Original Article Reconstructive option after radical mutilating surgery in children with genitourinary rhabdomyosarcoma: When sparing the bladder is not an option Leszek Komasara, 1 Joanna Stefanowicz, 2 Anna Bryks-Laszkowska, 1 Andrzej Gole z biewski 1 and Piotr Czauderna 1 1 Department of Surgery and Urology for Children and Adolescents, and 2 Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland Abbreviations & Acronyms ACS = appendiceal continent stoma AE = Abol-Enein principle/ method/technique AGLPP = artificially-generated leak point pressure B/P RMS = bladder/prostate rhabdomyosarcoma CCS = continent catheterizable stoma CET = common extramural tunnel CHT = chemotherapy CIC = clean intermittent catheterization COG = Children’s Oncology Group CR = complete remission CWS = Cooperative Weichteilsarkom Studiengruppe EFS = even free survival GU-RMS = genitourinary rhabdomyosarcoma IRS = Intergroup Rhabdomyosarcoma Study OS = overall survival RT = radiotherapy SET = separate extramural tunnel SIOP MMT = International Society of Pediatric Oncology Malignant Mesenchymal Tumor Group UIA = uretero-ileal anastomosis UIJ = uretero-ileal junction VUR = vesicoureteral reflux Correspondence: Joanna Stefanowicz M.D., Ph.D., Department of Paediatrics, Haematology and Oncology, Medical University of Gdansk, 7 Debinki Street, 80-952 Gdansk, Poland. Email: jstefanowicz@ gumed.edu.pl Received 3 August 2015; accepted 7 April 2016. Objectives: To present versatile surgical reconstructive techniques and their outcomes in pediatric patients with genitourinary rhabdomyosarcoma. Methods: We retrospectively analyzed the oncological and urological outcomes of seven patients treated between 1992 and 2014 according to the Cooperative Weichteilsarkom Studiengruppe protocols. Intergroup Rhabdomyosarcoma Study staging: local, six patients; and IV, one patient. Histology: embryonal, five patients; unclassified, one patient; triton tumor one patient. Surgical treatment included: cystectomy, uterectomy and partial vaginectomy, one patient; radical cystectomy, two patients; cystectomy, one patient; cystectomy with partial prostatectomy, one patient; partial cystectomy, one patient; and partial prostatectomy, one patient. Results: All patients were alive in complete remission at last follow up. In four cases, ileal conduit with ureteral reimplantation with serous-lined extramural tunnel (Abol-Enein technique) was carried out, which was followed by conversion into ileal continent bladder with continent appendiceal stoma for clean intermittent catheterization in three patients. In one boy, partial cystectomy and continent reconstruction was carried out during a single surgical procedure. One child with incontinent urinary diversion is still awaiting a continence solution. One child after partial prostatectomy is continent without any voiding disturbances. Conclusions: The timing and extent of radical surgery for treatment of genitourinary rhabdomyosarcoma depend on the local anatomical conditions, and the response to previous chemo- and radiotherapy. Cystectomy followed by various reconstructive techniques still remains an important option in the local treatment. Key words: bladder/prostate tumors, cystectomy, genito/urinary tract, reconstructive surgery, rhabdomyosarcoma. Introduction Approximately 25% of all pediatric soft tissue sarcomas in childhood arise from the genitouri- nary tract. 1–4 Genitourinary tract sarcomas are seen most frequently in the bladder and pros- tate. 2 Tumors localized in these regions have worse prognosis then paratesticular or vaginal tumors. 1,5,6 Bladder tumors tend to grow intraluminally in or near the trigone, and have a polypoid appearance. Bladder tumors tend to remain localized, but prostate tumors often dis- seminate early to the lungs, and sometimes to the bone marrow or bones. 2 GU-RMS most commonly are of embryonal histology (90%). Alveolar histology is distinctly unusual in GU- RMS. 2,4 Multimodal therapy involving surgery, chemotherapy and radiotherapy is necessary in B/P RMS, but the optimum use, timing and the intensity of these three treatment modalities must be planned with regard to known prognostic factors, including the location of the primary tumor, the extent of disease, the pathological subtype, age and the predicted sequelae of treat- ment. 1,3 Treatment of B/P RMS is generally based on a risk adapted approach and includes: surgical removal, radiotherapy for control of residual tumor bulk or microscopic residuum, and chemotherapy for primary citoreduction and eradication of metastases. 3 General principles © 2016 The Japanese Urological Association 1 International Journal of Urology (2016) doi: 10.1111/iju.13120