TY - JOUR
T1 - Sporadic desmoid tumors in the pediatric population
T2 - A single center experience and review of the literature
AU - Shkalim Zemer, Vered
AU - Toledano, Helen
AU - Kornreich, Liora
AU - Freud, Enrique
AU - Atar, Eli
AU - Avigad, Smadar
AU - Feinberg-Gorenshtein, Galina
AU - Fichman, Suzana
AU - Issakov, Josephine
AU - Dujovny, Tal
AU - Yaniv, Isaac
AU - Ash, Shifra
N1 - Publisher Copyright:
© 2017 Elsevier Inc.
PY - 2017
Y1 - 2017
N2 - Background/Purpose We present our long experience with desmoid tumors in children. Methods Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988–2016. There were 10 girls and 7 boys aged 1–17 years. Tumor sites included head and neck, trunk, extremity, and groin. Eight patients underwent radical resection, with complete remission in 7 and local relapse in one which was treated with chemotherapy. Four patients underwent incomplete surgical resection, three with adjuvant chemotherapy. Five patients underwent biopsy only and chemotherapy. Two of the 9 chemotherapy-treated patients also had intraarterial chemoembolization. Chemotherapy usually consisted of vincristine and actinomycin-D with or without cyclophosphamide or low-dose vinblastine and methotrexate. Two patients also received tamoxifen. Results After a median follow-up of 3.3 years, 10 patients were alive in complete remission, 5 had stable disease, and 2 had reduced tumor size. Five-year overall survival was 100%, and event-free survival, 87.5%. Ten were screened for CTNNB1 mutations. CTNNB1 gene sequencing yielded mutations in 5/10 samples tested: 3 T41A, 2 S45F. There was no association of CTNNB1 mutation with clinical outcome or prognosis. Conclusion Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach. Level of evidence LEVEL IV, treatment study.
AB - Background/Purpose We present our long experience with desmoid tumors in children. Methods Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988–2016. There were 10 girls and 7 boys aged 1–17 years. Tumor sites included head and neck, trunk, extremity, and groin. Eight patients underwent radical resection, with complete remission in 7 and local relapse in one which was treated with chemotherapy. Four patients underwent incomplete surgical resection, three with adjuvant chemotherapy. Five patients underwent biopsy only and chemotherapy. Two of the 9 chemotherapy-treated patients also had intraarterial chemoembolization. Chemotherapy usually consisted of vincristine and actinomycin-D with or without cyclophosphamide or low-dose vinblastine and methotrexate. Two patients also received tamoxifen. Results After a median follow-up of 3.3 years, 10 patients were alive in complete remission, 5 had stable disease, and 2 had reduced tumor size. Five-year overall survival was 100%, and event-free survival, 87.5%. Ten were screened for CTNNB1 mutations. CTNNB1 gene sequencing yielded mutations in 5/10 samples tested: 3 T41A, 2 S45F. There was no association of CTNNB1 mutation with clinical outcome or prognosis. Conclusion Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach. Level of evidence LEVEL IV, treatment study.
KW - CTNNB1 gene mutations
KW - Pediatric population
KW - Sporadic desmoid tumors
UR - http://www.scopus.com/inward/record.url?scp=85012277795&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2017.01.068
DO - 10.1016/j.jpedsurg.2017.01.068
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C2 - 28209418
AN - SCOPUS:85012277795
SN - 0022-3468
VL - 52
SP - 1637
EP - 1641
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 10
ER -