David Stones

Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence.

The high regional incidence of hepatocellular carcinoma (HCC) in South Africa also may be present in children of the region, although the link to hepatitis B (HBV) appears less clear. The objective of this study was to assess the incidence and probable causes of HCC in South African children.

Kaposi sarcoma in South African children

The incidence of Kaposis sarcoma (KS) in sub‐Saharan Africa, increased tens of times since the onset of the AIDS epidemic. There is, however, very little literature concerning the clinical features of this disease, its management and outcome in HIV‐positive children in Africa. This study describes retrospectively the clinical presentation of the malignancy, its management and outcome, in a series of HIV‐positive children.

Malignancies in South African children with HIV.

Objectives: In 2008 the South African Children’s Cancer Study Group decided to review the epidemiology, management, and chemotherapy response of HIV-positive children with malignancy. Methods: This is a retrospective analysis of data collected from the records of HIV-positive children diagnosed with malignancy at 7 university-based pediatric oncology units serving 8 of the 9 provinces in South Africa. Results: Two hundred eighty-eight HIV-positive children were diagnosed with 289 malignancies between 1995 and 2009. Age at diagnosis ranged from 17 days to 18.64 years; median 5.79 years. Of the 220 HIV-associated malignancies, there were 97 Kaposi sarcomas, 61 Burkitt lymphomas, 47 other B-cell lymphomas including 2 primary central nervous system lymphomas, 12 Hodgkin lymphomas, and 3 leiomyosarcomas. Sixty-nine patients presented with non–AIDS-defining malignancies. More than 80% presented with advanced disease. Most patients (76.7%) were naive to antiretroviral therapy; 22.2% did not have access because it only became available in public hospitals in 2004. One hundred ninety-seven children were treated with curative intent; 91 received palliative care due to advanced malignancy and/or advanced HIV disease. Nearly one third had coexisting pathology, mostly tuberculosis. Overall survival for the whole group was 33.7%, but was 57.8% for those treated with antiretroviral therapy and chemotherapy. Conclusions: The study shows more Kaposi sarcoma and fewer primary central nervous system lymphomas among HIV-positive children than that is reported in the developed world, but confirms a higher incidence of non-Burkitt B-cell lymphoma than in HIV-negative children. The high number of non–AIDS-defining malignancies underscores the prevalence of HIV-AIDS in South Africa. The overall survival should improve with universal access to antiretrovirals and earlier diagnosis.

Infection with human immunodeficiency virus-1 (HIV) among children with cancer in South Africa†

HIV increases the risk of certain cancers known, or thought, to have an underlying infectious etiology; the impact on the risk of cancer in children is less clear.

How much does it cost to treat children with Hodgkin lymphoma in Africa

Hodgkin lymphoma (HL) is a common B-cell childhood neoplasm and it has a higher incidence in the 0–14 year age group in developing countries compared to developed countries. Treatment achieves a cure rate of about 80%. In African countries with a small gross domestic product per capita the cost of treating HL in children may be prohibitive. To determine the direct costs of treatment of HL in South Africa and to propose a more cost-effective approach to investigation and treatment for children diagnosed with HL in Africa, tumor registry data for 138 children with HL from two South African hospitals were analysed retrospectively. The cost of treatment for stage 2 disease was calculated, including investigations and chemotherapy. The analysis included the cost of a follow-up period of 2 years. Stage 2 was the most common stage seen, and ABVD protocol was the most common protocol used. The total cost of diagnosing, staging, treating with chemotherapy and following up a child with stage 2 HL for 2 years post-therapy was ZAR 53178.20 = USD 6647.27 = EUR 4431.51. Follow-up expenditure was much higher than initial chemotherapy costs. The major factors driving the cost for the whole group of 138 patients were as follows: stage, radiologic imaging, radiotherapy, second-line chemotherapy, hospitalisation and febrile neutropenia. The total cost of treatment of HL is affordable for first world countries, but it remains expensive for developing countries, especially in Africa where the GDP is often under USD 2000 per head. Early diagnosis, use of less toxic protocols such as ABVD, close monitoring to prevent complications and elimination of unnecessary tests and investigations may reduce the overall cost.

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. Pediatr Blood Cancer 2009;52:883–885.

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.

Ethnicity and characteristics of Hodgkin lymphoma in children

To identify ethnic group differences in the prognostic of Hodgkin lymphoma (HL) in South African children.

Children With Cancer and HIV Infection: What is Different About Them?

Background: Children with HIV and cancer show an excess of non-Hodgkin lymphoma and leiomyosarcoma when compared with children with malignancy but without HIV. This study aimed at: (1) comparing the distribution of various cancers in South African children with malignancies and HIV versus children with malignancies but without HIV and (2) comparing the outcomes after therapy. Procedure: This is a retrospective comparative study of 84 black children with cancer and HIV, consecutively admitted at Tygerberg Children’s Hospital, Cape Town and Universitas Hospital, Bloemfontein, between 1995 and 2010, compared with 570 HIV-negative black children with malignant diseases, consecutively admitted at the 2 hospitals, between 2002 and 2010. Variables studied included age, sex ratio, number of cases of each malignancy, length of follow-up, treatment abandonment, and mortality. The statistical significance was tested with the Student t test and &khgr;2 test for P⩽0.05. Kaplan-Meyer survival curves were constructed. Results: The most frequent cancer was Kaposi sarcoma (39.3%), seen exclusively in children with HIV. Burkitt lymphoma was found more often (20.2%) in the HIV-positive group than in the HIV-negative one (2.8%, P<0.0001). Leiomyosarcoma, described as the second most frequent neoplasm encountered in HIV-positive children, was found in this series only once. The survival figures are much worse in the HIV-positive group: 26.2% versus 47.7% (P<0.0001), mainly due to drug toxicity. Conclusions: Kaposi sarcoma and Burkitt lymphoma occurred more often in children with HIV. These children have a lower tolerance of chemotherapy, even when combined with HAART.

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 Childrens 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.