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CASE REPORT
Year : 2001  |  Volume : 17  |  Issue : 2  |  Page : 181-182
 

Rhabdomyosarcoma of the bladder


Department of Urology, Guwahati Medical College Hospital, Guwahati, India

Correspondence Address:
P M Deka
Deptartment of Urology, Guwahati Medical College Hospital, Guwahati - 781 032, Assam
India
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Source of Support: None, Conflict of Interest: None


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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

How to cite this URL:
Deka P M, Rajeev T P. Rhabdomyosarcoma of the bladder. Indian J Urol [serial online] 2001 [cited 2019 Aug 20];17:181-2. Available from: http://www.indianjurol.com/text.asp?2001/17/2/181/21062



   Introduction Top


Rhabdomyosarcoma is the most common soft tissue sar­coma in the pediatric age group and accounts for 5-10% of all childhood tumours. [1] Rhabdomyosarcoma originates in the pelvis or genitourinary tract in 13-20% of cases. Blad­der and prostatic primaries account for 50% of genitourinary sarcomas. [2] 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 Top


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).

Chemotherapy Schedule:

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 Top


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. [3] Organ preserving treatment policies have been advocated since it became evident that many rhabdomyosarcomas are sensitive to chemotherapy. [4] 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 [5] Therefore the question remains - can intensive primary chemotherapy alone render long-term cure?

 
   References Top

1.Young JL, Miller RW. Incidence of malignant tumors in US chil­dren. J Pediatr 1975: 86: 254-258.  Back to cited text no. 1    
2.Exelby PR, Ghavirni F. Jereb B. Genitourinary rhabdomyosarcoma in children. J Pediatr Surg 1978; 13: 746-752.  Back to cited text no. 2    
3.Grosfeld JL. Weber TR. Weetman RM et al. Rhabdomyosarcoma in childhood: Analysis of survival in 98 cases. J Pediatr Surg 1983; 18: 141-146.  Back to cited text no. 3    
4.Voute PA, Vos A. Combination chemotherapy as primary treatment in children with rhabdomyosarcoma to avoid mutilating surgery or radiotherapy. In: Proceedings of the 13th annual meeting of the American Society of Clinical Oncology 1977: C244: 327.  Back to cited text no. 4    
5.Raney RB Jr, Gehan EA. Hays DM et al. Primary chemotherapy with or without radiation therapy and/or surgery for children with localized sarcoma of the bladder, prostate. vagina, uterus and cer­vix: A comparison of the results in Intergroup Rhabdomyosarcoma studies i and II. Cancer 1990: 66: 2072-2081.  Back to cited text no. 5    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]



 

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    Introduction
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