Favourable outcomes for childhood acute lymphoblastic leukaemia in an isolated setting: The Tasmanian experience.

BACKGROUND: Tasmania is a large, relatively isolated island located south of mainland Australia with limited tertiary level paediatric oncology services. AIMS: To benchmark regional outcomes for childhood acute lymphoblastic leukaemia (ALL) against published international standards. METHODS: We undertook a retrospective cohort study, analysing the clinical characteristics and health outcomes of all children diagnosed with and treated for ALL in Tasmania, Australia between 2006 and 2015. RESULTS: Thirty-five patients aged less than 18 years were diagnosed with ALL in the study's 10-year period. Twenty-eight cases were precursor B cell in origin, with 7 cases of T-cell ALL. The great majority of children (30/35; 86%) received their entire first line treatment in Tasmania. Major treatment-related toxicities, including allergic drug reactions, and episodes of acute pancreatitis, deep venous thrombosis and bacterial sepsis, were managed locally, with one death secondary to overwhelming infection and multiorgan failure. The overall and event-free survival rates for childhood ALL were 30/35 (86%) and 28/35 (80%), respectively. CONCLUSIONS: These results compare favourably with published results from large international cooperative group trials based in developed countries. Continued local treatment with appropriate support from a dedicated specialist paediatric oncology unit is therefore justified.

Refuelling of vehicles, the use of wood burners and the risk of acute lymphoblastic leukaemia in childhood.

It is plausible that exposure of the parents before birth or of the child to sources of benzene increases the risk of childhood acute lymphoblastic leukaemia (ALL). The aim of this analysis was to investigate whether refuelling a vehicle with petrol before birth or burning wood to heat the home before or after the child's birth increased the risk of childhood ALL. Data from 389 cases and 876 frequency-matched controls were analysed using unconditional logistic regression, adjusting for study matching factors and potential confounders. The odds ratio (OR) for the mother ever refuelling a vehicle with petrol for non-occupational purposes before or during the pregnancy was 0.97 [95% confidence interval (CI) 0.69, 1.38]. The OR for the father for this exposure in the year before conception was 0.88 [95% CI 0.52, 1.48]. The OR for use of a closed wood burner to heat the home in the year before or during pregnancy was 1.41 [95% CI 1.02, 1.94] and 1.25 [95% CI 0.92, 1.70] after birth. We found no evidence that non-occupational refuelling a vehicle with petrol in the year before or during pregnancy increased the risk of ALL in the offspring. There was weak evidence that burning wood in a closed burner to heat the home increased the risk, but there was no dose-response relationship and chance could explain the finding.

Bilateral Wilms' tumors: a single-center experience with 19 cases.

BACKGROUND/PURPOSE: Bilateral Wilms' tumors present a therapeutic challenge, particularly with delay in presentation, when there is poor response to chemotherapy and when associated with nephroblastomatosis. The primary aim of management is eradication of neoplasm, while at the same time preserving of renal function. METHODS: Nineteen bilateral Wilms' tumors were seen in our service between 1981 and 2003. This represented 10% of the 190 patients with Wilms' tumors seen during this period. There were 12 female and 7 male patients ranging in age from 7 months to 8 years. Fourteen had synchronous presentation, one of whom had liver metastasis at diagnosis. Of the 5 patients with metachronous tumors, 3 had their initial nephrectomies done elsewhere. Nephroblastomatosis was identified in 18 (95%) of the patients. Treatment was, in most cases, according to National Wilms Tumor Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumorectomy. Where indicated, nephrectomy (partial or complete) involved using ice dam topical cooling and vascular control, and in one case, bench surgery and extensive renal reconstruction with orthotopic autotransplantation. Revision tumorectomy was used on 3 occasions for recurrence in areas of nephroblastomatosis. There were 6 extrarenal relapses. RESULTS: Ten patients are alive and free of disease 1 to 15 years after treatment, all with well preserved renal function (lowest recorded glomerular filtration rate was 85 mL/min per 1.73 m2). Nine have died (2 of unrelated disease), including 6 of the 7 with spread outside the kidney. All 3 with unfavorable histology are alive. Of the 5 with metachronous presentations, 4 are alive, as are 7 of 10 who presented in the last decade. CONCLUSIONS: Appropriate chemotherapy and conservative nephron-sparing and innovative surgery can achieve good results with preservation of adequate renal function in nearly all cases. Nephroblastomatosis was an almost universal finding and requires close monitoring because Wilms' tumors developed in residual suspect areas. Revision surgery was effective. Unfavorable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.