Clinico-radiological and Pathological Characteristics of Inflammatory Myofibroblastic Tumors in Children: A Retrospective Study

Indian J Med Paediatr Oncol. 2017 Jul-Sep;38(3):261-265. doi: 10.4103/ijmpo.ijmpo_178_16.

Abstract

Purpose: Inflammatory myofibroblastic tumors (IMTs) are rare, benign lesions most often seen in the lung of young adults but can occur in children, in various sites. They mimic, clinically and radiologically, malignant tumors - especially sarcomas and lymphomas. The aim was to review the clinical, radiological, and pathological data of children with a diagnosis of IMT referred to our department.

Materials and methods: This retrospective study was conducted at the Department of Medical and Paediatric Oncology, Regional Cancer Centre, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India from January 2014 to December 2015.

Results: Among 288 pediatric tumors registered during the study, 5 (1.73%) had the diagnosis of IMTs. The main symptoms were abdominal distension and pain in 60% (three cases), breathlessness and cough in 20% (one case), and right axillary area swelling in 20% (one case). In three patients, complete surgical excision was done, whereas another patient with retroperitoneal mass had the residual disease and received chemotherapy followed by complete second surgery. In the case of mediastinal IMT, surgery was followed by local radiotherapy. At present, four patients are disease-free and one patient with mediastinal IMT has the residual progressive disease.

Conclusion: On presentation, IMT can constitute a formidable challenge, from diagnosis through to treatment.

Keywords: Inflammatory myofibroblastic tumors; macrolides; pseudotumors; steroids; tyrosine kinase.