Stefanie Kube1, Christian Vokuhl2, Tobias Dantonello3, Monika Scheer1, Erika Hallmen1, Simone Feuchtgruber1, Gabriele Escherich4, Felix Niggli5, Ingrid Kuehnle6, Thekla von Kalle7, Stefan Bielack1, Thomas Klingebiel8, Ewa Koscielniak1. 1. Pediatrics 5 (Oncology, Hematology, and Immunology), Stuttgart Cancer Center, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany. 2. Kiel Pediatric Tumor Registry, Department of Pediatric Pathology, University of Kiel, Kiel, Germany. 3. Pediatrics 3 (Cardiology, Intensive Care Medicine, Pulmonology, and Allergology), Klinikum Stuttgart, Olgahospital, Stuttgart, Germany. 4. Clinic of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 5. Department of Pediatric Oncology, University Children's Hospital, Zürich, Switzerland. 6. Department of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany. 7. Department of Pediatric Radiology, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany. 8. Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt am Main, Germany.
Abstract
BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a rare subgroup of soft tissue tumors. The outcome of patients with IMT has been reported as favorable when the tumor is completely resected. If surgical resection is not possible, systemic therapy has to be considered. However, the best systemic treatment and response rates are currently unclear. METHODS: Thirty-eight patients under the age of 21, who were registered between 2000 and 2014 with a primary diagnosis of IMT, were analyzed. RESULTS: IMT was typically localized intra-abdominally or in the pelvis. In 20 patients, the tumor was resected without further therapy; 17 patients were in complete remission at last evaluation and two patients were in partial remission. Eighteen patients received systemic therapy, 15 of whom had macroscopically incomplete resection. Systemic therapy most commonly consisted of regimens with dactinomycin, ifosfamide or cyclophosphamide, and vincristine, with or without doxorubicin, and it seemed to reduce tumor extension in individual cases. Five-year event-free survival was 74 ± 14% and 5-year overall survival was 91 ± 10% for all patients. The patients who died due to the disease were those with incomplete resection (n = 3). CONCLUSIONS: Surgery without further systemic therapy was a feasible and acceptable therapeutic option for every second patient with IMT. Standard chemotherapy for pediatric soft tissue sarcoma produced favorable results in individual cases and was able to shrink the tumor enough to enable resection. Superior efficacy of new targeted therapies such as anaplastic lymphoma kinase-inhibitors compared to standard chemotherapy has to be proven in the future.
BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a rare subgroup of soft tissue tumors. The outcome of patients with IMT has been reported as favorable when the tumor is completely resected. If surgical resection is not possible, systemic therapy has to be considered. However, the best systemic treatment and response rates are currently unclear. METHODS: Thirty-eight patients under the age of 21, who were registered between 2000 and 2014 with a primary diagnosis of IMT, were analyzed. RESULTS: IMT was typically localized intra-abdominally or in the pelvis. In 20 patients, the tumor was resected without further therapy; 17 patients were in complete remission at last evaluation and two patients were in partial remission. Eighteen patients received systemic therapy, 15 of whom had macroscopically incomplete resection. Systemic therapy most commonly consisted of regimens with dactinomycin, ifosfamide or cyclophosphamide, and vincristine, with or without doxorubicin, and it seemed to reduce tumor extension in individual cases. Five-year event-free survival was 74 ± 14% and 5-year overall survival was 91 ± 10% for all patients. The patients who died due to the disease were those with incomplete resection (n = 3). CONCLUSIONS: Surgery without further systemic therapy was a feasible and acceptable therapeutic option for every second patient with IMT. Standard chemotherapy for pediatric soft tissue sarcoma produced favorable results in individual cases and was able to shrink the tumor enough to enable resection. Superior efficacy of new targeted therapies such as anaplastic lymphoma kinase-inhibitors compared to standard chemotherapy has to be proven in the future.
Authors: Giacomo Giulio Baldi; Mehdi Brahmi; Salvatore Lo Vullo; Elena Cojocaru; Olivier Mir; Michela Casanova; Bruno Vincenzi; Tommaso Martino De Pas; Giovanni Grignani; Maria Abbondanza Pantaleo; Jean Yves Blay; Robin Lewis Jones; Axel Le Cesne; Anna Maria Frezza; Alessandro Gronchi; Paola Collini; Angelo Paolo Dei Tos; Carlo Morosi; Luigi Mariani; Paolo Giovanni Casali; Silvia Stacchiotti Journal: Oncologist Date: 2020-07-12
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17
Authors: Andrea Ferrari; Bernadette Brennan; Michela Casanova; Nadege Corradini; Pablo Berlanga; Reineke A Schoot; Gema L Ramirez-Villar; Akmal Safwat; Gabriela Guillen Burrieza; Patrizia Dall'Igna; Rita Alaggio; Lisa Lyngsie Hjalgrim; Susanne Andrea Gatz; Daniel Orbach; Max M van Noesel Journal: Cancer Manag Res Date: 2022-09-23 Impact factor: 3.602