TY - JOUR
T1 - Inflammatory myofibroblastic tumor
T2 - The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG)
AU - Casanova, Michela
AU - Brennan, Bernadette
AU - Alaggio, Rita
AU - Kelsey, Anna
AU - Orbach, Daniel
AU - van Noesel, Max M.
AU - Corradini, Nadege
AU - Minard-Colin, Veronique
AU - Zanetti, Ilaria
AU - Bisogno, Gianni
AU - Gallego, Soledad
AU - Merks, Johannes H.M.
AU - De Salvo, Gian Luca
AU - Ferrari, Andrea
N1 - Funding Information:
The authors thank the patients, caregivers, and medical staff involved in this study from the recruiting countries (Italy, France, UK and Ireland, Belgium, Czech Republic, Spain, Netherlands, Argentina, and Israel). The authors thank Fondazione Citta'della Speranza, Italy.
Publisher Copyright:
© 2020 Elsevier Ltd
PY - 2020/3
Y1 - 2020/3
N2 - 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.
AB - 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.
KW - ALK inhibitors
KW - European pediatric Soft Tissue Sarcoma Study Group
KW - Inflammatory myofibroblastic tumor
KW - Outcome
KW - Prognostic factors
KW - Response to chemotherapy
UR - http://www.scopus.com/inward/record.url?scp=85078401269&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2019.12.021
DO - 10.1016/j.ejca.2019.12.021
M3 - Article
C2 - 32007712
AN - SCOPUS:85078401269
SN - 0959-8049
VL - 127
SP - 123
EP - 129
JO - European Journal of Cancer
JF - European Journal of Cancer
ER -