Bladder leiomyosarcoma with total laparoscopic intracoropreal orthotopic managment- case report.

Bladder leiomyosarcoma is a rare malignancy, it's nonurothelial tumors and account less than 5% of all bladder malignancies. Because of the very low incidence less than 200 cases reported in the literature, etiology is still little know, also there is lack of consensus regarding definitive treatment for this rare tumors. The most common clinical presentation is gross hematuria, which is reported in 81% of cases, followed by pollakiuria (28%) and dysuria (19%), respectively. Herein, we present the case of a patient diagnosed with a bladder leiomyosarcoma and treated with laparoscopic radical cystoprostatectomy and totally intracorporal orthotopic ileal diversion.

Introduction

Nonurothelial tumors of the bladder are rare and account for less than 5% of all bladder malignancies. More than 100 cases of leiomyosarcoma have been reported, which represent 0.1% of all nonepithelial tumors. The most common clinical presentation is gross hematuria, which is reported in 81% of cases, followed by pollakiuria (28%) and dysuria (19%), respectively. These tumors occur in older adults of either sex and are characterized by an aggressive behavior. Because of the very low incidence etiology is still little know, that's why there is lack of consensus regarding definitive treatment for this relatively rare tumors. Bladder sarcomas were once thought to have a bad prognosis, recent studies suggest that adequate radical surgical treatment with negative margins is able to achieve optimal cancer control outcomes. Herein, we present the case of a patient diagnosed with a high grade bladder leiomyosarcoma and treated with laparoscopic radical cystoprostatectomy and totally intracorporal orthotopic ileal neobladder. The follow-up of 12 months there is no evidence of local or distant recurrences.

Case report

A 63-year-old man presented with dysuria, macroscopic hematuria with clots and lower urinary tract symptoms for a period of 6 months. On the ultrasound was found a single bladder mass approximately 4.5 cm × 4.7 cm × 4 cm in size with left hydroureter and left hydronephrosis. A contrast-enhanced computed tomography scan showed a well-defined, heterogeneous, enhancing endophytic soft tissue lesion on the left bladder wall with obstruction of the left ureter and hydronephrosis on the left kidney no evidence of metastatic disease(Fig. 1). The patient underwent transurethral resection of the tumor, pathology result was leiomyosarcoma high grade. Patient was refer to our institution were we performed radical treatment. We performed laparoscopic radical cystoprostatectomy with extended pelvic lymph node dissection, and totally intracorporal urinary diversion. Histopathology result confirmed the diagnosis an invasive high grade leiomyosarcoma[Fig. 2, Fig. 3], with negative surgical margins and no lymph node metastasis (0/1). On postoperative day 8, the patient was discharged from the hospital. Follow-up CT scan 6 months after surgery showed no sign of recurrence.

Fig. 1

Well-defined, heterogeneous, enhancing endophytic soft tissue lesion on the left bladder wall, approximately 4.5 cm × 4.7 cm × 4 cm in size.

Fig. 2

Infiltrative tumor with well defined border of comprimed adjacent tissue, without formation of genuine fibrous capsule.

Fig. 3

The tumor is composed of cellular bundles of atypical spindle cells with evident smooth muscle differentiation, moderate nuclear atypia, low mitotic count (1 MF/10 HPF), perinuclear cytoplasmic vacuoles.

Discussion

Leiomyosarcomas are rare, and they represent less than 1% of malignant bladder tumors. The specific macroscopic characteristics of the disease for the first time was described by Bergman et al.,. In 1989, 15 cases of bladder leiomyosarcoma were reported, it was state that more than 60% of tumors have aggressive characteristics, and the main clinical manifestation is gross hematuria(81%). There is no general consensus on the treatment, but it is considered as a highly aggressive entity that requires an aggressive surgical extirpation with negative surgical margins. The standard surgical technique (en bloc removal of the bladder with prostate and seminal vesicles in men and the uterus, cervix, and vaginal cuff in women, including extended pelvic lymph node dissection) may result in low rates of positive surgical margins and low rates of local tumor recurrence. Partial cystectomy with sole resection of the mass, even when tumor-free margins are achieved, is just a palliative treatment. The overall local recurrence rate of leiomyosarcoma is about 16%, and most recurrences are seen in the pelvis. 5-year disease-specific survival rates of 84% making bladder leiomyosarcoma survival rates better than the rates for patients with sarcomas of other organs.

Conclusion

Early detection and surgical intervention of the urinary bladder leiomyosarcoma can significantly improve overall survival. Herein, we present the case of high grade leiomyosarcoma, treated with minimally invasive surgery with negative surgical margins. Recent studies suggest that adequate radical surgical treatment with negative margins is able to achieve optimal cancer control outcomes.