirements for the evaluation of activity of systemic treatments

Author

Stacchiotti, Silvia, primary, Maria Frezza, Anna, additional, Demetri, George D., additional, Blay, Jean-Yves, additional, Bajpai, Jyoti, additional, Baldi, Giacomo G., additional, Baldini, Elizabeth H., additional, Benjamin, Robert S., additional, Bonvalot, Sylvie, additional, Bovée, Judith V.M.G., additional, Callegaro, Dario, additional, Casali, Paolo G., additional, D'Angelo, Sandra P., additional, Davis, Elizabeth J., additional, Dei Tos, Angelo P., additional, Demicco, Elizabeth G., additional, Desai, Jayesh, additional, Dileo, Palma, additional, Eriksson, Mikael, additional, Gelderblom, Hans, additional, George, Suzanne, additional, Gladdy, Rebecca A., additional, Gounder, Mrinal M., additional, Gupta, Abha A., additional, Haas, Rick, additional, Hayes, Andrea, additional, Hohenberger, Peter, additional, Jones, Kevin B., additional, Jones, Robin L., additional, Kasper, Bernd, additional, Kawai, Akira, additional, Kirsch, David G., additional, Kleinerman, Eugenie S., additional, Le Cesne, Axel, additional, Maestro, Roberta, additional, Martin Broto, Javier, additional, Maki, Robert G., additional, Miah, Aisha B., additional, Palmerini, Emanuela, additional, Patel, Shreaskumar R., additional, Raut, Chandrajit P., additional, Razak, Albiruni R.A., additional, Reed, Damon R., additional, Rutkowski, Piotr, additional, Sanfilippo, Roberta G., additional, Sbaraglia, Marta, additional, Schaefer, Inga-Marie, additional, Strauss, Dirk C., additional, Strauss, Sandra J., additional, Tap, William D., additional, Thomas, David M., additional, Trama, Annalisa, additional, Trent, Jonathan C., additional, van der Graaf, Winette T.A., additional, van Houdt, Winan J., additional, von Mehren, Margaret, additional, Wilky, Breelyn A., additional, Fletcher, Christopher D.M., additional, Gronchi, Alessandro, additional, Miceli, Rosalba, additional, and Wagner, Andrew J., additional

Published
2022
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7. Chemotherapy in patients with localized angiosarcoma of any site: A retrospective european study

Author

Conforti, Fabio, primary, Gronchi, Alessandro, additional, Penel, Nicholas, additional, Jones, Robin L., additional, Broto, Javier M., additional, Sala, Isabella, additional, Bagnardi, Vincenzo, additional, Napolitano, Andrea, additional, Pala, Laura, additional, Pennacchioli, Elisabetta, additional, Catania, Chiara, additional, Queirolo, Paola, additional, Grigani, Giovanni, additional, Merlini, Alessandra, additional, Stacchiotti, Silvia, additional, Comandone, Alessandro, additional, Vincenzi, Bruno, additional, Quagliuolo, Vittorio, additional, Bertuzzi, Alexia, additional, Boglione, Antonella, additional, Palassini, Elena, additional, Baldi, Giacomo G., additional, Blay, Jean-Yves, additional, Ryckewaert, Thomas, additional, Toulmonde, Maud, additional, Italiano, Antoine, additional, Le Cesne, Axel, additional, Ray-Coquard, Isabelle, additional, Cruz, Josefina, additional, Hernández-León, Carmen N., additional, Trufero, Javier M., additional, da Silva Moura, David, additional, Muñiz, Nadia H., additional, and De Pas, Tommaso, additional

Published
2022
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9. Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case‐series analysis from the Italian Rare Cancers Network

Author

Baldi, Giacomo G., primary, Lo Vullo, Salvatore, additional, Grignani, Giovanni, additional, Vincenzi, Bruno, additional, Badalamenti, Giuseppe, additional, Mastore, Marinella, additional, Buonomenna, Ciriaco, additional, Morosi, Carlo, additional, Barisella, Marta, additional, Frezza, Anna Maria, additional, Provenzano, Salvatore, additional, Simeone, Noemi, additional, Picozzi, Fernanda, additional, Mariani, Luigi, additional, Casali, Paolo G., additional, and Stacchiotti, Silvia, additional

Published
2022
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10. Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature

Author

Frezza, Anna M., primary, Ravi, Vinod, additional, Lo Vullo, Salvatore, additional, Vincenzi, Bruno, additional, Tolomeo, Francesco, additional, Chen, Tom Wei‐Wu, additional, Teterycz, Pawel, additional, Baldi, Giacomo G., additional, Italiano, Antoine, additional, Penel, Nicolas, additional, Brunello, Antonella, additional, Duffaud, Florance, additional, Hindi, Nadia, additional, Iwata, Shintaro, additional, Smrke, Alannah, additional, Fedenko, Alexander, additional, Gelderblom, Hans, additional, Van Der Graaf, Winette, additional, Vozy, Aurore, additional, Connolly, Elizabeth, additional, Grassi, Massimiliano, additional, Benjamin, Robert S., additional, Broto, Javier‐Martin, additional, Grignani, Giovanni, additional, Jones, Robin L., additional, Kawai, Akira, additional, Tysarowski, Andrzej, additional, Mariani, Luigi, additional, Casali, Paolo G., additional, and Stacchiotti, Silvia, additional

Published
2021
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11. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case‐series analysis within the Italian Rare Cancer Network

Author

Stacchiotti, Silvia, primary, Simeone, Noemi, additional, Lo Vullo, Salvatore, additional, Baldi, Giacomo G., additional, Brunello, Antonella, additional, Vincenzi, Bruno, additional, Palassini, Elena, additional, Dagrada, GianPaolo, additional, Collini, Paola, additional, Morosi, Carlo, additional, Greco, Francesca G., additional, Sbaraglia, Marta, additional, Dei Tos, Angelo P., additional, Mariani, Luigi, additional, Frezza, Anna Maria, additional, and Casali, Paolo G., additional

Published
2020
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13. Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network

Author

Frezza, Anna Maria, primary, Assi, Tarek, additional, Lo Vullo, Salvatore, additional, Ben‐Ami, Eytan, additional, Dufresne, Armelle, additional, Yonemori, Kan, additional, Noguchi, Emi, additional, Siontis, Brittany, additional, Ferraro, Richard, additional, Teterycz, Pawel, additional, Duffaud, Florence, additional, Ravi, Vinod, additional, Vincenzi, Bruno, additional, Gelderblom, Hans, additional, Pantaleo, Maria A., additional, Baldi, Giacomo G., additional, Desar, Ingrid, additional, Fedenko, Alexander, additional, Maki, Robert G., additional, Jones, Robin L., additional, Benjamin, Robert S., additional, Blay, Jean Yves, additional, Kawai, Akira, additional, Gounder, Mrinal, additional, Gronchi, Alessandro, additional, Le Cesne, Axel, additional, Mir, Olivier, additional, Czarnecka, Anna M., additional, Schuetze, Scott, additional, Wagner, Andrew J., additional, Adam, Julien, additional, Barisella, Marta, additional, Sbaraglia, Marta, additional, Hornick, Jason L., additional, Meurgey, Alexandra, additional, Mariani, Luigi, additional, Casali, Paolo G., additional, Thornton, Katherine, additional, and Stacchiotti, Silvia, additional

Published
2019
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15. Advanced epithelioid haemangioendotelioma: Fever, pain, and pleural effusion predict a worse outcome.

Author

Vincenzi, Bruno, primary, Frezza, Anna Maria, additional, Brunello, Antonella, additional, D'Ambrosio, Lorenzo, additional, Baldi, Giacomo G., additional, Badalamenti, Giuseppe, additional, Grignani, Giovanni, additional, Napolitano, Andrea, additional, Valeri, Sergio, additional, Improta, Luca, additional, Spalato Ceruso, Mariella, additional, Silletta, Marianna, additional, Dei Tos, Angelo Paolo, additional, Gronchi, Alessandro, additional, and Stacchiotti, Silvia, additional

Published
2019
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17. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case‐series analysis within the Italian Rare Cancer Network.

Author

Stacchiotti, Silvia, Simeone, Noemi, Lo Vullo, Salvatore, Baldi, Giacomo G., Brunello, Antonella, Vincenzi, Bruno, Palassini, Elena, Dagrada, GianPaolo, Collini, Paola, Morosi, Carlo, Greco, Francesca G., Sbaraglia, Marta, Dei Tos, Angelo P., Mariani, Luigi, Frezza, Anna Maria, and Casali, Paolo G.

Subjects
RAPAMYCIN, PROGRESSION-free survival, DISEASE progression, TRANSCRIPTION factors, DIAGNOSIS, MENSTRUATION disorders
Abstract

Background: The objective of this study was to report on a retrospective series of patients with epithelioid hemangioendothelioma (EHE) who received treatment with sirolimus within the Italian Rare Cancer Network. Methods: From January 2005, 38 adult patients with advanced EHE received continuous‐dosing sirolimus, 5 mg daily, until they developed either toxicity or disease progression. Disease progression in the 6 months before the start of treatment was required. Each pathologic diagnosis was reviewed. The daily dose of sirolimus was adjusted based on plasma levels. Response was retrospectively assessed by local investigators using Response Evaluation Criteria in Solid Tumors, version 1.1 (RECIST). Survival was estimated using the Kaplan‐Meier method. Results: All 38 patients (WW Domain Containing Transcription Regulator 1 [WWTR1]‐positive, n = 37; transcription factor E3 [TFE3]‐positive, n = 1) had disease progression before starting sirolimus (at baseline, 13 of 38 patients had the presence of serosal effusions and systemic symptoms). Thirty‐seven patients were evaluable for response (there was 1 early interruption). The best RECIST responses were a partial response in 4 patients (10.8%), stable disease in 28 patients (75.7%), and disease progression in 5 patients (13.5%). At a 41.5‐month median follow‐up (interquartile range [IQR], 23.9‐56.8 months), the median PFS was 13 months (95% CI, 3.7 months to not estimated [NE]), and the median OS was 18.8 months (95% CI, 10.6 months to NE). In patients who had serosal effusions at baseline, the median PFS was 4.8 months (IQR, 3.5‐11.7 months), and the median OS was 10.6 months (IQR, 5.1‐13.0 months), compared with 47.8 months (IQR, 11.4 months to NE) and 47.8 months (IQR, 15.7 months to NE), respectively, in patients without serosal effusions. Overall, sirolimus was fairly well tolerated, with 10 patients reporting irregular menstruation/ovary disfunction. Conclusions: The current results confirm that sirolimus is active in EHE, leading to prolonged stabilization in most patients who present without serosal effusions. Serosal effusions are confirmed as an unfavorable prognostic sign associated with short survival, and sirolimus displays limited activity in this subgroup. In a large retrospective study of systemic treatment for epithelioid hemangioendothelioma, sirolimus achieved prolonged disease stabilization in patients who previously had progressive, advanced disease, with a 10% overall risk ratio according to RECIST, an overall median progression‐free survival of 13 months, and a median progression‐free survival of 47.8 months in patients without serosal effusion. In patients who had serosal effusion, however, sirolimus had limited activity, and patients had a poor prognosis, with a median survival less than 1 year. [ABSTRACT FROM AUTHOR]

Published
2021
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18. Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network.

Author

Frezza, Anna Maria, Assi, Tarek, Lo Vullo, Salvatore, Ben‐Ami, Eytan, Dufresne, Armelle, Yonemori, Kan, Noguchi, Emi, Siontis, Brittany, Ferraro, Richard, Teterycz, Pawel, Duffaud, Florence, Ravi, Vinod, Vincenzi, Bruno, Gelderblom, Hans, Pantaleo, Maria A., Baldi, Giacomo G., Desar, Ingrid, Fedenko, Alexander, Maki, Robert G., and Jones, Robin L.

Subjects
SARCOMA, PROGRESSION-free survival, ANTHRACYCLINES, HEART tumors, CARDIOTOXICITY, PROTEINS, RESEARCH, BLOOD vessels, HETEROCYCLIC compounds, RESEARCH methodology, ANTINEOPLASTIC agents, DEOXYCYTIDINE, PROGNOSIS, EVALUATION research, MEDICAL cooperation, TREATMENT effectiveness, COMPARATIVE studies, SULFONAMIDES
Abstract

Background: Intimal sarcoma (InS) is an exceedingly rare neoplasm with an unfavorable prognosis, for which new potentially active treatments are under development. We report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with InS.Methods: Seventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis. Patients with MDM2-positive InS who were treated with anthracycline-based regimens, gemcitabine-based regimens, or pazopanib between October 2001 and January 2018 were selected. Local pathological review was performed to confirm diagnosis. Response was assessed by RECIST1.1. Recurrence-free survival (RFS), progression-free survival (PFS) and overall survival were computed by Kaplan-Meier method.Results: Seventy-two patients were included (66 anthracycline-based regimens; 26 gemcitabine-based regimens; 12 pazopanib). In the anthracycline-based group, 24 (36%) patients were treated for localized disease, and 42 (64%) patients were treated for advanced disease. The real-world overall response rate (rwORR) was 38%. For patients with localized disease, the median RFS was 14.6 months. For patients with advanced disease, the median PFS was 7.7 months. No anthracycline-related cardiac toxicity was reported in patients with cardiac InS (n = 26). For gemcitabine and pazopanib, the rwORR was 8%, and the median PFS was 3.2 and 3.7 months, respectively.Conclusion: This retrospective series shows the activity of anthracycline-based regimens in InS. Of note, anthracyclines were used in patients with cardiac InS with no significant cardiac toxicity. The prognosis in patients with InS remains poor, and new active drugs and treatment strategies are needed. [ABSTRACT FROM AUTHOR]

Published
2020
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21. Trabectedin in advanced synovial sarcomas: A multicenter retrospective study from four European institutions and the Italian Rare Cancer Network

Author

Sanfilippo, R., Dileo, Palma, Blay, Jean-Yves, Constantinidou, Anastasia, Cesne, Axel Le, Benson, Charlotte, Vizzini, L., Contu, Marianna, Baldi, Giacomo G., P, Angelo Dei Tos, Casali, Paolo G., and Constantinidou, Anastasia [0000-0001-5316-7574]

Subjects
Oncology, Adult, Male, medicine.medical_specialty, Cancer Research, Adolescent, medicine.medical_treatment, Antineoplastic Agents, Dioxoles, chemotherapy, synovial sarcoma, Clinical Reports, Synovial sarcoma, Sarcoma, Synovial, Internal medicine, Tetrahydroisoquinolines, medicine, Chemotherapy, Humans, Pharmacology (medical), Trabectedin, Aged, Retrospective Studies, Metastatic Synovial Sarcoma, Pharmacology, Soft tissue sarcoma, Ifosfamide, Synovial, business.industry, Medicine (all), Retrospective cohort study, Sarcoma, Middle Aged, medicine.disease, Surgery, soft tissue sarcoma, trabectedin, Female, business, medicine.drug
Abstract

Treatment options for patients with metastatic synovial sarcoma are limited. Over recent years, trabectedin has emerged as an effective agent for patients with advanced soft tissue sarcomas resistant to anthracyclines and ifosfamide. The aim of this retrospective analysis was to study the efficacy of trabectedin in the subgroup of synovial sarcomas. A retrospective analysis was carried out on patients with advanced synovial sarcoma treated with trabectedin at four European reference sarcoma centers and within the Italian Rare Cancer Network between 2000 and 2013. Radiological response, progression-free, and overall survival, as well as serious and unexpected adverse events were retrospectively assessed. Sixty-one patients with metastatic synovial sarcoma were identified. The median number of previous chemotherapy regimens was 2 (range 1-6). Nine patients had a partial response, in addition to two minor responses, and 19 patients had stable disease, for an overall response rate of 15% and a tumor control rate of 50%. The median progression-free survival was 3 months, with 23% of patients free from progression at 6 months. The median progression-free survival in responding patients was 7 months. Trabectedin is a therapeutic option for palliative treatment of a subset of patients with metastatic synovial sarcoma. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. 26 6 678 681

Published
2015

23. Current status and unanswered questions on the use of Denosumab in giant cell tumor of bone

Author

Gaston, Czar Louie, primary, Grimer, Robert J., additional, Parry, Michael, additional, Stacchiotti, Silvia, additional, Dei Tos, Angelo Paolo, additional, Gelderblom, Hans, additional, Ferrari, Stefano, additional, Baldi, Giacomo G., additional, Jones, Robin L., additional, Chawla, Sant, additional, Casali, Paolo, additional, LeCesne, Axel, additional, Blay, Jean-Yves, additional, Dijkstra, Sander P. D., additional, Thomas, David M., additional, and Rutkowski, Piotr, additional

Published
2016
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24. Hormonal manipulation with toremifene in sporadic desmoid-type fibromatosis

Author

Fiore, Marco, primary, Colombo, Chiara, additional, Radaelli, Stefano, additional, Callegaro, Dario, additional, Palassini, Elena, additional, Barisella, Marta, additional, Morosi, Carlo, additional, Baldi, Giacomo G., additional, Stacchiotti, Silvia, additional, Casali, Paolo G., additional, and Gronchi, Alessandro, additional

Published
2015
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25. Trabectedin in advanced synovial sarcomas

Author

Sanfilippo, Roberta, primary, Dileo, Palma, additional, Blay, Jean-Yves, additional, Constantinidou, Anastasia, additional, Le Cesne, Axel, additional, Benson, Charlotte, additional, Vizzini, Laura, additional, Contu, Marianna, additional, Baldi, Giacomo G., additional, Dei Tos, Angelo P., additional, and Casali, Paolo G., additional

Published
2015
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26. Activity of chemotherapy in a series of patients with locally advanced or metastatic pleomorphic rhabdomyosarcoma: A retrospective analysis.

Author

Libertini, Michela, primary, Hindi, Nadia, additional, Provenzano, Salvatore, additional, Bertulli, Rossella, additional, Sanfilippo, Roberta, additional, Stacchiotti, Silvia, additional, Palassini, Elena, additional, Fumagalli, Elena, additional, Baldi, Giacomo G., additional, Vincenzi, Bruno, additional, Dei Tos, Angelo Paolo, additional, Gronchi, Alessandro, additional, and Casali, Paolo Giovanni, additional

Published
2014
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27. Solitary fibrous tumor of all sites: outcome of late recurrences in 14 patients

Author

Baldi, Giacomo G, primary, Stacchiotti, Silvia, additional, Mauro, Valentina, additional, Dei Tos, Angelo P, additional, Gronchi, Alessandro, additional, Pastorino, Ugo, additional, Duranti, Leonardo, additional, Provenzano, Salvatore, additional, Marrari, Andrea, additional, Libertini, Michela, additional, Pilotti, Silvana, additional, and Casali, Paolo G, additional

Published
2013
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28. Bone metastases in soft tissue sarcoma patients: A survey of natural, prognostic value, and treatment.

Author

Vincenzi, Bruno, primary, Santini, Daniele, additional, Schiavon, Gaia, additional, Frezza, Anna Maria, additional, Dileo, Palma, additional, Silletta, Marianna, additional, Bertoldo, Francesco, additional, Badalamenti, Giuseppe, additional, Baldi, Giacomo G., additional, Zovato, Stefania, additional, Berardi, Rossana, additional, Tucci, Marco, additional, Whelan, Jeremy, additional, Tirabosco, Roberto, additional, Dei Tos, Angelo Paolo, additional, and Tonini, Giuseppe, additional

Published
2012
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29. International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms: Outcome of Primary Localized Disease.

Author

Giani C, Salawu A, Ljevar S, Denu RA, Napolitano A, Palmerini E, Connolly EA, Ogura K, Wong DD, Scanferla R, Rosenbaum E, Bajpai J, Li ZC, Bae S, D'Ambrosio L, Bialick S, Wagner AJ, Lee ATJ, Koseła-Paterczyk H, Baldi GG, Brunello A, Lee YC, Loong HH, Boikos S, Campos F, Cicala CM, Maki RG, Hindi N, Figura C, Almohsen SS, Patel S, Jones RL, Ibrahim T, Karim R, Kawai A, Carey-Smith R, Boyle R, Taverna SM, Lazar AJ, Demicco EG, Bovee JVMG, Dei Tos AP, Fletcher C, Baumhoer D, Sbaraglia M, Schaefer IM, Miceli R, Gronchi A, and Stacchiotti S

Abstract

The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists. The primary endpoint was overall survival (OS). Secondary endpoints were crude cumulative incidence (CCI) of local recurrence (LR), CCI of distant metastases (DM), and post-metastases OS (p-OS). Two hundred ninety-four patients (239 LGFMS, 32 SEF, and 23 H-LGFMS/SEF) were identified. At a median(m-) follow-up (FU) of 57.1 months, 12/294 patients died. The 5- and 10-year OS were 99.0% and 95.9% in LGFMS, 86.2% and 67.0% in SEF, and 84.8% and 84.8% in H-LGFMS/SEF, respectively. Predictors of worse OS included pathology, age at surgery, systemic therapy, and radiotherapy. LR developed in 13/294 (4.4%) patients. The observed m-time to LR was 10.7 months. The 5- and 10-yr CCI-LR were 4.7% in LGFMS and 6.6% in SEF, respectively. There were no LR events in H-LGFMS/SEF. The sole predictor of higher risk of LR was histology. DM developed in 23/294 (7.8%) patients. The observed m-time to DM was 28.2 months. The 5- and 10-yr CCI-DM were 1.3% and 2.7% in LGMFS, 29.9% and 57.7% in SEF, 48.9% and 48.9% in H-LGFMS/SEF, respectively. Predictors of higher risk of DM were histology, systemic therapy, and radiotherapy. Primary localized LGFMS treated with complete surgical resection has an excellent prognosis, while about 50% of H-LGFMS/SEF and SEF develop DM within 5 to 10 years. Very long-term FU is needed to understand absolute cure rates., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Published
2024
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