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Tumori ; 102(6): 582-587, 2016 Dec 01.
Article in English | MEDLINE | ID: mdl-27647224

ABSTRACT

PURPOSE: Before the wait-and-see policy became the standard approach in abdominal wall desmoid tumors, surgery was performed on a systematic basis. Surgery remains indicated for progressing tumors but its extent is debatable. The abdominal wall is a common site of origin of sporadic desmoids, usually associated with a favorable prognosis. We analyzed the results of surgery at this specific site. METHODS: Data from 33 patients affected by sporadic desmoid tumors of the abdominal wall (31 primary, 2 recurrent) consecutively treated at our cancer center between January 2000 and September 2013 were retrospectively studied. RESULTS: Twenty-nine patients underwent surgery upfront and 1 after progression during the initial wait-and-see period. Prosthetic reconstruction of the abdominal wall was required in 28 patients. The average hospital stay was 5 days. Three patients developed surgical complications. Local recurrence-free survival was 90% at 5 and 10 years. Three patients had an uneventful childbirth during the follow-up after surgery. CONCLUSIONS: Desmoid tumors of the abdominal wall have a favorable prognosis after surgical resection, which remains a safe and effective treatment. Wild-type tumors are common, whereas the incidence of S45F mutation in the beta-catenin gene is lower than in other anatomic sites. Upfront surgery may be considered in selected women who wish to bear a child.


Subject(s)
Fibromatosis, Abdominal/pathology , Fibromatosis, Abdominal/surgery , Adult , Disease Progression , Female , Fibromatosis, Abdominal/genetics , Fibromatosis, Abdominal/mortality , Humans , Male , Middle Aged , Mutation , Neoplasm Recurrence, Local , Postoperative Complications , Prognosis , Proportional Hazards Models , Treatment Outcome , Tumor Burden
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