The high incidence of primary liver cancer in Malaysian males is not observed in childhood, where it constitutes 0.16 per 1000 paediatric hospital admissions and 3.20/0 of all childhood malignancies at the University Hospital, Kuala Lumpur. This frequency is comparable to that reported from several developed countries. The commonest liver tumour in children is the hepatoblastoma which is probably of embryonal origin and has a similar world wide "incidence. The relative infrequency of hepatocellular carcinoma in childhood and its association with cirrhosis, the hepatitis B antigen and its prevalence in the older age group helps to substantiate an acquired environmental aetiology.
A 13-year old boy with acute lymphoblastic leukemia on chemotherapy developed neutropenia and acute cellulitis progressing to fulminating septicemia due to Pseudomonas pseudomallei. Septicemic melioidosis should be considered in the differential diagnosis of a febrile illness in children who are susceptible to infections.
A review of all cases of nephroblastoma admitted to the University Hospital over a 10 year period reveals that its incidence relative to the other childhood tumours and epidemiological features are similar to other centres. The majority of patients presented with either stage III or IV disease. During the period 1968·1972 the number of defaulters was high and survival was poor. Following the introduction of treatment protocol, default rate has fallen and 5 of 7 patients have survived more than 2 years. Earlier referral and education of the parents should help improve the outcome for children with Wilms' tumour in Malaysia.
A report is presented of seven patients with acute septicaemic melioidosis seen at the University Hospital, Kuala Lumpur, Malaysia, during 1976-1979. All had associated disorders which rendered them more susceptible to infection. As prognosis depends on early diagnosis it is important that this disease be considered in the differential diagnosis of a septicaemic illness in such patients from endemic areas. The treatment of choice is a combination of tetracyclines and chloramphenicol, initially used in massive doses, and continued for at least six month to prevent relapses.
A review of rhabdomyosarcoma in childhood reveals that the pattern and results of treatment have changed with the introduction of multimodal therapy. Outcome in our series have been poor due to advanced disease, poor compliance to follow up resulting from poor socio-economics and educational levels of our patients and their faith in traditional medicine. Improvement in the prognosis can only be anticipated with earlier diagnosis and reduction in defaulter rate.
A 13 year review at the University Hospital, Kuala Lumpur reveals that chronic myeloid leukaemia (CML) constitutes 4.3% of all childhood leukaemia. Adult type of CML occurs in older children and is associated with marked splenomegaly, leukocytosis and thrombocytosis and the presence of Philadelphia chromosome. Although the initial response to busulphan was encouraging most of the patients succumbed; 2 patients underwent acute lymphoblastic transformation. Juvenile CML occurs in younger children and is associated with less marked splenomegaly, leukocytosis and thrombocytopenia and the presence of elevated fetal haemoglobin levels. The disease is characterised by an acute fulminating course. Despite improved survival in acute lymphoblastic leukaemia, the outlook for chronic myeloid leukaemia in childhood remains poor and treatment needs re-evaluation.
A review of 20 cases of neuroblastoma at the University Hospital, Kuala Lumpur from 1967 to 1980 reveals six infants aged 2 to 13 months with stage IV·S disease, associated with an unusually good prognosis. Four of the six patients presented with hepatomegaly, one had skin nodules and another paresis of the lower limbs. The primary tumour was located infra-diaphragmatically in all cases, four had disease in the bone marrow but none had radiological evidence of bone involvement. Although not systematic, with limited treatment of low dose radiation and mild chemotherapy, four patients are alive and well, one absconded and one died of septicaemia. It is important to define this special category as an unexpectedly good survival is possible with minimal therapy. Death is more likely to result from over-zealous treatment than from the disease itself.
Campylobacter Jejuni is being increasingly recognised as a cause of bacteraemia enteritis and two infants with this condition are described. Awareness of the organism. as a possible cause of septicaemia is important because it has special growth requirements and delay in the diagnosis can be detrimental in a disease which usually only responds to erythromycin, gentamicin and chloramphenicol.
Malignant lymphoma constitutes the third most common childhood malignancy seen at the University Hospital, Kuala Lumpur and can be categorised into Hodgkin's disease and non-Hodgkin's lymphoma. Both diseases demonstrate a higher preference for Chinese males. The majority of patients presented with stage IV disease. High default rate and poor compliance to treatment were associated with poor overall cure rates but encouraging results have been obtained in those who adhered to therapy. There is an obvious need to educate the public on the improved outlook 'or childhood malignancies and 'or earlier referral to help reduce the higher mortality and morbidity associated with advanced disease.
One hundred four children with acute lymphoblastic leukaemia were diagnosed at the University Hospital, Kuala Lumpur, Malaysia, between 1976 and 1982; 87 were evaluable with respect to treatment. They were divided into good prognosis (GP) and bad prognosis (BP) groups based on their initial total white cell count, their treatment differing only during the maintenance phase. Remission was achieved in 82 patients (94%) of whom ten (12%) subsequently died in remission from infection. Twenty-eight (34%) relapsed while on treatment and three while off therapy. Eleven patients ceased treatment after 3 yr of continuous complete remission (CCR). Three of these later relapsed, two within the first year. Survival in CCR was significantly better in the GP group up to 30 months, after which the difference diminished. There was no difference in survival between boys and girls. The overall disease-free survival at 3 yr and 5 yr was 40% and 25%, respectively, with a median follow-up period of 20 months (range 4-69 months). The reasons for the relatively low survival rates as compared with those in developed countries are discussed.
From 1967-82, 9 children with testicular relapse (TR) of acute lymphoblastic leukaemia (ALL) were diagnosed out of 99 boys treated, an incidence of 9.1%. The median time from the onset of ALL until diagnosis was 28 months (range 3-41 months). All were asymptomatic; six were detected on routine examination while three were diagnosed only on biopsy. Routine biopsy prior to stopping chemotherapy is useful in detecting occult TR. Biopsies should be done on both the testes regardless of the clinical findings. The age, leucocyte count and hepatosplenomegaly at diagnosis of ALL were not found to be significant factors in influencing relapse. Eight children were in bone marrow remission at the time of TR, but three had preceding or concurrent meningeal leukaemia while in the other five the testis was the first and only site of relapse. Radiotherapy was effective in local disease control but failed to prevent bone marrow relapse in all except two patients despite continuation of chemotherapy. The median time from onset of TR until bone marrow relapse was 7 months (range 3-13 months) and the median time until death, was 11 months (range 6-18 months). The frequency of testicular relapse may be related to the intensity of either the initial induction therapy or the consolidation chemotherapy. Further studies are required to determine whether the incidence of testicular relapse will decline with more intensive early treatment.
The simultaneous expression of both lymphoid and myeloid phenotypic features in acute leukaemia is rare. We report 3 cases of biphenotypic hybrid acute leukaemia seen in our institution. All 3 patients achieved remission with treatment for acute lymphoblastic leukaemia but two subsequently relapsed while on treatment. The hybrid acute leukaemias are important areas for further research both for delineation of basic biology and choice of optimal treatment.
In the 7-year period between 1980 and 1987, six cases of childhood primary hepatocellular carcinoma (PHC) were confirmed histologically in our institution. Hepatitis B surface antigen (HBsAg) seropositivity was confirmed in five of the cases, and tissue HBsAg was shown in four of these using the Shikata's orcein stain. An associated maternal HBsAg seropositivity was shown in two of the seropositive children. The youngest seropositive patient who developed PHC was 7 years old. The mother of this patient was also seropositive. These observations support a causal relation between childhood Hepatitis B virus infection and PHC. The importance of vertical or perinatal transmission of HBV in the causation of childhood PHC and the prophylactic role of childhood vaccination is emphasized. Attention is also drawn to the relative short malignant transformation time seen in some of these patients.
A case of primary hepatocellular carcinoma (PHC) developing in a 10 year old boy who contracted Hepatitis B virus (HBV) infection in the course of maintenance phase chemotherapy for acute lymphoblastic leukemia was seen at University Hospital, Kuala Lumpur. This case is of interest in that it (1) supports an etiological relationship between HBV infection and PHC, (2) manifested a distinctly short malignant transformation time, and (3) draws attention to the possible contributory role of chemotherapy in increasing the risk of developing PHC.
Clear cell sarcoma of kidney (CCSK) is a rare but distinct tumor of childhood frequently confused with Wilms' tumor (nephroblastoma). It has a characteristic histology, a marked predilection for metastasis to bone, and an aggressive clinical course with a high relapse rate in spite of surgical excision, chemotherapy and radiotherapy. We report the first histologically proven CCSK in a Malaysian patient. This was an 8-mth-old Malay boy who was clinically diagnosed to have stage I Wilms' tumor. Despite treatment, he developed multiple metastases 10 mths after initial presentation and died soon after. Emphasis is placed on recognizing this entity in view of (1) its naturally aggressive behaviour and (2) the prospect of improving prognosis with currently recommended intensified chemotherapeutic regimes. Its immunohistochemical profile of vimentin-positivity and negativity for epithelial membrane antigen, cytokeratin and Factor-8 related antigen is more in favour of a mesenchymal or glomerular origin than a tubular or vascular origin.
The haematological findings and case history of 3 patients with the association of acute myeloid leukemia and translocation involving the long arm of chromosome no. 11 are presented. The recipient chromosome for the translocated material from chromosome 11 differs in all the three cases being namely chromosomes 1, 10 and 17.