Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients.
OBJECTIVE
To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.


METHODS
Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy.


RESULTS
Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p=0.0002).


CONCLUSIONS
A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins.
Bonvalot
S
S
Department of Surgery, Institut Gustave Roussy, 39 rue Camille Desmoulins, 94805 Villejuif, France. bonvalot@igr.fr
Eldweny
H
H
Haddad
V
V
Rimareix
F
F
Missenard
G
G
Oberlin
O
O
Vanel
D
D
Terrier
P
P
Blay
J Y
JY
Le Cesne
A
A
Le Péchoux
C
C
D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D018222:Desmoid Tumors / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D018714:Radiotherapy, Adjuvant; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D016019:Survival Analysis
D000970:Antineoplastic Agents