| Added by | pmartino |
|---|---|
| Group name | EquipePM |
| Item Type | Journal Article |
| Title | Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients |
| Creator | Honoré et al. |
| Author | C. Honoré |
| Author | J. B. Delhorme |
| Author | E. Nassif |
| Author | M. Faron |
| Author | G. Ferron |
| Author | E. Bompas |
| Author | O. Glehen |
| Author | A. Italiano |
| Author | F. Bertucci |
| Author | D. Orbach |
| Author | M. Pocard |
| Author | F. Quenet |
| Author | J. Y. Blay |
| Author | S. Carrere |
| Author | C. Chevreau |
| Author | O. Mir |
| Author | A. Le Cesne |
| Abstract | BACKGROUND: Despite being associated with a very poor prognosis, long-term survivors across all series of Desmoplastic Small Round Cell Tumor (DSRCT) have been reported. AIM OF THE STUDY: To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis. METHODS: 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. RESULTS: 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 PCI<12 (HR?=?0.32, p?=?0.0004), MD Anderson stage I (HR?=?0.25, p? |
| Publication | Surgical Oncology |
| Volume | 29 |
| Pages | 107-112 |
| Date | Jun 2019 |
| Journal Abbr | Surg Oncol |
| Language | eng |
| DOI | 10.1016/j.suronc.2019.04.002 |
| ISSN | 1879-3320 |
| Short Title | Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? |
| Library Catalog | PubMed |
| Extra | 00000 PMID: 31196472 |
| Tags | Adolescent, Adult, Chemotherapy, Chemotherapy, Cancer, Regional Perfusion, Child, Child, Preschool, clinic, Combined Modality Therapy, Cure, Cytoreduction Surgical Procedures, Desmoplastic Small Round Cell Tumor, Female, Follow-Up Studies, Humans, Hyperthermia, Induced, Male, Middle Aged, Patient Selection, Peritoneal Neoplasms, Prognosis, Retrospective Studies, Surgery, Survival, Survival Rate, Young Adult |
| Date Added | 2020/01/03 - 10:24:11 |
| Date Modified | 2020/01/03 - 10:24:30 |
| Notes and Attachments | PubMed entry (Attachment) |
Institut de Recherche en Cancérologie de
