Erdheim-Chester disease: an unusual aetiology of bilateral lipomatous perinephric masses.
A 53-year-old man presented with a history of progressive abdominal distention for 1 year. Physical examination revealed large palpable masses in the bilateral flank regions. Contrast-enhanced CT of the abdomen showed bilateral, symmetrical large perinephric masses with fat attenuating areas, which was further confirmed on MRI. CT of the paranasal sinuses revealed circumscribed extraconal soft tissue mass in the left orbit, causing scalloping and erosion of the left orbital roof. Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography/CT showed FDG uptake in the bilateral perinephric masses. Based on imaging appearance, a diagnosis of Erdheim-Chester disease (ECD) was suggested. Ultrasound-guided biopsy from perinephric masses revealed a sheet of histiocytes with sprinkled lymphocytes and plasma cells in the background. The histiocytes were immunopositive for CD68, S100 and immunonegative for CD1a, which confirmed the diagnosis of ECD. The patient was started on interferon-α-2a and showed symptomatic improvement.
['Singh|Rashmi|R|', 'Naranje|Priyanka|P|', 'Ramateke|Prashant|P|', 'Damle|Nishikant Avinash|NA|']
D031249:Erdheim-Chester Disease D006644:Histiocytes D006801:Humans D008279:Magnetic Resonance Imaging D008297:Male D008875:Middle Aged D049268:Positron-Emission Tomography D014057:Tomography, X-Ray Computed
haematology (incl blood transfusion) pathology radiology