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Updated: Dec 29, 2025
Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG)
Michela Casanova1, Bernadette Brennan2, Rita Alaggio3
1Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
Insights
Inflammatory myofibroblastic tumor (IMT) shows a good prognosis in pediatric patients, regardless of ALK status or resectability. Chemotherapy remains effective for advanced IMT, but further research is needed on ALK inhibitors.
Area of Science:
- Pediatric Oncology
- Oncology
- Pathology
Background:
- Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic proliferation.
- Accurate diagnosis and treatment protocols are crucial for improving patient outcomes.
Purpose of the Study:
- To report clinical findings and treatment outcomes for pediatric patients with IMT.
- To evaluate the prognostic significance of ALK status and resectability in IMT.
- To assess the efficacy of systemic therapies for advanced IMT.
Main Methods:
- Prospective registration of patients (<25 years) with IMT across 9 countries (2005-2016).
- Centralized pathology review and mandatory ALK immunohistochemistry.
- Analysis of clinical data, treatment responses, and survival outcomes.
Main Results:
- 60 eligible IMT cases (median age 9.5 years); 40 ALK-positive, 20 ALK-negative.
- Five-year event-free survival (EFS) of 82.9% and overall survival (OS) of 98.1%.
- Overall response to systemic therapy was 64%, with notable responses to vinblastine-methotrexate and ALK inhibitors.
Conclusions:
- IMT demonstrates a favorable prognosis in pediatric patients, including those with unresectable or ALK-negative disease.
- Chemotherapy remains a viable treatment for advanced IMT.
- Larger studies are required to define the role of ALK inhibitors in IMT treatment.
Introduction:
We report the clinical findings and results of treatment in the cohort of patients with inflammatory myofibroblastic tumor (IMT) managed according to the European pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol from 2005 to 2016.
Methods:
Patients (<25 years old) with IMT from 9 countries were prospectively registered via a web-based system. Their histology was reviewed by a national/international pathology panel. Immunohistochemistry for ALK assessment was mandatory. No adjuvant therapy was suggested for initially resected tumors. No specific systemic therapy was recommended for cases of unresectable disease.
Results:
Among 80 cases of IMT registered, 20 were excluded because pathology review led to a revised diagnosis. Of the remaining 60 patients (median age 9.5 years), 59 had localized, and 1 had multifocal/metastatic disease. The lung was the primary site in 14 cases. IMT developed as a second tumor in 2 cases. Forty cases were ALK-positive, and 20 were ALK-negative. Five-year event-free survival (EFS) and overall survival (OS) were 82.9% and 98.1%, respectively. No clinical variables correlated statistically with the outcome: survival was the same for ALK-positive and ALK-negative cases. The overall response to systemic therapy was 64%: 8/10 cases responded to vinblastine-methotrexate chemotherapy, and 5/5 to ALK-inhibitors.
Conclusions:
This study demonstrated a good overall prognosis for IMT, even for initially unresectable disease and in ALK-negative cases. Chemotherapy is still a valid option for advanced disease. Larger studies involving both pediatric and adult patients are needed to clarify the role of ALK inhibitors.
