A 55-year-old woman who was diagnosed with Werner syndrome presented with macroscopic hematuria. Her medical history included hypertension and diabetes mellitus, which are common comorbidities in Werner syndrome. She had also been treated for chronic leg ulcers for 5 years. Although no tumor was observed in the bladder, enhanced computerized tomography (CT) showed a tumor in the middle part of the left ureter that appeared to be the cause of hydronephrosis. Based on these findings, the patient was diagnosed with left ureteral cancer, and laparoscopic nephroureterectomy was performed. The histological evaluation showed invasive urothelial carcinoma (pT2N0M0). Cystoscopy at 4-month followup showed tumor recurrence on the trigone of the bladder, and transurethral resection of the bladder tumor (TURBT) was performed. Although the histological evaluation showed invasive urothelial carcinoma (pT1), the surgical specimens obtained by repeat TURBT identified no residual malignancy histopathologically. Intravesical bacillus Calmette-Guérin therapy was refused. Three months after repeat TURBT, multiple papillary tumors recurred on the posterior wall and bladder neck. One week before the third TURBT for recurrent tumors, the patient started to complain of urinary incontinence. During the operation, a vesicovaginal fistula (VVF) was confirmed (fig. 1). Although histological evaluation showed the presence of carcinoma in situ, intravesical bacillus Calmette-Guérin therapy could not be performed because of the VVF. CT imaging for pre-surgical evaluation for radical cystectomy demonstrated not only the VVF (fig. 2), but also 2 masses in the anterior aspect of the right kidney (fig. 3). During the operation, tissue samples were first collected from the peri-renal masses for rapid intraoperative diagnosis. Although no malignant lesion was reported on frozen section examination, the masses were removed without nephrectomy, and then radical cystectomy was also performed. The final diagnosis of the peri-renal masses was high-grade sarcoma, which is consistent with dedifferentiated liposarcoma. This patient refused additional surgical intervention with right nephrectomy because of the difficulty of hemodialysis. She underwent chemotherapy for 17 months and died 24 months after the diagnosis of liposarcoma.

Figure 1. Cystoscopic view of VVF. Finger inserted in vagina can be seen (arrow).

Figure 2. Sagittal CT imaging of VVF. Arrow indicates VVF track from trigone of bladder into vagina.

Figure 3. Abdominal CT imaging before cystectomy showing heterogeneous masses (arrows) in anterior aspect of right kidney

Werner syndrome is a rare autosomal recessive disorder characterized by premature aging.¹ Diabetes mellitus, arteriosclerosis, and malignant tumors are common in this syndrome, resulting in a shorter lifespan. Malignancies associated with Werner syndrome are more frequently nonepithelial than epithelial.² In addition, the frequency of urothelial carcinomas is low even among epithelial.² In the present case, urothelial carcinoma was diagnosed when the patient presented with gross hematuria, but she eventually died of liposarcoma that occurred during the treatment process.

Intractable skin ulcers are also a major problem in Werner syndrome, which may have a significant impact on the quality of life of the patient. Although the etiology of the skin ulcers is multifactorial, they are due to a combination of decreased adipose tissue, delayed wound healing due to fibroblast aging, and microcirculatory disorders associated with diabetes mellitus and/or arteriosclerosis.^3,4 In this patient, cystoscopic observation after 2 TURBTs showed no obvious ulcer formation in the bladder. However, a VVF formed despite no obvious cause other than surgery, and it was considered that the delayed wound healing associated with Werner syndrome was involved in the formation of the VVF.

Extreme care should be taken when performing TURBT in patients with diseases involving delayed wound healing, including Werner syndrome.