Retroperitoneal sarcomas: patterns of care in advanced stages, prognostic factors and focus on main histological subtypes: a multicenter analysis of the French Sarcoma Group.
BACKGROUND
Retroperitoneal sarcomas (RPS) are heterogeneous. Advanced stages include unresectable locoregional (LR) disease, abdominal sarcomatosis and distant metastasis. There is no available report assessing palliative chemotherapy in advanced RPS. This study analyzes management and outcome in a large cohort of patients with advanced RPS, considering main histological subtypes separately.


METHODS
We conducted a retrospective analysis of adult patients diagnosed with a RPS between 1 January 1988 and 31 December 2008 across 12 centers of the French Sarcoma Group. All cases were centrally reviewed by an expert pathologist.


RESULTS
Five-hundred eighty-six patients were included, 299 patients received palliative chemotherapy, with a median of two lines (range 0-8). Fifty patients underwent palliative surgery. Two hundred fifty-five patients (85%) were assessable for response after first line of chemotherapy. Among them, 69 patients (27%) had progressive disease, 145 (57%) had stable disease, 37 (14.5%) had partial response and 4 (1.5%) complete response. Median time from first line of palliative chemotherapy to progression was 5.9 months [4.9-7.3] and median overall survival (OS), 15.8 months [13-18]. In multivariate analysis, prognosis factors independently associated with poor OS were male gender, performance status (PS) >1 and grade >1. There was no difference according to stage of disease. Palliative surgery did not appear to add any survival benefit.


CONCLUSIONS
These results emphasize the scarcity of available options for RPS in the advanced setting and the urgent need to develop new strategies. Patients with good PS should be included in clinical trials and best supportive care should be considered in those with poor PS.
Department of Medical Oncology, Institut Bergonié, Bordeaux. Electronic address: maudtoulmonde@hotmail.com.
M
M
Toulmonde
Department of Surgical Oncology, Institut Gustave Roussy, Villejuif.
S
S
Bonvalot
Department of Medical Oncology, Centre Léon Bérard, Lyon.
I
I
Ray-Coquard
Department of Surgical Oncology, Institut Bergonié, Bordeaux.
E
E
Stoeckle
Department of Radiation Oncology, Institut Régional du Cancer Montpellier, Montpellier.
O
O
Riou
Department of Medical Oncology, Centre Georges François Leclerc, Dijon.
N
N
Isambert
Department of Medical Oncology, Centre René Gauducheau, Nantes.
E
E
Bompas
Department of Medical Oncology, Centre Oscar Lambret, Lille.
N
N
Penel
Department of Medical Oncology, Centre François Baclesse, Caen.
C
C
Delcambre-Lair
Department of Medical Oncology, Centre Antoine Lacassagne, Nice.
E
E
Saada
Departments of Medical Oncology.
A
A
Lecesne
Radiation Oncology, Institut Gustave Roussy, Villejuif.
C
C
Le Péchoux
Department of Medical Oncology, Centre Léon Bérard, Lyon.
J Y
JY
Blay
Department of Medical Oncology, Institut Curie, Paris.
S
S
Piperno-Neumann
Department of Medical Oncology, Centre Claudius Regaud, Toulouse.
C
C
Chevreau
Department of Medical Oncology, Centre Jean Perrin, Clermont-Ferrand.
J O
JO
Bay
Department of Clinical and Epidemiological Research, Institut Bergonié, Bordeaux.
V
V
Brouste
Department of Pathology, Institut Gustave Roussy, Villejuif.
P
P
Terrier
Department of Pathology, Centre Léon Bérard, Lyon.
D
D
Ranchère-Vince
Department of Pathology, Institut Bergonié, Bordeaux, France.
A
A
Neuville
Department of Medical Oncology, Institut Bergonié, Bordeaux.
A
A
Italiano
D000328:Adult; D018943:Anthracyclines / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003695:Delivery of Health Care; D018450:Disease Progression; D005260:Female; D005602:France; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D008080:Liposarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D008297:Male; D009362:Neoplasm Metastasis / Q000473:pathology / Q000628:therapy; D010166:Palliative Care; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000532:radiotherapy / Q000601:surgery; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy / Q000401:mortality* / Q000532:radiotherapy / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome
D018943:Anthracyclines
chemotherapy; palliative; prognosis; retroperitoneal sarcoma