Presentation
Fever, productive cough, shortness of breath, and fever for two weeks, as well as weight loss.
Patient Data
An enhancing intraluminal lesion was found in the left main bronchus which causes total obstruction of the left lung. The left lung is collapsed with compensatory hyperinflation of the right lung.
Case Discussion
The patient underwent left total pneumonectomy and lymph node clearance.
Histopathology report
Sections show fragments of tumor tissue composed of spindle cell proliferation in intersecting fascicles and storiform patterns associated with prominent inflammatory infiltrates. The spindle cells exhibit ovoid to round, vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Mitotic figures are seen. The stroma is loose to hyalinized and densely infiltrated by lymphocytes, plasma cells and neutrophils with abundant foamy macrophages (highlighted by CD68). No respiratory epithelium was seen. No lymphovascular permeation was detected.
Immunohistochemistry
The neoplastic cells are diffusely positive for ALK1 with focal positivity to SMA (tram track). They are negative for PanCK, S100 and desmin.
Final interpretation: Inflammatory myofibroblastic tumor (IMT)
An inflammatory myofibroblastic tumor (IMT) is an ultra-rare tumor comprising myofibroblastic and fibroblastic spindle cells with inflammatory cell components. It is most commonly found in children and young adults. The mainstay of treatment for IMT is total surgical resection with an excellent prognosis and a low recurrence rate. The five-year survival rate can be up to 91 %, with a 4% recurrence rate after resection.
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