Clinico-radiological and pathological characteristics of inflammatory myofibroblastic tumors in children: A retrospective study

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.

Giant inflammatory myofibroblastic tumor of the hypopharynx: an unusual cause of acute upper airway obstruction in a 6-year-old child

“…Anti-inflammatory treatment, chemotherapy, steroids, or immunomodulators as potential remedial and effective management strategies are recommended for adult patients who may not be able to undergo possible complete excision or in ALK-negative cases with a higher frequency of metastasis and invasiveness. 12 In conclusion, in this child, the initial symptom of a large IMT was acute UAO. This report highlights the importance of differential diagnosis in a child with acute UAO.…”

“…Although there is no consensus on the treatment of IMTs, complete excision is critical for cure and can result in a favorable prognosis in children. Anti-inflammatory treatment, chemotherapy, steroids, or immunomodulators as potential remedial and effective management strategies are recommended for adult patients who may not be able to undergo possible complete excision or in ALK-negative cases with a higher frequency of metastasis and invasiveness 12…”