title: Sporadic desmoid tumors in the pediatric population: A single center experience and review of the literature.
abstract: OBJECTIVE
  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 1-17years. 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 3.3years, 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.
  
  
  CONCLUSIONS
  Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach.
  
  
  METHODS
  LEVEL IV, treatment study.
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Vered
authors.initials: V
authors.lastname: Shkalim Zemer
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Helen
authors.initials: H
authors.lastname: Toledano
authors.affiliation: Department of Imaging, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Liora
authors.initials: L
authors.lastname: Kornreich
authors.affiliation: Department of Pediatric Surgery, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Enrique
authors.initials: E
authors.lastname: Freud
authors.affiliation: Department of Diagnostic Radiology, Rabin Medical Center - Hasharon Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Atar
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Molecular Oncology, Felsenstein Medical Research Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Smadar
authors.initials: S
authors.lastname: Avigad
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Molecular Oncology, Felsenstein Medical Research Center, Rabin Medical Center - Beilinson Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Galina
authors.initials: G
authors.lastname: Feinberg-Gorenshtein
authors.affiliation: Department of Pathology, Rabin Medical Center - Beilinson Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Suzana
authors.initials: S
authors.lastname: Fichman
authors.affiliation: Unit of Bone and Soft Tissue Tumors, Institute of Pathology, Sourasky Medical Center, Tel Aviv 64239, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Josephine
authors.initials: J
authors.lastname: Issakov
authors.affiliation: Pediatric Oncology Unit, Emek Medical Center, Afula 1834111, Israel.
authors.forename: Tal
authors.initials: T
authors.lastname: Dujovny
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
authors.forename: Isaac
authors.initials: I
authors.lastname: Yaniv
authors.affiliation: Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel. Electronic address: shifraa@clalit.org.il.
authors.forename: Shifra
authors.initials: S
authors.lastname: Ash
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D018572:Disease-Free Survival; D005260:Female; D018222:Desmoid Tumors / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D017211:Treatment Failure
keywords: CTNNB1 gene mutations; Pediatric population; Sporadic desmoid tumors