Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients.
Adolescent
Adult
Chemotherapy, Cancer, Regional Perfusion
/ mortality
Child
Child, Preschool
Combined Modality Therapy
Cytoreduction Surgical Procedures
/ mortality
Desmoplastic Small Round Cell Tumor
/ mortality
Female
Follow-Up Studies
Humans
Hyperthermia, Induced
/ mortality
Male
Middle Aged
Patient Selection
Peritoneal Neoplasms
/ mortality
Prognosis
Retrospective Studies
Survival Rate
Young Adult
Chemotherapy
Cure
Desmoplastic small round cell tumor
Surgery
Survival
Journal
Surgical oncology
ISSN: 1879-3320
Titre abrégé: Surg Oncol
Pays: Netherlands
ID NLM: 9208188
Informations de publication
Date de publication:
Jun 2019
Jun 2019
Historique:
received:
07
08
2018
revised:
18
12
2018
accepted:
08
04
2019
entrez:
15
6
2019
pubmed:
15
6
2019
medline:
19
12
2019
Statut:
ppublish
Résumé
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 PCI<12 (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.
Sections du résumé
BACKGROUND
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
OBJECTIVE
To analyze patients 'characteristics associated with a prolonged survival after DSRCT diagnosis.
METHODS
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
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 < 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.
CONCLUSION
CONCLUSIONS
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.
Identifiants
pubmed: 31196472
pii: S0960-7404(18)30308-6
doi: 10.1016/j.suronc.2019.04.002
pii:
doi:
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
107-112Informations de copyright
Copyright © 2019. Published by Elsevier Ltd.