RECONSTRUCTIVE SURGERY FOR CHILDREN WITH PELVIC RHABDOMYOSARCOMA - 05/09/11

Doi : 10.1016/S0094-0143(05)70096-3

Terry W. Hensle, MD ^a,^b, David T. Chang, MD ^a,^c

^a Department of Urology, College of Physicians and Surgeons, Columbia University (TWH, DTC)

^b Department of Pediatric Urology, The Babies and Children's Hospital of New York (TWH)

^c The J. Bentley Squier Urological Clinic, The New York Presbyterian Hospital, Presbyterian Campus (DTC), New York, New York

Résumé

Rhabdomyosarcoma is the most common soft-tissue sarcoma found in children and can arise almost anywhere skeletal muscle is found.^{23 McLaughlin K.P., Rink R.C. Bladder and prostate rhabdomyosarcoma Urologic Surgery in Infants and Children Philadelphia: WB Saunders (1998).

Cliquez ici pour aller à la section Références} It represents 4% to 8% of malignant solid tumors in children, ranking behind central nervous system tumors, lymphoma, neuroblastoma, and Wilms' tumor.^{29 Snyder H.M., D'Angio G.J., Evans A.E. , et al. Pediatric oncology Campbell's Urology Philadelphia: WB Saunders (1998).

Cliquez ici pour aller à la section Références} Genitourinary sites comprise approximately 20% of all pediatric rhabdomyosarcomas and represent a special subset of tumors arising from the bladder, prostate, paratesticular areas, vagina, uterus, and, rarely, the kidney and ureter.^{23 McLaughlin K.P., Rink R.C. Bladder and prostate rhabdomyosarcoma Urologic Surgery in Infants and Children Philadelphia: WB Saunders (1998).

Cliquez ici pour aller à la section Références}

Historically, radical surgery, usually involving total pelvic exenteration, was used as first-line therapy, resulting in survival rates of approximately 30%.^{23 McLaughlin K.P., Rink R.C. Bladder and prostate rhabdomyosarcoma Urologic Surgery in Infants and Children Philadelphia: WB Saunders (1998).

Cliquez ici pour aller à la section Références} These relatively poor cure rates were associated with significant social and emotional long-term sequelae in children who survived the extensive surgery.

Over the past 40 years, a dramatic change has occurred in the use of surgery in the overall treatment of rhabdomyosarcoma. In particular, cure rates are improved, and the role of radical surgery is diminished with advances in radiotherapy and with the development of more effective chemotherapeutic agents. Nonetheless, extirpative surgery still has an important role as an adjunctive treatment modality for genitourinary rhabdomyosarcoma.

Reconstructive surgery has become an integral part of the total plan in patients undergoing radical surgery for rhabdomyosarcoma. Advances in surgical techniques can often provide a reasonable lifestyle for patients.

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