Sporadic desmoid tumors in the pediatric population: A single center experience and review of the literature

Vered Shkalim Zemer, Helen Toledano, Liora Kornreich, Enrique Freud, Eli Atar, Smadar Avigad, Galina Feinberg-Gorenshtein, Suzana Fichman, Josephine Issakov, Tal Dujovny, Isaac Yaniv, Shifra Ash*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1637-1641
Number of pages5
JournalJournal of Pediatric Surgery
Issue number10
StatePublished - 2017


  • CTNNB1 gene mutations
  • Pediatric population
  • Sporadic desmoid tumors


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