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Thứ Bảy, 17 tháng 11, 2012


An 18-year-old man with an established history of neurofibromatosis type 1 (NF1) presented with weight loss and umbilical protrusion. He denied any pain, urinary symptoms, or gastrointestinal symptoms. An initial ultrasound (US) examination of the abdomen and pelvis demonstrated bilateral hydronephrosis and bladder wall thickening, which led to urologic consultation and, ultimately, cystoscopy. At cystoscopy, the bladder mucosa was noted to be irregular and compressed, but no focal intraluminal mass was appreciated. There was some apparent difficulty in identifying the ureteral orifices.
Further imaging with computed tomography (CT) and magnetic resonance (MR) imaging showed a large mass involving the bladder wall and causing the hydronephrosis. Bilateral nephrostomy tubes and ureteric stents were placed in an antegrade fashion, which succeeded in relieving the urinary obstruction.

Subsequently, the nephrostomy tubes were removed and the ureteric stents were left in place. The patient underwent a surgical biopsy, which demonstrated pathologic changes diagnostic of neurofibroma. The possibility of malignant degeneration was considered given the size of the mass, resulting in the referral of the patient to a tertiary-care center for radical cystectomy and urinary diversion.

Myofibroblastic tumor, also known as inflammatory pseudotumor or pseudosarcoma, is a benign tumor with mesenchymal origin. Bladder location is very uncommon. We report the case of a 58-year-old man with a history of von Recklinghausen’s disease who complained for painless macroscopic hematuria 5 months after suprapubic prostatectomy. The radiograph evaluation revealed a bladder tumor, and the pathologic examination following a transurethral resection showed inflammatory myofibroblastic tumor of the bladder. The patient finally underwent a radical cystectomy due to the uncertain pathogenesis of inflammatory myofibroblastic tumor as well as the rarity of cases published on bladder tumors in Von Recklinghausen’s patients.

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