|Year : 2001 | Volume
| Issue : 2 | Page : 181-182
Rhabdomyosarcoma of the bladder
PM Deka, TP Rajeev
Department of Urology, Guwahati Medical College Hospital, Guwahati, India
P M Deka
Deptartment of Urology, Guwahati Medical College Hospital, Guwahati - 781 032, Assam
Source of Support: None, Conflict of Interest: None
Keywords: Rhabdomyosarcoma; Bladder; Chemotherapy
|How to cite this article:|
Deka P M, Rajeev T P. Rhabdomyosarcoma of the bladder. Indian J Urol 2001;17:181-2
| Introduction|| |
Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric age group and accounts for 5-10% of all childhood tumours.  Rhabdomyosarcoma originates in the pelvis or genitourinary tract in 13-20% of cases. Bladder and prostatic primaries account for 50% of genitourinary sarcomas.  Bladder primaries are usually of embryonal type and have a better prospect than other localizations. These pelvic tumors remain often unnoticed till the mass effect causes urinary and/or excretory symptoms.
| Case Report|| |
A 1½-year-old male child presented with history of gross/total/painless hernaturia with passage of clots of 3-months' duration. Straining during micturition was also present. On clinical examination: pallor present; pulse 96/ min, regular abdomen soft; bladder not palpable; external genitalia normal. Urine routine examination revealed plenty of RBC's/HPF, pus cells 15-18/HPF; culture was sterile. Hb%: 8 gin%, ESR: 46 mm AEFH, RFT was within normal limit. Chest X-ray and X-ray KUB did not reveal any abnormalities. Ultrasound scan KUB [Figure - 1] revealed a mixed echoic mass (3.5x2 cm) at bladder base just superior to the bladder neck. Contrast-enhanced CT scan abdomen [Figure - 2] showed a 4 x 2 cm lobulated mass in the posterio-lateral aspect of the bladder (bilaterally) with extension to bladder base, fat plane between bladder and rectum was preserved. Cystoscopy revealed papillary growth occupying both lateral walls/posterior wall and extending to the bladder neck. Biopsy was taken. Histopathology examination showed features of botryoid type of embryonal rhabdomyo sarcoma [Figure - 3]. We planned for total cystectomy and continent urinary diversion. Parents of the patient were not willing for a radical surgery or radiotherapy. Hence patient was put on intensive primary chemotherapy using vincristine, actinomycin D and cyclophosphamide (VAC regime).
Vincristine 2 mg/m 2 IV weekly for 13 doses (covering 12 weeks) starting day 0, followed by a dose at week 16.
Actinomycin D 15 µg/kg/day IV for 5 days starting day 0, day 84, day 112.
Cyclophosphamide 10 mg/kg/day IV 3 days starting day 0, day 84, day 112. Also one dose of 20 mg/kg was given on days 21, 42 and 63.
Starting week 20, the following course was repeated every 4 weeks through week 104.
Vincristine 2 mg/m 2 IV on days 0 and 4.
Actinomycin D 15 µa/kg/day IV for 5 days starting day 0.
Cyclophosphamide 10 mg/kg/day IV for 3 days starting day 0.
Patient tolerated the chemotherapy and showed complete response. Patient has been followed up for 30 months and has adequate bladder function till date.
| Comments|| |
The optimal mode of treatment for children with genitourinary sarcomas is not clearly established. Debate still continues as to the best mode of treatment for rhabdomyosarcomas of bladder and prostate. Traditionally, aggressive surgical resection has been the mainstay of treatment of these patients.  Organ preserving treatment policies have been advocated since it became evident that many rhabdomyosarcomas are sensitive to chemotherapy.  Extirpation of urogenital organs in children for embryonal rhabdomyosarcomas is a high price for cure of the disease. Radical extirpative surgery results in several serious problems including loss of normal rectal and bladder function, altered appearance and self-esteem and disturbance of normal sexual function. Advances in chemotherapeutic regimens and radiotherapy delivery systems have expanded the treatment options available. Today, pelvic exenteration is not the initial procedure of choice, but reserved for those not responding to initial chemotherapy or those relapsing after initial sucess. Careful monitoring is essential to identify late bladder deterioration and to avoid pressure induced injury to the upper urinary tract. We present here a case of botryoid type embryonal rhabdomyosarcoma, which showed complete response to primary chemotherapy and has adequate bladder function till date (i.e., 30 months after the start of treatment). Reports in literatures say that rhabdomyosarcoma of the bladder and prostate recurs late, frequently 2 to 3 years after the start of treatment  Therefore the question remains - can intensive primary chemotherapy alone render long-term cure?
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]