Rapports de cas cliniques et médicaux

Retroperitoneal Desmoid-type Fibromatosis of the Distal Ureter: A Discussion of the Imaging Differential Diagnoses

Abstract

Desmond Lim Shi Wei, Edmund Chiong, Derrick Lian Wen Quan and Lim Mei Chin

A 42-year-old man in remission for chronic myeloid leukaemia (CML) was found to have worsening renal function. Computed Tomography (CT) of the urinary system showed a soft tissue lesion of the right distal ureter which had an atypical appearance for primary malignancy. Due to concerns of recurrence, further evaluation with contrast-enhanced Magnetic Resonance Imaging (MRI) was performed revealing the fibrous nature of the lesion. Considering the resultant obstructive uropathy and the need to exclude recurrence of prior haematological malignancy, resection and histological examination was performed. Resection of the affected segment of the right distal ureter was performed with reimplantion (Psoas Hitch) and Heineke-Mikulicz Bladder reconstruction. Histological examination revealed desmoid-type fibromatosis involving the ureter which is extremely rare. We present the diagnostic process and describe the imaging characteristics through multi-planar CT and MRI images of the tumour obtained prior to resection. A discussion of the imaging differential diagnoses is included with companion images. This entity is important to recognise due to the local aggressiveness of this tumour with propensity for recurrence.