Genito-urinary tumours can be divided into tumours of the urinary tract and tumours of the genital organs. Renal and ovarian tumours will be excluded from this chapter and we will focus on bladder-prostate tumours, female genital tract (vulva, vagina and uterus) and scrotal tumours (testicular and paratesticular tumours).
Bladder prostate tumours mainly consist of malignant mesenchymal tumours especially rhabdomyosarcoma. These tumours are usually chemosensitive and chemotherapy represents the first step of treatment. Local treatment is challenging in term of bladder function and late sequelae with an attempt to perform conservative surgical procedures associated or not with radiotherapy. Due to the very young age of the patients (median: 2 years), radiotherapy is best represented by brachytherapy. Eighty per cent of the patients with localized disease will be cured by the multidisciplinary treatment, with a permanent attempt to decrease the burden of therapy.
Malignant tumours of the female genital tract tumours (mainly rhabdomyosarcoma and vitellin tumours) are also very challenging in term of local control. They are also very sensitive to chemotherapy. In SIOP studies, 40 % of girls with RMS are cured without local treatment. Brachytherapy is the most interesting local treatment in case of residual disease after chemotherapy, with 90 % of cure rate and less than 20 % of vaginal stenosis, avoiding total vaginectomy and hysterectomy.
Scrotal tumours in prepubertal males are mainly testicular tumours, often benign (teratoma, stromal tumours) allowing to perform testis sparing surgery. For malignant tumours (germ cell tumours of the testis and paratesticular rhabdomyosarcoma), an adequate surgical procedure at diagnosis is mandatory (inguinal orchidectomy with high ligation of cord, avoiding scrotal contamination) to cure the patients without increasing post operative chemotherapy for inadequate surgical procedure. Prognosis is excellent for young males, worse for adolescents who often develop nodal metastasis.
In conclusion, local treatment is a key point in the multidisciplinary approach of genito-urinary tumours in children. The goal to decrease the burden of therapy and avoid long term sequelae without jeopardizing survival is obtained by adequate surgical procedures, associated or not with complementary radiotherapy techniques.
Numanoğlu KV, Tatli D. A rare cause of hemorrhagic shock in children: bladder hemangioma. J Pediatr Surg. 2008;43:e1–3.CrossRefPubMedGoogle Scholar
Demirca N, Mehme T, Cera N, et al. Urethral polyp in children: a review of literature and report of two cases. Int J Urol. 2006;13:841–3.CrossRefGoogle Scholar
Lot S, Lopez-Beltran A, Montironi R, et al. Soft tissue tumours of the urinary bladder. Part II: malignant neoplasms. Hum Pathol. 2007;38:7143–51.Google Scholar
Mc Kenney AK. An approach to classification of spindlecell proliferations in the urinary bladder. Adv Anat Pathol. 2005;12:312–23.CrossRefGoogle Scholar
Godzinski J, Flamant F, Rey A. Value of postchemotherapy bioptical verification of complete clinical remission in previously incompletely resected (stage I & II pT3) malignant mesenchymal tumours in children. International Society of Paediatric Oncology Malignant Mesenchymal Tumours study. Med Pediatr Oncol. 1984;1994(22):22–6.Google Scholar
Jenney M, Oberlin O, Audry G, et al. Conservative approach in localised rhabdomyosarcoma of the bladder and prostate: results from International Society of Paediatric Oncology (SIOP) studies: malignant mesenchymal tumour (MMT) 84, 89 and 95. Pediatr Blood Cancer. 2014;61:217–22.CrossRefPubMedGoogle Scholar
Hays D, Raney Jr RB, Lawrence Jr W, et al. Bladder and prostatic tumours in the intergroup rhabdomyosarcoma study (IRS I): results of therapy. Cancer. 1982;50:1472–82.CrossRefPubMedGoogle Scholar
Hays D, Raney RB, Wharam MD, et al. Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. J Pediatr Hematol Oncol. 1995;17:46–52.CrossRefPubMedGoogle Scholar
Arndt C, Rodeberg D, Philip P, et al. Does bladder preservation (as a surgical principle) lead to retaining bladder function in bladder/prostate rhabdomyosarcoma? Results from Intergroup Rhabdomyosarcoma study IV. J Urol. 2004;171:2396–403.CrossRefPubMedGoogle Scholar
Audry G, Oberlin O, Martelli H, et al. The role of conservative surgery in bladder/prostate rhabdomyosarcoma – an update of experience of the SIOP. Med Pediatr Oncol. 1998;31:198.Google Scholar
Crist WM, Anderson JR, Meza JL, et al. Intergroup rhabdomyosarcoma study-IV: results for patients with non metastatic disease. J Clin Oncol. 2001;19:3091–4102.PubMedGoogle Scholar
Seitz G, Dantonello TM, Int-Veen C, et al. Treatment efficiency, outcome and surgical the Rhabdomyosarcoma: a report from the cooperative soft tissue sarcoma trial CWS-96. Pediatr Blood Cancer. 2011;56:718–24.CrossRefPubMedGoogle Scholar
Haie-Meder C, Breton-Callu C, Oberlin O, et al. Brachytherapy in the treatment of vesicoprostatic rhabdomyosarcoma in children. Cancer Radiother. 2000;4:145–9.Google Scholar
Haie-Meder C, Mazeron R, Martelli H, et al. Brachytherapy role in pediatric rhabdomyosarcomas. Cancer Radiother. 2013;17:155–8.CrossRefPubMedGoogle Scholar
Cotter SE, Herrup DA, Friedmann A, et al. Proton radiotherapy for pediatric bladder/prostate rhabdomyosarcoma: clinical outcomes and dosimetry compared to intensity-modulated radiation therapy. Int J Radiat Oncol Biol Phys. 2011;81:1367–73.CrossRefPubMedGoogle Scholar
Heinzelmann F, Thorwarth D, Lamprecht U, et al. Comparison of different adjuvant radiotherapy approaches in childhood bladder/prostate rhabdomyosarcoma treated with conservative surgery. Strahlenther Onkol. 2011;187:715–21.CrossRefPubMedGoogle Scholar
Eastham JA, Kattan MW, Rogers E, et al. Risk factors for urinary incontinence after radical prostatectomy. J Urol. 1996;156:1707–13.CrossRefPubMedGoogle Scholar
Majoros A, Bach D, Keszthelyi A, et al. Urinary incontinence and voiding dysfunction after radical retropubic prostatectomy (prospective urodynamic study). Neurourol Urodyn. 2006;25:2–7.CrossRefPubMedGoogle Scholar
Sacco E, Prayer-Galetti T, Pinto F, et al. Urinary incontinence after radical prostatectomy: incidence by definition, risk factors and temporal trend in a large series with a long term follow-up. BJU Int. 2006;97:1234–41.CrossRefPubMedGoogle Scholar
Martelli H, Haie-Meder C, Branchereau S, et al. Conservative surgery + brachytherapy treatment for boys with prostate and/or bladder neck rhabdomyosarcoma: a single team experience. J Pediatr Surg. 2009;44:190–6.CrossRefPubMedGoogle Scholar
Martelli H, Boubnova J, Minard-Colin V, et al. Quality of life (QoL) of long-term survivors of bladder-prostate rhabdomyosarcoma (BPRMS), locally treated with conservative surgery and brachytherapy (BT). A single team experience. 45th Congress of the International Society of Paediatric Oncology (SIOP), Hong-Kong, 26–28 Sept 2013.Google Scholar
Andrassy R, Hays D, Raney RB, et al. Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from Intergroup rhabdomyosarcoma Study III. J Pediatr Surg. 1995;30:1034–7.CrossRefPubMedGoogle Scholar
Martelli H, Oberlin O, Rey A. Conservative treatment for girls with nonmetastatic Rhabdomyosarcoma of the genital tract: a report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol. 1999;17:2117–22.PubMedGoogle Scholar
Martelli H, Rey A, Spicer R. Rhabdomyosarcoma of the female genital tract. Overall results of MMT 84, 89 and 95 studies. On behalf of the SIOP MMT Committee. 40th annual conference of the International Society of Paediatric Oncology, Berlin, 2008. Online publication. http://journals.wiley.com/mpo. Wiley Interscience.
Kayton ML, Wexler LH, Lewin SN, et al. Pediatric radical abdominal trachelectomy for anaplastic embryonal rhabdomyosarcoma of the uterine cervix: an alternative to radical hysterectomy. J Pediatr Surg. 2009;44:862–7.CrossRefPubMedGoogle Scholar
De Lambert G, Haie-Meder C, Guerin F, et al. A new surgical approach of temporary ovarian transposition for children undergoing brachytherapy: technical assessment and dose evaluation. J Pediatr Surg. 2014;49:1177–80.Google Scholar
Magné N, Oberlin O, Martelli H, et al. Vulval and vaginal rhabdomyosarcoma in children: update and reappraisal of Institut Gustave Roussy brachytherapy experience. Int J Radiat Oncol Biol Phys. 2008;72:878–83.CrossRefPubMedGoogle Scholar
Baranzelli MC, Flamant F, De Lumley L, et al. Treatment of non metastatic, non seminomatous malignant germ cell tumour in childhood/ experience of the Société Française d’ oncologie pédiatrique MGCT 85–89 Study. Med Pediatr Oncol. 1993;21:395–401.CrossRefPubMedGoogle Scholar
Mann JR, Raafat F, Robinson K, et al. UKCCSG’s germ cell tumour (GCT) studies: improving outcome for children with malignant extracranial non-gonadal tumours – carboplatin, etoposide and bleomycin are effective and less toxic than previous regimens. Med Pediatr Oncol. 1998;30:217–27.CrossRefPubMedGoogle Scholar
Martelli H, Patte C, Lavrand F, et al. Malignant germ cell tumours of the vagina: report of the TGM 95 French study. Pediatr Blood Cancer. 2004;43:346.Google Scholar
Lacy J, Capra M, Allen L. Endodermal sinus tumour of the infant vagina treated exclusively with chemotherapy. J Pediatr Hematol Oncol. 2006;28:768–71.CrossRefPubMedGoogle Scholar
Senekjian EK, Frey KW, Anderson D, Herbst AL. Local therapy in stage I clear cell adenocarcinoma of the vagina. Cancer. 1987;60:1319–24.CrossRefPubMedGoogle Scholar
Morice P, Thiam-Ba R, Castaigne D, et al. Fertility results after ovarian transposition for pelvic malignancies treated by external irradiation or brachytherapy. Hum Reprod. 1998;13:660–3.CrossRefPubMedGoogle Scholar
Rushton HG, Belman AB, Sesterhenn I, et al. Testicular sparing surgery for prepubertal teratoma of the testis: a clinical and pathological study. J Urol. 1990;144:726–30.PubMedGoogle Scholar
Renedo DE, Trainer TD. Intratubular germ cell neoplasia (ITGCN) with p53 and PCNA expression expression and adjacent mature teratoma in an infant testis. Am J Surg Pathol. 1994;18:947–52.CrossRefPubMedGoogle Scholar
Kamal NM, Jouini R, Yahya S, Haiba M. Benign intrascrotal lipoblastoma in a 4-month-old infant: a case report and review of literature. J Pediatr Surg. 2011;46:E9–12. Review.CrossRefPubMedGoogle Scholar
Avallone AN, Avallone MA, Share S, et al. Epithelioid hemangioma-a rare scrotal tumour of childhood. Urology. 2012;80:707–9.CrossRefPubMedGoogle Scholar
Ferrari A, Bisogno G, Casanova M, et al. Paratesticular rhabdomyosarcoma: report from the Italian and German cooperative group. J Clin Oncol. 2002;20:449–55.CrossRefPubMedGoogle Scholar
Steward RJ, Martelli H, Oberlin O, et al. Treatment of children with non metastatic paratesticular Rhabdomyosarcoma: results of the MMT studies (MMT 84 and MMT 89) of the International Society of Pediatric Oncology. J Clin Oncol. 2003;21:793–8.CrossRefGoogle Scholar
Cecchetto G, de Corti F, Rogers T, et al. Surgical compliance with guidelines for paratesticular rhabdomyosarcoma (RMS). Data from the European Study on non metastatic RMS. J Pediatr Surg. 2012;47:2161–2.CrossRefPubMedGoogle Scholar