Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience.

PURPOSE: In a retrospective study we analyzed the outcome of patients treated for rhabdomyosarcoma (RMS) of the bladder/prostate with special attention to radical surgery. METHODS: In 25 patients with genitourinary RMS (15 bladder/10 prostate) the median age at diagnosis was 4 years [1-18], and 8 patients had a stage II RMS, 12 stage III and 5 stage IV. In 19/25 (12 bladder/7 prostate), radical surgery and urinary diversion were performed. Urinary diversion comprised 2 continent anal diversions, 11 continent cutaneous diversions, 4 colon conduits and 2 urethral diversions (2 + 3 years of age). In the younger child with urethral diversion, a cutaneous appendix stoma was additionally constructed in case of inability to void spontaneously. RESULTS: 4/19 patients who underwent radical surgery died of metastatic RMS; 1 patient with neurofibromatosis died of a secondary tumor. After median follow-up of 132 months (14-420), 14 patients currently have no evidence of disease. 8/14 patients who survived developed 17 complications requiring operative revision. All patients with a continent diversion are continent. The patients with orthotopic bladder substitution are continent day & night and void spontaneously. CONCLUSION: For RMS confined to the bladder or bladder neck, radical cystoprostatectomy and orthotopic bladder substitution are an option. Urethral diversion using the ileocecal segment (Mainz-pouch I) offers the advantage of utilizing the appendix as an additional continent cutaneous stoma, which enables parents to evacuate residual urine in young boys, until able to empty the pouch completely themselves. For all other patients with vital tumor after primary chemotherapy, cutaneous urinary diversion is an option. Long-term complication rates in this complex group of patients are acceptable.

Late structural alterations of cerebral white matter in long-term survivors of childhood leukemia.

PURPOSE: To look for the presence and age-dependence of late structural alterations of otherwise normal-appearing cerebral gray and white matter after radiation and chemotherapy in adult survivors of acute lymphoblastic leukemia (ALL) during childhood. MATERIALS AND METHODS: In a group of 13 adult survivors 17-37 years old, who had been treated by total brain radiation (18-24 Gy) and chemotherapy 16-28 years ago, prospective MR examinations including diffusion tensor imaging (DTI) were performed. Evaluation included volumetry, calculation of mean diffusivity (MD) and fractional anisotropy (FA), and comparison of results to an age-matched control group. RESULTS: DTI showed significantly reduced FA values in the temporal lobes (difference of 0.069 units, P < 0.001), hippocampi (difference of 0.033 units, P < 0.001), and thalami (difference of 0.046 units, P = 0.001), which were accompanied by significant white matter volume loss (difference of 92 cm(3), P < 0.001). Significant elevations of MD were limited to the temporal white matter (difference of 42 x 10(-6) mm(2)/s, P = 0.005). Global and frontal white matter MD correlated negatively to increasing age of the survivors (P < 0.01). CONCLUSION: With regard to structural white matter alterations, adult long-term survivors of childhood ALL, who had received total brain radiation and chemotherapy, apparently show the same overall age dependence as controls. Follow-up studies are needed for confirmation.

Recovery kinetics of heart rate and oxygen uptake in long-term survivors of acute leukemia in childhood.

Analysis of the recovery period following physical exercise has gained importance in evaluating cardiopulmonary capacity, not only in athletes but also in patients with proven or suspected heart failure. The purpose of this study was to apply these methods to long-term survivors of acute lymphoblastic leukemia (ALL) in childhood, who are at risk of developing anthracycline-induced cardiomyopathy. Nine children (mean age 12 years) and 10 adults (mean age 24 years) were included in the study after treatment for childhood ALL. Recovery of oxygen uptake and heart rate following maximal spiroergometric exercise was compared to that in 29 trained and untrained age-matched controls. The change in oxygen uptake (DeltaVO2) and heart rate (DeltaHR) between maximal effort and 60 s of recovery did not differ significantly, either between children after oncological therapy (DeltaVO2: 14.95 ml/kg, DeltaHR: 35 bpm) and healthy children (DeltaVO2: 15.85 ml/kg, DeltaHR: 37 bpm), or between adult former oncological patients (DeltaVO2: 13.1 ml/kg, DeltaHR: 27 bpm) and untrained adults (DeltaVO2: 15.7 ml/kg, DeltaHR: 31 bpm). There was, however, a significant difference in DeltaVO2 between trained adults (DeltaVO2: 24.5 ml/kg) and both untrained adult controls (DeltaVO2: 15.7 ml/kg, p=0.004) and adult patients (DeltaVO2: 13.1 ml/kg, p=0.0002). This difference was not detected for heart rate. In conclusion, the recovery period did not reveal a discernible difference in cardiopulmonary capacity between former ALL patients and untrained age-matched controls. We did confirm that heart rate and oxygen uptake recovery serve as indicators of physical fitness.

Thioguanine offers no advantage over mercaptopurine in maintenance treatment of childhood ALL: results of the randomized trial COALL-92.

The German cooperative study group for childhood acute lymphoblastic leukemia (COALL-92) was designed to examine the clinical effectiveness of thioguanine (TG) versus mercaptopurine (MP) in maintenance treatment of childhood acute lymphoblastic leukemia (ALL) in a randomized multicenter trial. TG and MP are prodrugs and have to be converted intracellularly to 6-thioguanine nucleotides (TGNs) for cytostatic activity. TG is converted into TGN in fewer steps and has been shown to be more cytotoxic in equimolar doses in vitro compared with 6-MP. Therefore, a higher effectiveness of TG in maintenance treatment was postulated. Of 521 patients enrolled into the protocol, 474 were randomized to receive either MP or TG during maintenance therapy in a daily oral dose. After a median observation time of 6.6 years, the probability of event-free survival was 79% +/- 3% for the MP group (238 children) and 78% +/- 3% in the TG group (236 patients). In spite of TGN levels, exceeding those of the MP group 7 times, treatment with TG did not improve the outcome but was more complicated to handle due to a specific toxicity profile of prolonged myelosuppression with marked thrombocytopenia. Therefore, MP should remain the preferred drug for maintenance treatment of ALL, unless other studies demonstrate superiority of TG in larger trials or selected patient groups.