Inflammatory myofibroblastic tumors-A retrospective analysis of the Cooperative Weichteilsarkom Studiengruppe

Pediatric Blood & Cancer
Stefanie KubeEwa Koscielniak

Abstract

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. Thirty-eight patients under the age of 21, who were registered between 2000 and 2014 with a primary diagnosis of IMT, were analyzed. 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). Surgery without further systemic therapy...Continue Reading

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Citations

May 3, 2019·Der Pathologe·C Vokuhl
Nov 7, 2019·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Oscar Lopez-NunezLea F Surrey
Sep 13, 2020·The Oncologist·Silvia StacchiottiPaolo Giovanni Casali
Jul 23, 2020·Case Reports in Oncological Medicine·Sumaira KhalilAmna Kaneez Fatima Raja
Jun 21, 2020·Pediatric Surgery International·Natalia PetrasovaMichal Rygl
Feb 3, 2020·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Michela CasanovaAndrea Ferrari
Mar 13, 2021·Surgical Oncology Clinics of North America·Joerg FuchsDavid A Rodeberg
Jul 11, 2021·Current Problems in Cancer·Priya MahajanRajkumar Venkatramani
Aug 3, 2021·Experimental and Therapeutic Medicine·Yangkun WangSunan Wang

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