We present a 7-month-old male infant with stage I Wilms tumor who unexpectedly died from a catastrophic intracerebral hemorrhage, 4 months after completion of chemotherapy and complete surgical ...resection of the tumor. The precise etiology underlying the fatal event remains unclear as postmortem was refused, but we postulate spontaneous hemorrhage from an underlying cerebral vascular malformation as the most likely cause, which led to the child's unfortunate demise. Although extremely rare, cerebral vascular anomalies have previously been reported in children with Wilms tumor. The coexistence of the 2 uncommon disorders may be related to their congenital origin. Wilms tumor diagnosed in very young infants have clinical and morphologic attributes that do not pertain in older children and the risk of associated congenital anomalies is also much higher among those discovered in the first year of life. This raises the question whether routine magnetic resonance imaging should not be performed in infants less than a year with Wilms tumor, as part of the initial evaluation, to exclude cerebral metastases and underlying vascular malformations.
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.
Data were obtained from seven participating pediatric oncology units and from the tumor registry to review hepatic tumors in children in South Africa.
One hundred ninety-four children (ages 0-14 years) presented with malignant primary hepatic tumors (1988-2003). One hundred twelve tumors (57%) were hepatoblastoma (HB), 68 tumors (35%) were hepatocellular carcinoma (HCC) (including 9 patients with the fibrolamellar variant, 6 of which occurred in black children), 10 tumors (5%) were sarcoma of the liver, and 4 tumors were lymphoma. The ratio of HB to HCC (1.67) was markedly lower compared with other reports, suggesting a greater prevalence of HCC. Correlation with population statistics indicated an incidence of 1.066 malignant liver tumors per year per 10(6) children age < 14 years (HB, 0.61 per 10(6) children; HCC, 0.39 per 10(6)). Two-thirds of patients with HCC were positive for HBV surface antigen (HBsAg), and HCC occurred mostly in black African patients (93%). The mean age of onset was 1.47 years for HB and 10.48 years for HCC. A preponderance of males (3.5:1.0) was noted in the HBsAg-positive group that was not reflected elsewhere. Serum alpha-fetoprotein (AFP) levels were raised both in patients with HB (100%; most AFP levels were very high) and in patients with HCC (69%), although 15% of patients with HCC had low or normal AFP levels.
It appeared from the current results that HCC is more prevalent among children in South Africa compared with the children in more developed countries, although their rates were lower that the rates noted in adults. A collaborative approach will be required to improve their diagnosis and management.
Introduction. Idiopathic thrombocytopenic purpura (ITP) is the most common bleeding disorder of childhood. Aim. To describe the management of ITP in South Africa. Methods. A survey was sent to 410 ...doctors in the country, describing four different scenarios in children newly diagnosed with ITP and soliciting responses concerning the diagnosis and management. Results. Steroids were the first line of choice in treating ITP. Anti-D immunoglobulin was not considered in the management, and most practitioners would perform a bone marrow aspiration even if no treatment with steroids would be given. The vast majority of the patients would be treated in hospital. Conclusions. This is the first study done in South Africa (and in Africa), and it shows a great variation in the management of children with newly diagnosed ITP. Prospective studies in developing countries where various constraints to health care delivery exist are required to produce evidence-based recommendations for this patient group.
Hodgkin’s disease (HD) in children corresponds to a large degree to HD in adults. Non-Hodgkin’s Lymphoma (NHL) in children, however, differs from NHL in adults with respect to the classification, ...natural history, management and course. For practical reasons clinicians generally classify and treat NHL in children as either B-cell or T-cell disease.
Over the past 22 years, the Paediatric Oncology Unit of the Tygerberg Hospital has treated HD with three different regimens. Use of the CLVPP and MOPP/ABVD regimens resulted in late relapses that adversely affected event free survival (EFS). For the last four years HD has been treated according to the regimen suggested by Schellong with good short term survival rates.
Lymphoblastic or T-cell NHL is treated with regimens normally used for acute lymphoblastic leukaemia (e.g. BFM protocols) or modified leukaemia treatments for leukaemia-lymphoma syndromes (e.g. LSA2L2). We lately use a modified BFM regimen with a 70% EFS for all stages.
Three consecutive regimens have been used to treat B-cell NHL over the past 22 years. The first was a COMP regimen, followed by the LMB-89 and LMB-96 regimens. Although toxicity has increased with the increased intensity of the treatment regimen, EFS has improved from 25% to 87% for all B-cell NHL. The majority of patients had stage III and IV disease. Although the LMB regimens are toxic, the implementation is manageable provided good laboratory back up and supportive facilities are available.
Hereditary spherocytosis (HS) is an inherited haemolytic anaemia, characterized by spheroidal, osmotically fragile red blood cells. This disorder exhibits heterogeneity in terms of both clinical ...severity and underlying molecular defect. We have studied a South African Cape Coloured individual with severe HS owing to a band 3 deficiency caused by two mutations, occurring in trans, in the band 3 gene: a novel variant that we have designated band 3 Cape Town and a previously described mutation, band 3 Prague III. Analysis of erythrocyte membrane proteins indicated a deficiency of both band 3 and protein 4·2, as well as a decreased functional capacity of band 3 to transport anions. Band 3 Cape Town is defined by a GAG→AAG point mutation at codon 90, substituting a glutamic acid with a lysine in the cytoplasmic domain of the molecule, while band 3 Prague III is a codon 870 CGG→TGG point mutation, replacing an arginine with a tryptophan in the transmembrane region of band 3. mRNA is transcribed from both mutant alleles, implying that mutant proteins are synthesized, but are either degraded prior to membrane incorporation or insertion is impaired. We conclude that the combination of these two mutations exacerbated the clinical presentation of the proband.
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