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

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

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74 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)123-129
Number of pages7
JournalEuropean Journal of Cancer
Publication statusPublished - Jan 2020


  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols/therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Inflammation/drug therapy
  • Male
  • Myofibroblasts/drug effects
  • Neoplasms, Muscle Tissue/drug therapy
  • Prognosis
  • Prospective Studies
  • Survival Rate


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