1. Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients
- Author
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François Quenet, Christine Chevreau, J.-Y. Blay, Matthieu Faron, G. Ferron, Daniel Orbach, A. Le Cesne, E Nassif, S. Carrere, Antoine Italiano, François Bertucci, Jean-Baptiste Delhorme, Olivier Mir, Marc Pocard, Emmanuelle Bompas, Charles Honoré, Olivier Glehen, AIRPARIF - Surveillance de la qualité de l'air en Île-de-France, Department of Surgical Oncology Institut Claudius Regaud, Service d'Oncologie Médicale [Centre hospitalier Lyon Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Institut Bergonié [Bordeaux], UNICANCER, Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Carcinose Angiogenèse et Recherche Translationnelle, Angiogenese et recherche translationnelle (CART U965), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Recherche en Cancérologie de Montpellier (IRCM - U1194 Inserm - UM), CRLCC Val d'Aurelle - Paul Lamarque-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Claudius Regaud, Institut Gustave Roussy (IGR), Sarcome, Département de médecine oncologique [Gustave Roussy], Institut Gustave Roussy (IGR)-Institut Gustave Roussy (IGR), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Paoli-Calmettes, and Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Aix Marseille Université (AMU)
- Subjects
Male ,0301 basic medicine ,MESH: Combined Modality Therapy ,Desmoplastic small-round-cell tumor ,Survival ,medicine.medical_treatment ,[SDV]Life Sciences [q-bio] ,0302 clinical medicine ,MESH: Child ,MESH: Peritoneal Neoplasms ,Child ,Peritoneal Neoplasms ,MESH: Middle Aged ,Systemic chemotherapy ,Female sex ,Cytoreduction Surgical Procedures ,MESH: Follow-Up Studies ,Middle Aged ,Prognosis ,Combined Modality Therapy ,3. Good health ,Survival Rate ,Oncology ,MESH: Young Adult ,Child, Preschool ,030220 oncology & carcinogenesis ,Female ,Hyperthermic intraperitoneal chemotherapy ,Cure ,Adult ,medicine.medical_specialty ,Poor prognosis ,Adolescent ,MESH: Survival Rate ,education ,[SDV.BC]Life Sciences [q-bio]/Cellular Biology ,MESH: Prognosis ,Young Adult ,03 medical and health sciences ,medicine ,Overall survival ,Humans ,Chemotherapy ,MESH: Cytoreduction Surgical Procedures ,MESH: Patient Selection ,MESH: Hyperthermia, Induced ,Retrospective Studies ,MESH: Adolescent ,MESH: Humans ,business.industry ,Patient Selection ,MESH: Child, Preschool ,MESH: Adult ,MESH: Retrospective Studies ,Hyperthermia, Induced ,medicine.disease ,MESH: Desmoplastic Small Round Cell Tumor ,MESH: Male ,Surgery ,030104 developmental biology ,Chemotherapy, Cancer, Regional Perfusion ,Conventional PCI ,Desmoplastic small round cell tumor ,MESH: Chemotherapy, Cancer, Regional Perfusion ,business ,MESH: Female ,Follow-Up Studies - Abstract
Despite being associated with a very poor prognosis, long-term survivors across all series of Desmoplastic Small Round Cell Tumor (DSRCT) have been reported.To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis.All consecutive patients treated for DSRCT in nine French expert centers between 1991 and 2018 were retrospectively analyzed. Patients with a follow-up of less than 2 years were excluded and cure defined as being disease-free at least 5 years.100 pts were identified (median age 25 years, 89% male). 27 had distant metastases at diagnosis and 80 pts underwent upfront chemotherapy (CT). 71 pts were operated, 20 pts without prior CT). Surgery was macroscopically complete (CC0/1) in 50 pts. Hyperthermic intraperitoneal Chemotherapy (HIPEC) was administered during surgery in 15 pts 54 pts had postoperative CT and 26 pts had postoperative whole abdomino-pelvic RT (WAP-RT). After a median follow-up of 103 months (range 23-311), the median overall survival (OS) was 25 months. The 1- year, 3-year and 5-year OS rates were 90%, 35% and 4% respectively. 5 patients were considered cured after a median disease-free interval of 100 months (range 22-139). Predictive factors of cure were female sex (HR = 0.49, p = 0.014), median PCI12 (HR = 0.32, p = 0.0004), MD Anderson stage I (HR = 0.25, p 0.0001), CC0/1 (HR = 0.34, p 0.0001), and WAP-RT (HR = 0.36, p = 0.00013). HIPEC did not statistically improve survival.Cure in DSRCT is possible in 5% of patients and is best achieved combining systemic chemotherapy, complete cytoreductive surgery and WAP-RT. Despite aggressive treatment, recurrence is common and targeted therapies are urgently needed.
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- 2019
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