The Association between Tumour Markers and Meta-iodobenzylguanidine Scans in South African Children with High-risk Neuroblastoma.

AIMS: Diagnostic and post-induction 123I-meta-iodobenzylguanidine (123I-mIBG) scans have prognostic significance in the treatment of neuroblastoma, but data from low- and middle-income countries are limited due to resource constraints. The aim of this study was to determine the association between neuroblastoma-associated tumour markers (lactate dehydrogenase [LDH], ferritin and MYCN amplification) and 123I-mIBG scans (modified Curie scores and metastatic disease patterns) in predicting complete metastatic response rates (mCR) and overall survival. MATERIALS AND METHODS: Two hundred and ninety patients diagnosed with high-risk neuroblastoma in South Africa between January 2000 and May 2018 and a subanalysis of 78 patients with diagnostic 123I-mIBG scans were included. Data collection included LDH, ferritin and MYCN amplification at diagnosis. Two nuclear physicians independently determined the modified Curie scores and pattern of distribution for each diagnostic and post-induction 123I-mIBG scans with high inter-rater agreement (r = 0.952) and reliability (K = 0.805). The cut-off values for the diagnostic and post-induction modified Curie scores of ≥7.0 (P = 0.026) and 3 (P = 0.009), respectively, were generated. The association between the tumour markers and the modified Curie score of the 123I-mIBG scans was determined using post-induction mCR and 2-year overall survival. RESULTS: Diagnostic LDH (P < 0.001), ferritin (P < 0.001) and the diagnostic modified Curie scores (P = 0.019) significantly predicted mCR. Only ferritin correlated with diagnostic modified Curie scores (P = 0.003) but had a low correlation coefficient of 0.353. On multivariable analysis, the only significant covariate for 2-year overall survival at diagnosis was LDH <750 U/l (P = 0.024). A post-induction chemotherapy modified Curie score ≤3.0 had a 2-year overall survival of 46.2% compared with 30.8% for a score >3.0 (P = 0.484). CONCLUSION: LDH, ferritin and the diagnostic 123I-mIBG scans significantly predicted mCR, but only LDH predicted 2-year overall survival. Ferritin and the modified Curie scores correlated with each other. MYCN amplification neither correlated with any aspect of the 123I-mIBG scans nor significantly predicted mCR or 2-year overall survival. LDH and ferritin are therefore appropriate neuroblastoma tumour markers to be used in low- and middle-income countries with limited or no access to mIBG scans and/or MYCN amplification studies.

Nephroblastoma--a 25-year review of a South African unit.

RATIONALE: To determine the outcome of patients with nephroblastoma in a South African hospital. OBJECTIVE: To determine if there is a difference in the outcome of patients with nephroblastoma comparing two treatment protocols SIOP (Société International D'Oncologie Pédiatrique Protocol) versus NWTS (National Wilms' Tumour Study Protocol). METHODS AND RESULTS: A retrospective audit of 25 years (1983-2007), of children diagnosed with nephroblastoma in Tygerberg Hospital. One hundred and seven patients were included in the study and 98 were analyzed. The average age at diagnosis was 3.8 years. Most patients (37%) presented with stage 1 of the disease, followed by patients with stage 3 (27%). Most patients were treated according to the SIOP protocol (61%). Gender and race did not influence the outcome. Patients with stage 1 and 2 of the disease had the best outcome (76% versus 43% for stages 3 and 4). The SIOP group had a better outcome than the NWTS group (p value 0.001). The two groups had an equal distribution of the stage of presentation. The tumor volumes were bigger in the NWTS group (1004cm3 compared to 613cm3). Nutritional status did not influence the outcome although more patients were underweight for age in the SIOP group. The statistical methods used were: Kaplan Meier, Gehan's Wilcoxon Test, Chi -square test and the Fisher exact test. DISCUSSION: Contrary to the other studies, patients treated according to the SIOP protocol had a statistically significant better outcome. Larger collaborative studies are needed to investigate this result in Africa.

The Cost of Nephroblastoma Treatment in South Africa: A Very Cost-Effective Investment with Guidelines for the Rest of Africa

Background. Nephroblastoma is one of the most common childhood malignancies in Africa; but with a survival rate significantly lower than in developed countries. In African countries with a small gross domestic product (GDP) per capita; the cost of treating nephroblastoma may be prohibitive. Objectives. To determine the direct costs of treatment of nephroblastoma in South Africa (SA) and to propose a more cost-effective approach to investigations and treatment for the disease in Africa. Methods. Data from 2000 - 2010 from two SA paediatric oncology units were retrospectively analysed. The costs included investigations; chemotherapy and radiotherapy; comparing early-v. advanced-stage disease. In both units; the nephroblastoma International Society of Paediatric Oncology (SIOP) protocol was used. Results. Stage I disease was the most common; followed by stage IV. The total cost of diagnosis; staging and treatment of stage I disease was ZAR9 304.97 (EUR882.80 or USD1 093.40); compared with a five-times higher cost for stage IV (ZAR48 293.62 (EUR4 581.9 or USD5 674.9)). Treating one patient averted more than 32 disability adjusted life years. The investigation and treatment of early- and advanced-stage disease is very cost-effective when compared with the local GDP per capita. Conclusion. The cost of investigation and treatment of nephroblastoma remains a challenge everywhere; but especially in Africa. However; it is a very cost-effective disease to treat and children in Africa should not be denied treatment

Hemopure transfusion in a child with severe anemia.

A 23-month-old girl presented with heart failure from extremely severe sickle cell anemia. The family refused blood transfusion on religious grounds (Jehovah's Witness). Alternative options acceptable to this religion, such as iron, erythropoietin, or folic acid were rejected as useless in the particular situation of the child. The patient was transfused with Hemopure, a product that consists of polymerized bovine hemoglobin. This is the first case reported in the literature of a child transfused, in an emergency situation, with this product.

Viral isolates during febrile neutropaenia in children with cancer.

We prospectively studied South African children with cancer for viral isolates during episodes of febrile neutropaenia. Viruses were found in seven (31.8 per cent) and bacteria in five (22.7 per cent) of 22 episodes. The most common isolate was the herpes simplex virus and the most common source was from nasopharyngeal aspirates. There was no dual detection of viral and bacterial isolates. This study emphasizes the important contribution of viruses to febrile neutropaenia.