Inflammatory myofibroblastic tumour of the lung manifesting as progressive atelectasis

Abstract

A 6-year-old girl with an inflammatory myofibroblastic tumour of the lung is presented. The radiological features of this lesion are often atypical, as in this case, presenting as progressive atelectasis. The differential diagnosis is extensive and can be difficult, despite modern imaging techniques.

Imaging observations of pulmonary inflammatory myofibroblastic tumors in patients over 40 years old

“…Inflammatory myofibroblastic tumor (IMT) is a rare disorder that was previously referred to by a variety of synonyms, including inflammatory pseudotumor, plasma cell granuloma, fibroxanthoma, fibrous histiocytoma and xanthogranuloma (1). In 2002, the World Health Organization classification scheme defined IMT as a ‘distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes and eosinophils’ (2).…”

Pulmonary and Extrathymic Mediastinal Tumors

“…Locally aggressive IMTs are occasionally seen and may invade the mediastinum, chest wall, or diaphragm (Hedlund et al 1999;Verbeke et al 1999;Yousem et al 2004). CT and MRI are both useful in characterizing the extent of these aggressive lesions in relation to the airways, esophagus, cardiovascular structures, and chest wall (Hedlund et al 1999).…”

“…When these tumors extend beyond the organ of origin at diagnosis, up to 46 % relapse locally (Janik et al 2003). Relapse or invasion of the mediastinum has occasionally been treated with immunosuppressive therapy or even multiagent chemotherapy with good results (Verbeke et al 1999;Janik et al 2003). Metastases have been documented to the lung, liver, bone, and brain (Coffin et al 2007;Siminovich et al 2012).…”

“…Hilar lymphadenopathy and pleural effusion are not commonly associated (Mas Estelles et al 1995). Although bronchi and vessels may be trapped within the pulmonary masses and become narrowed distally or even obliterated, atelectasis is observed in only 14 % of pediatric patients (Verbeke et al 1999).…”

Inflammatory Myofibroblastic Tumor of Mesentery: A Case Report

“…They belong to a rare class of pseudosarcomatous inflammatory lesions that are usually benign and nonmetastasizing. This tumor has predilection for any age [1]. It can arise virtually from any anatomical site from the central nervous system to the gastrointestinal tract.…”