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Surgical Management of Genitourinary Rhabdomyosarcoma

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The past few decades have witnessed unprecedented advances in the management of genitourinary rhabdomyosarcoma. The introduction of combination chemotherapy and adoption of multi-disciplinary approach in 1960s have resulted in dramatic improvement in the survival. Upfront radical surgery has been largely replaced with more conservative procedures resulting in less morbidity and improved function while maintaining survival. The discovery and incorporation of oncogenic-fusion proteins in rhabdomyosarcoma risk-stratification was another leap towards better outcomes.  Besides local control, surgery is crucial for risk-stratification, relief of urinary tract obstruction and reconstruction of urogential tract. Incomplete tumor resection has been associated with unfavorable outcome. Herein, we describe the evolution of role of surgery in the management of genitourinary rhabdomyosarcoma. Further, basics of surgical management are reviewed and areas of potential controversy are addressed. This review contains 11 figures, 5 tables, and 51 references. Keywords: rhabdomyosarcoma, children, bladder, prostate, multidisciplinary treatment, surgery, chemotherapy, cystectomy, survival, outcome, urinary diversion, fertility.
Title: Surgical Management of Genitourinary Rhabdomyosarcoma
Description:
The past few decades have witnessed unprecedented advances in the management of genitourinary rhabdomyosarcoma.
The introduction of combination chemotherapy and adoption of multi-disciplinary approach in 1960s have resulted in dramatic improvement in the survival.
Upfront radical surgery has been largely replaced with more conservative procedures resulting in less morbidity and improved function while maintaining survival.
The discovery and incorporation of oncogenic-fusion proteins in rhabdomyosarcoma risk-stratification was another leap towards better outcomes.
  Besides local control, surgery is crucial for risk-stratification, relief of urinary tract obstruction and reconstruction of urogential tract.
Incomplete tumor resection has been associated with unfavorable outcome.
Herein, we describe the evolution of role of surgery in the management of genitourinary rhabdomyosarcoma.
Further, basics of surgical management are reviewed and areas of potential controversy are addressed.
This review contains 11 figures, 5 tables, and 51 references.
Keywords: rhabdomyosarcoma, children, bladder, prostate, multidisciplinary treatment, surgery, chemotherapy, cystectomy, survival, outcome, urinary diversion, fertility.

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