Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG)

Casanova, Michela; Brennan, Bernadette; Alaggio, Rita; Kelsey, Anna; Orbach, Daniel; Noesel, Max M. van; Corradini, Nadège; Minard‐Colin, Véronique; Zanetti, Ilaria; Bisogno, Gianni; Gallego, Soledad; Merks, Johannes H. M.; Salvo, Gian Luca De; Ferrari, Andrea

doi:10.1016/j.ejca.2019.12.021

Cited by 78 publications

(127 citation statements)

References 20 publications

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“…Responses were observed irrespectively to ALK status. Our results are consistent with what has previously been reported [25–29].…”

Section: Discussionsupporting

confidence: 94%

“…Two cases in this series were also included in our study. A total of 8 objective responses were seen in 10 patients aged 0–24 years with IMTs treated with vinblastine and methotrexate, whereas no responses were identified in 3 patients treated with ifosfamide‐based chemotherapy [29]. Interestingly, our series, which confirmed the impressive activity of the MTX‐V regimen on a similar number of patients, also indicates prolonged disease control with this schedule, in excess of 2 years, similarly to that observed in another mesenchymal tumor, desmoid fibromatosis [31].…”

Section: Discussionmentioning

confidence: 99%

“…Although most IMTs at diagnosis are localized and can be cured with surgery, systemic therapies still play a crucial role for patients with locally advanced disease (particularly in challenging primary sites) and in those developing metastatic lesions. Because of the extreme rarity of IMTs, data on chemotherapy in this sarcoma subtype are limited, retrospective, and mostly focused on childhood [25][26][27][28][29].…”

Section: Discussionmentioning

confidence: 99%

“…Both patients with localized disease treated with curative intent and those with advanced disease (locally advanced not eligible for surgical resection or definitive radiotherapy, or metastatic) were included. Two patients included in this case series had also been included in a retrospective study recently published by the European pediatric Soft Tissue Sarcoma Study Group (EpSSG) [29].…”

Section: Methodsmentioning

confidence: 99%

“…For patients with advanced disease, ALK inhibitors, such as crizotinib, can be active [23,24]. Currently there are limited data regarding the role of conventional chemotherapy in this disease, and they are limited to small retrospective experiences, mainly focused on the pediatric population [25][26][27][28][29].…”

Section: Introductionmentioning

confidence: 99%

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The Activity of Chemotherapy in Inflammatory Myofibroblastic Tumors: A Multicenter, European Retrospective Case Series Analysis

et al. 2020

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Background This study aimed to review the activity of cytotoxic chemotherapy in patients with inflammatory myofibroblastic tumors (IMTs) treated at nine European sarcoma reference centers. Materials and Methods Patients of any age, with histologically proven IMT, treated with anthracycline‐based methotrexate plus/minus vinorelbine/vinblastine (MTX‐V) or other chemotherapeutic regimens between 1996 and 2018 were retrospectively reviewed. Diagnosis was confirmed at the local level by an expert pathologist. Response was retrospectively assessed by local investigators by RECIST v1.1. Progression‐free survival (PFS), relapse‐free survival (RFS), and overall survival (OS) were computed by Kaplan‐Meier method. Results Thirty‐eight patients were included. Twenty‐five patients (8 localized, 17 advanced disease) received an anthracycline‐based regimen; 21 were evaluable for response. Overall response rate (ORR) was 10/21 (47.6%). At a 70.8‐month median follow‐up (FU), median RFS and median OS were not reached (NR) in patients with localized disease; median PFS and median OS were 6.3 (interquartile range [IQR]: 1.9–13.4) and 21.2 (IQR: 7.7–40.7) months in patients with advanced disease. Thirteen patients received MTX‐V (4 localized, 9 advanced disease), all evaluable for response. ORR was 7/13 (53.8%). At a 56.6‐month median FU, median RFS and median OS were 42.5 (IQR: 12.9–61.2) months and NR (no death events) in patients with localized disease, and NR (IQR: 24.9 to NR) and 83.4 months (IQR: 83.4 to NR) in patients with advanced disease. In the “other‐regimens group,” responses were seen in 3/4 patients treated with oral cyclophosphamide and 1/2 with docetaxel/gemcitabine. Conclusion Anthracycline‐based and MTX‐V regimens are very effective in IMT, with a similar ORR in both groups. MTX‐V achieved a prolonged disease control. Responses were also seen with oral cyclophosphamide and docetaxel/gemcitabine, but few patients were treated with these schedules. Implications for Practice Inflammatory myofibroblastic tumor (IMT) is an ultrarare sarcoma with known sensitivity to anaplastic lymphoma kinase (ALK) inhibitors in ALK‐fused cases, although ALK inhibitors are not licensed in the disease. The current knowledge on the activity of cytotoxic chemotherapy is limited. This multi‐institutional retrospective study on pediatric and adult patients with IMT shows that cytotoxic chemotherapy, and in particular anthracycline‐based and methotrexate plus/minus vinorelbine/vinblastine regimens, represents a treatment option and can be considered in IMT patients irrespectively from ALK status. This study provides a benchmark for future studies on new medical therapies.

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“…Responses were observed irrespectively to ALK status. Our results are consistent with what has previously been reported [25–29].…”

Section: Discussionsupporting

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The Activity of Chemotherapy in Inflammatory Myofibroblastic Tumors: A Multicenter, European Retrospective Case Series Analysis

et al. 2020

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Development and validation of a nomogram for predicting survival in patients with malignant myofibroblastic tumor

Wang

Liu

2023

Cancer Medicine

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Background Malignant myofibroblastic tumors are a rare group of soft tissue sarcomas, for which a prognosis prediction model is lacking. Based on the Surveillance, Epidemiology, and End Results (SEER) database and cases from Nanjing Drum Tower Hospital, the current study constructed and validated a nomogram to assess overall survival of patients with malignant myofibroblastic tumors. Methods Data of patients with myofibroblastic tumors diagnosed between 2000 and 2018 were extracted from the SEER database. Similarly, data of patients with myofibroblastic tumor in Nanjing Drum Tower Hospital between May 2016 and March 2022 were collected. Then, we conducted univariate and multivariate Cox analyses to identify independent prognostic parameters to develop the nomogram. The model was evaluated by concordance index (C‐index), calibration curve, the area under the curve (AUC), decision curve analysis (DCA), Kaplan–Meier analysis, and subgroup analyses. Results Seven variables were selected to construct the nomogram. The results of the C‐index (0.783), calibration curve, the AUCs, and subgroup analyses demonstrated the accurate predictive capacity and excellent discriminative ability of the nomogram. The DCA of the model indicated its better clinical net benefit than that of the traditional system. Conclusion Evaluation of the predictive performance of the nomogram revealed the superior sensitivity and specificity of the model and the higher prediction accuracy of the outcomes compared with those of the traditional system. The established nomogram may assist patients in consultation and help physicians in clinical decision‐making.

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The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

et al. 2023

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Background Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data. Methods The INSTRuCT non‐rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors. Results and Conclusion This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research.

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Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG)

Cited by 78 publications

References 20 publications

The Activity of Chemotherapy in Inflammatory Myofibroblastic Tumors: A Multicenter, European Retrospective Case Series Analysis

The Activity of Chemotherapy in Inflammatory Myofibroblastic Tumors: A Multicenter, European Retrospective Case Series Analysis

Development and validation of a nomogram for predicting survival in patients with malignant myofibroblastic tumor

The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

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