TY - JOUR
T1 - Sporadic desmoid tumors of the abdominal wall
T2 - The results of surgery
AU - Mussi, Chiara Erminia
AU - Colombo, Piergiuseppe
AU - Chiara, Lo Russo
AU - Kasangian, Anaid
AU - Cananzi, Ferdinando Carlo Maria
AU - Marrari, Andrea
AU - Morenghi, Emanuela
AU - De Sanctis, Rita
AU - Quagliuolo, Vittorio
PY - 2016/11/1
Y1 - 2016/11/1
N2 - 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.
AB - 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.
KW - Aggressive fibromatosis
KW - Beta-catenin mutation
KW - Desmoid tumor
KW - Pregnancy
KW - S45F mutation
KW - Tumor of abdominal wall
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U2 - 10.5301/tj.5000552
DO - 10.5301/tj.5000552
M3 - Article
AN - SCOPUS:85012188667
VL - 102
SP - 582
EP - 587
JO - Tumori
JF - Tumori
SN - 0300-8916
IS - 6
ER -