Childhood cancer incidence in South Africa, 1987-2007.

BACKGROUND: Childhood cancer is an emerging problem in Africa. Its extent is hazy because data are scarce, but it should be addressed. This is the first report from the South African Children's Tumour Registry (SACTR), which covers the whole of South Africa (SA). It provides minimal estimates of cancer incidence and discusses the challenges of cancer surveillance and control in a child population in a middle-income country. Only about 2% of the African population is covered by cancer registries producing comparable incidence data. OBJECTIVE: To present and interpret incidence patterns and trends of childhood cancer over a 21-year period. The results should raise awareness of the problem of childhood cancer in an African population and provide sensible data for taking this problem in hand. METHODS: All eligible and validated cancer cases registered in the SACTR over the period 1987-2007 and classified according to the International Classification of Childhood Cancer were included. Population data were retrieved from official sources and estimated for the population subcategories. Incidence rates were standardised to the world standard and time trends were evaluated using joinpoint models, adjusting for sex and age. RESULTS: Based on the 11,699 cases, the overall age-standardised average annual incidence rate was 45 per million. Threefold differences in the overall incidence rates were observed between the ethnic groups, ranging from 116 for whites to 37 for black Africans, and they differed by diagnostic group. Differences between the nine provinces of SA relate to the ethnic composition and prevailing socioeconomic status. The overall incidence rate declined by 1.2% per year for the whole country (p<0.01). However, the decline was mainly observed during the first few years of the study period, after which rates stabilised or increased. CONCLUSIONS: Diagnosis and notification of childhood cancer should improve. The differences in incidence between ethnic groups suggest the priorities for cancer control.

The impact of ethnicity on wilms tumor: characteristics and outcome of a South african cohort.

Background. Nephroblastoma is the commonest renal tumour seen in children. It has a good prognosis in developed countries with survival rates estimated to be between 80% and 90%, while in Africa it remains low. Method. Retrospective study of patients diagnosed with nephroblastoma who are seen at 4 paediatric oncology units, representing 58.5% of all South African children with nephroblastoma and treated following SIOP protocol between January 2000 and December 2010. Results. A total of 416 patients were seen at the 4 units. Over 80% of our patients were African and almost 10% of mixed ethnicity. The most common stage was stage 4. The median survival was 28 months after diagnosis with the mixed ethnicity patients recording the longest duration (39 months) and the white patients had the shortest median survival. The overall 5-year survival rate was estimated to be 66%. Stage 2 patients did significantly better (85%). Conclusions. Our patients are similar with regard to gender ratio, median age, and age distribution as described in the literature, but in South Africa the more advanced stage disease seen than in other developed countries is translated into low overall survival rate.

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

Sialoblastoma and hepatoblastoma in a newborn infant.

We report a case of a newborn infant who had simultaneous sialoblastoma and hepatoblastoma tumours at birth. The diagnoses were made on post mortem examination. Both of these are rare tumours in the neonatal period.

Calvarial tuberculosis.

Tuberculosis of the skull is very rare. We report on two children: one presented with orbital and scalp swellings and was found to have lytic lesions on skull X-ray, while the other presented with chronic discharging scalp ulcers typical of tuberculosis of the scalp and also showed lytic skull lesions. Both patients had evidence of vertebral and lung tuberculosis.

Fanconi anemia. a statistical evaluation of cytogenetic results obtained from South African families.

Fanconi anemia (FA) is a rare autosomal recessive genetic disorder showing progressive bone marrow failure, and various phenotypic abnormalities. The lymphocytes show an increased sensitivity to the clastogenic agents diepoxybutane (DEB) or mytomycin C (MMC), measured as chromosomal aberrations. Statistical analysis of chromosome aberration yield showed that: (i) differentiation between obligate carriers and the control group was not possible; (ii) homozygotes were clearly distinguishable from heterozygotes as well as from controls by analyzing only 20 metaphase spreads per person; (iii) most of the FA patients had only one cell line present as measured by distribution of chromosomal damage among cells analyzed; (iv) and when the DEB sensitivity of a patient was high, the amount of cells without damage was low.

Imerslund-Gräsbeck syndrome in an African patient.

Imerslund-Gräsbeck syndrome (IGS) is a rare cause of megaloblastic anaemia in young children. We wish to report the first case described from Africa. The diagnosis of IGS was made on the findings of a low vitamin B12 level, mild proteinuria, and a vitamin B12 absorption test unaffected by the intrinsic factor. The patient responded well to treatment with intramuscular vitamin B12.

Sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy is a well recognised, but rare cause of lymphadenopathy in the first decade of life. Three cases presenting with nodal disease are described. The eyelids were involved in one case. The clinical, laboratory, and biopsy findings are discussed and compared with previously reported cases.

Pneumatoceles as a complication of paraffin pneumonia.

Paraffin pneumonia is a common form of poisoning, but pneumatoceles are an uncommon complication. This is a report of 6 patients who developed pneumatoceles after the ingestion of paraffin. These children's ages varied from under 18 months to 4 years; they were clinically more ill and were hospitalised longer than those who did not develop pneumatoceles. The pneumatoceles were discovered at the earliest on day 6 and, although extensive and in 2 cases bilateral, caused no clinical impairment of respiratory function. The pneumatoceles appear to resolve spontaneously but this may take more than 200 days.

Computerized tomography in pneumatocoeles after paraffin ingestion.

Paraffin pneumonia is a common form of poisoning but pneumatocoeles are an uncommon complication. This is a report of the computerized tomography (CT) of seven patients who developed pneumatocoeles after the ingestion of paraffin. The finding on the CT are presented and the situation and characteristics of the pneumatocoeles are indicated.

Salmonella meningitis. A report of 4 cases.

Four cases of salmonella meningitis occurring in young children from an area in which typhoid fever is endemic are described. The paucity of clinical signs and misleading initial lumbar puncture results in some cases are outlined, and the clinical picture, treatment and mortality are discussed.