|Year : 2004 | Volume
| Issue : 2 | Page : 63-66
Cystectomy with orthotopic neobladder ; an option for giant leiomyoma of urinary bladder : A case report and the review of literature
Kamal Jeet Singh, Amit Suri, Prashant Lavania, Anil Mandhani
SGPGIMS, Lucknow, India
Department of Urology, SGPGIMS, Rae Barelli Road, Lucknow
Source of Support: None, Conflict of Interest: None
Keywords: Bladder leiomyoma, cystectomy
|How to cite this article:|
Singh KJ, Suri A, Lavania P, Mandhani A. Cystectomy with orthotopic neobladder ; an option for giant leiomyoma of urinary bladder : A case report and the review of literature. Indian J Urol 2004;20:63-6
|How to cite this URL:|
Singh KJ, Suri A, Lavania P, Mandhani A. Cystectomy with orthotopic neobladder ; an option for giant leiomyoma of urinary bladder : A case report and the review of literature. Indian J Urol [serial online] 2004 [cited 2020 May 26];20:63-6. Available from: http://www.indianjurol.com/text.asp?2004/20/2/63/37177
| Case Report|| |
47 year old female presented with obstructive and irritative voiding symptoms and hematuria for last 3 years. On examination patient had gross pallor and palpable suprapubic lump. Ultrasonography revealed a large hyperechoic mass with bilateral hydroureteronephrosis. There were multiple echogenic masses in uterus suggestive of fibroids. CT scan revealed heterogeneously enchancing polypoidal soft tissue mass inside the bladder lumen with diffuse thickening of bladder wall with an exophytic component which was inseparable from the uterus and extending into the left adnexa with dilated uterine cervix [Figure - 1], [Figure - 2]. On cystoscopy tumor was broad based and was arising from the trigone and the posterior wall of bladder but ureteric orifices were free of tumor. TUR biopsy was done which showed features of Leiomyoma. Patient underwent cystectomy and hysterectomy with orthotopic neobladder using ileum. Postoperative course of the patient was uneventful and she was discharged on CIC. Patient is doing fine and voiding on her own eight months postoperatively.
| Comments|| |
Leiomyoma of urinary bladder though rare, yet is the most common benign mesenchymal tumor of the urinary bladder and accounts for less than 0.43% of all bladder tumors , . Around 200 cases of this tumor have been reported in literature  .
Though most common site is kidney capsule, it can occur anywhere in the genitourinary system . They can weigh from few grams to up to 9 kilograms  . Their size can vary few mil limeter to as large as 30 cm ,
Exact pathogenesis is unknown but following possible theories have been put forward. Hormonal influences could result in simultaneous occurrences of leiomyoma in urinary bladder and uterus or mbryonic rests of tissues residing in bladder may develop into leiomyoma. Another theory suggests perivascular and muscular inflammation leading to metaplastic transformation and development of these tumors  .
Leiomyoma was considered to occur with equal incidence in either gender  , but recent studies indicate much higher incidence in females (76%) 
Clinical Presentation and Diagnosis
Most of leiomyomas are asymptomatic and incidentally detected on routine gynaecological examination. Patients with leiomyoma present with variety of symptoms depending upon location of the tumor [Table - 1]. Leiomyoma producing obstructive symptoms are detected earlier, but 2 cases have been reported with renal failure  .
Hematuria is not a common symptom but occurs when tumor outgrows the blood supply as in present case. 39% patients have concomitant uterine fibroids and 50% of these tumors were palpable on bimanual examination 
On ultrasonography, leiomyoma appears smooth walled lesion with numerous internal echoes and homogenous echotexture. Echogenicity of the tumor depends upon the fibrous content. There can be presence of concomitant fibroids uterus and when these tumors are obstructive, hydro-ureteronephrosis maybe seen .
On excretory urography, leiomyoma appears as a smooth filling defect in the bladder and spmetimes with ureteric dilatation and hydronephrosis.
Ultrasonography has been found to be superior than IVU in diagnosis, localization and measurement of leiomyomas  . Ultrasound has an ability to document the solid nature of the tumor, its sub mucosal location, site of origin and relationship with other organs  . On CT scan, these tumors appear smooth filling defect with attenuation values of 25-50 Hounsfield units. The drawback is in larger tumors, the planes between surrounding organs could not be appreciated as it was so in the present case. On MRI, leiomyomas give an intermediate intensity on T1 weighted images giving good contrast between it and low intensity of the urine. On T2 weighted images it gives foci of high and low intensity along with good contrast between the tumor and intermediate bladder muscle making extravesical spread determination easy ,
They are similar to uterine leiomyomas. Grossly they are firm, circumscribed, whorled in appearance and white tan in color. Usually there is no necrosis or hemorrhage with in the tumor. In bladder they can occur as endovesical(63%), intramural(7%) or extravesical (30%)  , while Goluboff et al reported occurrence of endovesical in 86%, extravesical 11% and intramural in 3% in their series of patients  .
Microscopically, there are fascicles of smooth muscles with moderate amount of eosinophillic cytoplasm separated by connective tissue. The degree of vascularity of tumor varies. Rarely epitheloid cells may be seen. The mitotic activity is rare in leiomyoma but if present it is usually less than two per HPF. Mills et. al. had reserved the diagnosis of leiomyoma of bladder for smooth muscle tumor with low cellularity and minimal to absent mitotic activity 
On immuno-hisotchemistry, these tumors are strongly positive for smooth muscle actin, muscle specific actin, desmin and vimentin, but are negative for cytokeratin, intermediate filament, s-100 and c-kit  .
Most of these tumors are diploid(62%), while 25% are near diploid and 12.5% aneuploid  .
As in case of uterine leiomyomas treatment is offered to symptomatic patients and nonincidentally detected tumors. Growth of leiomyoma is not an indication for surgery as risk of developing leiomyosarcoma is only 0.27% as compared to a risk of 0.23% in leiomyoma of stable size  . Various parameters are used in determining the type of surgery like - size, site, extent of lesion and involvement of sphincters or ureters.
In general smaller lesions are managed by transurethral resection (TUR) with low re-operation rates while larger lesions require open surgery, varing from a simple enucleation, partial cystectomy to cystoprostatectomy with reconstruction of bladder. 
Enucleation of large trigonal leiomyomas is described which gives a good surgical outcome but tumor involving full thickness of bladder wall are not suitable for transurethral resection even if they are small  More so, even after complete enucleation, tumor recurrence has been reported , With this concern and presence of deep seeded broad base tumor at the trigone in the present case, decision of cystectomy with orthotopic continent diversion was taken. On 8 months of follow up the patient is doing fine, who otherwise would have had uncertain outcome with conservative treatment.
The option of such a surgery should be considered in large benign leiomyoma with diffuse involvement in order to give a definite cure to the patient.
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[Figure - 1], [Figure - 2]
[Table - 1]