Vered Shkalim Zemer1, Helen Toledano1, Liora Kornreich2, Enrique Freud3, Eli Atar4, Smadar Avigad5, Galina Feinberg-Gorenshtein5, Suzana Fichman6, Josephine Issakov7, Tal Dujovny8, Isaac Yaniv1, Shifra Ash9. 1. 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. 2. 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. 3. 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. 4. Department of Diagnostic Radiology, Rabin Medical Center - Hasharon Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel. 5. 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. 6. Department of Pathology, Rabin Medical Center - Beilinson Hospital, Petach Tikva 4941492, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel. 7. 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. 8. Pediatric Oncology Unit, Emek Medical Center, Afula 1834111, Israel. 9. 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.
Abstract
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 1-17years. 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. CONCLUSION: Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach. LEVEL OF EVIDENCE: LEVEL IV, treatment study.
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 1-17years. 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. CONCLUSION: Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach. LEVEL OF EVIDENCE: LEVEL IV, treatment study.
Authors: Mohammed S Albokashy; Mohammed S Halawani; Anoof T Eshky; Khalid Alsaad; Hatim A Khoja; Samir M Bawazir Journal: J Surg Case Rep Date: 2021-05-27