Geintourinary Malignancies in Children
ABSTRACT The modern management of pediatric genitourinary malignancies has resulted in survival rates that are dramatically better than figures from just a few decades ago. This is largely due to advances in multimodal treatment, collaborative efforts, and multidisciplinary management. Nevertheless, issues related to long-term side effects, treatment-related morbidity, and progression or recurrences remain important and pressing in terms of research directions and areas for improvement. In this Editorial Comment the author attempts to employ the current state of the art, masterfully summarized in the accompanying review by Drs Grimsby and Ritchey, to provide a view of trends that are likely to become increasingly important in the future, highlighting common patterns in treatment philosophy seen in other areas of oncology: more selective or patient-tailored treatment strategies, refined protocols and -whenever possible- tissue sparing and minimally invasive surgical interventions.
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ABSTRACT: Approximately 5% of children with Wilms tumor present with bilateral disease. The treatment challenge is to achieve a high cure rate while maintaining adequate long-term renal function. The authors of this report assessed the feasibility and outcome of nephron-sparing surgery in patients with bilateral Wilms tumor who were treated at a single institution. A retrospective review was performed of all children who were treated at St. Jude Children's Research Hospital for synchronous, bilateral Wilms tumors from 1999 through 2006. Imaging studies, surgical techniques, and pathology reports were reviewed. The outcomes evaluated included surgical complications, tumor recurrence, renal function, and patient survival. Twelve patients with synchronous, bilateral Wilms tumors were identified, including 10 patients who underwent successful bilateral nephron-sparing procedures. One patient who presented with renal failure and anaplastic histology underwent bilateral nephrectomies, and 1 patient with intra-atrial tumor extension underwent an ipsilateral nephrectomy/thrombectomy and subsequent contralateral partial nephrectomy. Postoperative complications included persistent urine leak in 3 patients, macroscopic residual tumor in 2 patients, and pyelonephritis in 1 patient. Long-term complications included local tumor recurrence in 2 patients, intestinal obstruction in 2 patients, ureteropelvic junction obstruction in 1 patient, and renal failure in 1 patient. The overall survival rate was 83% (mean follow-up, 3.9 years); both patients who died had bilateral, diffuse, anaplastic histology. All patients who had bilateral Wilms tumors with favorable histology, except for 1 patient who had extensive tumor thrombus, underwent successful bilateral partial nephrectomies. Complications were minimal, and long-term renal function and survival were excellent. From this experience, the authors concluded that bilateral nephron-sparing surgery should be considered for all patients who have bilateral Wilms tumor with favorable histology, even if preoperative imaging studies suggest that the lesions are unresectable.Cancer 06/2008; 112(9):2060-70. DOI:10.1002/cncr.23406 · 4.90 Impact Factor
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ABSTRACT: In children with testicular masses characteristic ultrasonographic features may occasionally allow the preoperative determination of a benign process and permit a testis sparing procedure to be performed. Often the amount of normal parenchyma surrounding the mass may be seemingly negligible, which may prompt some investigators to wonder what a testis sparing procedure might realistically spare. We retrospectively reviewed 7 consecutive patients with intratesticular lesions who underwent surgical exploration. Several of these children seemingly had complete replacement of the testis with the pathological process with little to no discernible normal parenchyma. The surgical technique involved delivery of the testis and control of the vessels. The capsule was incised and the normal parenchyma was bluntly separated to the level of the tumor. The mass was then mobilized circumferentially and enucleated intact. The residual parenchyma was reapproximated, the capsule was closed and the testicle was replaced in the scrotum. All patients were followed postoperatively by physical examination and ultrasonography. The series consists of 7 children 10 to 188 months old (mean age 68). The final diagnosis was cystic teratoma in 4 cases, epidermoid tumor in 2 and a simple cyst in 1. Postoperative physical examinations revealed symmetrical testes. Postoperative ultrasonography demonstrated normal echo texture and testicular volumes. Furthermore, no tumor recurrence was detected. Classic ultrasonographic patterns of testicular masses may permit preoperative identification of a benign process, permitting a testis sparing procedure to be planned. However, ultrasonography underestimates the amount of normal residual parenchyma because this tissue is compressed against the capsule into a thin rim. The amount of normal residual parenchyma identified on preoperative ultrasound should not be used as a factor when deciding whether a testis sparing procedure might be appropriate.The Journal of Urology 11/2007; 178(4 Pt 2):1730-2. DOI:10.1016/j.juro.2007.05.096 · 3.75 Impact Factor
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ABSTRACT: We determine patient and tumor characteristics, event-free and overall survival, methods of local control, rate of bladder preservation and proportion with normal bladder function for patients with localized bladder/prostate (BP) rhabdomyosarcoma (RMS) treated on the Fourth Intergroup Rhabdomyosarcoma Study (IRS IV). We reviewed the records of 90 patients with nonmetastatic BP RMS enrolled on IRS IV for presenting characteristics, details of therapy and outcome. Of the 90 records 88 had sufficient information for review. Patient age distribution was less than 1 year for 7 patients, 1 to 9 years for 71 and 10 or greater years for 10. Tumors commonly arose in the bladder (70%), had favorable histology (embryonal or botryoid 80%), large (69% greater than 5 cm), unresectable (84% group III) and invasive (56% T2). Local therapy included radiation in 74 patients, and most patients underwent second-look operations after radiation. All patients received alkylating based chemotherapy. With a median followup of 6.1 years there have been 3 second malignancies, 1 toxic death and 18 relapses, for an event-free survival rate of 77%. Bladders were retained without relapse at last contact in 55 patients. Of those 55 patients 36 and of the entire group 40% had normal function determined by history. Of patients with nonmetastatic BP RMS on IRS IV 82% survived 6 years. Bladder function was preserved in 55% (36/66) of event-free survivors. Of all patients entered on study 40% (36 of 88) survive event-free with apparently normal functioning bladders. More precise long-term evaluation of bladder and sexual function will require application of better tools such as urodynamic studies and validated patient surveys.The Journal of Urology 07/2004; 171(6 Pt 1):2396-403. DOI:10.1097/01.ju.0000142659.78358.f9 · 3.75 Impact Factor