The implementation of a national paediatric oncology protocol for neuroblastoma in South Africa.

PURPOSE: The aim of the World Health Organization-International Paediatric Oncology Society is to improve childhood cancer survival in low- and middle-income countries to 60% by 2030. This can be achieved using standardised evidence-based national treatment protocols for common childhood cancers. The aim of the study was to describe the development and implementation of the SACCSG NB-2017 neuroblastoma (NB) treatment protocol as part of the treatment harmonisation process of the South African Children's Cancer Study Group. METHODS: The Consolidated Framework for Implementation Research was used to identify factors that could influence the implementation of the national NB protocol as a health care intervention. The evaluation was done according to five interactive domains for implementation: intervention characteristics, inner setting, outer setting, individual or team characteristics and the implementation process. RESULTS: The protocol was developed over 26 months by 26 physicians involved in childhood cancer management. The process included an organisational phase, a resource identification phase, a development phase and a research ethics approval phase. Challenges included nationalised inertia, variable research ethical approval procedures with delays and uncoordinated clinical trial implementation. CONCLUSION: The implementation of the national NB protocol demonstrated the complexity of the implementation of a national childhood cancer treatment protocol. However, standardised paediatric cancer treatment protocols based on local expertise and resources in limited settings are feasible.

Age at diagnosis as a prognostic factor in South African children with neuroblastoma.

PURPOSE: Low- and middle-income countries (LMICs) reported a higher median age at diagnosis of neuroblastoma (NB) compared to high-income countries. The aim was to determine if the optimal age at diagnosis, which maximizes the difference in overall survival between younger versus older patients in the South African population was similar to the internationally validated 18 months age cut-point. METHODS: Four hundred sixty NB patients diagnosed between 2000 and 2016 were included. Receiver operating characteristic (ROC) curves were used to predict potential age cut-point values for overall survival in all risk group classifications. Risk ratios, sensitivity, specificity, and positive and negative predictive values at the specific cut-points were estimated with 95% confidence intervals, and time to mortality by age at the specific cut-points was shown with Kaplan-Meier curves and compared using log-rank tests. RESULTS: The median age at diagnosis for the total cohort was 31.9 months (range 0.2-204.7). For high-risk (HR), intermediate-risk, low-risk, and very low-risk patients, the median age at diagnosis was, respectively, 36 months (range 0.4-204.7), 16.8 months (range 0.7-145.1), 14.2 months (range 2.0-143.5), and 8.7 months (range 0.2-75.6). The ROC curves for the total NB cohort (area under the curve [AUC] 0.696; P < .001) and HR (AUC 0.682; P < .001) were analyzed further. The optimal cut-point value for the total cohort was at 19.1 months (sensitivity 59%; specificity 78%). The HR cohort had potential cut-point values identified at 18.4 months age at diagnosis (sensitivity 45%; specificity 87%) and 31.1 months (sensitivity 67%; specificity 62%). The 19.1 months cut-point value in the total cohort and the 18.4 months cut-point value in HR were as useful in predicting overall survival as 18 months age at diagnosis. CONCLUSION: The 18 months cut-point value appears to be the appropriate age for prognostic determination, despite the higher median age at diagnosis in South Africa.

The importance of local control management in high-risk neuroblastoma in South Africa.

PURPOSE: To investigate the impact of local therapies on high-risk neuroblastoma (HR-NB) outcomes in South Africa. METHODS: Data from 295 patients with HR-NB from nine pediatric oncology units between 2000 and 2014 were analysed. All patients received chemotherapy. Five-year overall (OS) and event free survival (EFS) were determined for patients who had received local therapy, either surgery or radiotherapy or both. RESULTS: Surgery was performed in only 35.9% (n = 106/295) patients. Surgical excision was done for 34.8% (n = 85/244) of abdominal primaries, 50.0% (n = 11/22) of thoracic primaries; 22.2% (n = 2/9) neck primaries and 66.7% (n = 8/12) of the paraspinal primaries. Only 15.9% (n = 47/295) of all patients received radiotherapy. Children, who had surgery, had an improved five-year OS of 32.1% versus 5.9% without surgery (p < 0.001). Completely resected disease had a five-year OS of 30.5%, incomplete resections 31.4% versus no surgery 6.0% (p < 0.001). Radiated patients had a five-year OS of 21.3% versus 14.2% without radiotherapy (p < 0.001). Patients who received radiotherapy without surgical interventions, had a marginally better five-year OS of 12.5% as opposed to 5.4% (p < 0.001). Patients who underwent surgery had a longer mean overall survival of 60.9 months, while patients, who were irradiated, had a longer mean overall survival of 7.9 months (p < 0.001). On multivariate analysis, complete metastatic remission (p < 0.001), surgical status (p = 0.027), and radiotherapy status (p = 0.040) were significant predictive factors in abdominal primaries. CONCLUSION: Surgery and radiotherapy significantly improve outcomes regardless of the primary tumor site, emphasizing the importance of local control in neuroblastoma.

The evaluation of induction chemotherapy regimens for high-risk neuroblastoma in South African children.

Achieving remission after induction therapy in high-risk neuroblastoma (HR-NB) is of significant prognostic importance. This study investigated remission after induction-chemotherapy using three standard neuroblastoma protocols in the South African (SA) setting. Retrospective data of 261 patients with HR-NB diagnosed between January 2000 and December 2016, who completed induction chemotherapy with standard treatment protocols were evaluated. The treatment protocols were either OPEC/OJEC or the St Jude NB84 protocol (NB84) or rapid COJEC (rCOJEC). The postinduction metastatic complete remission (mCR) rate, 2-year overall survival (OS) and 2-year event free survival (EFS) were determined as comparative denominators. The majority (48.3%; n = 126) received OPEC/OJEC, while 70 patients received (26.8%) rCOJEC and 65 (24.9%) NB84. Treatment with NB84 had the best mCR rate (36.9%), followed by OPEC/OJEC (32.5%) and rCOJEC (21.4%). The 2-year OS of treatment with NB84 was 41% compared to OPEC/OJEC (35%) and rCOJEC (24%) (p = 0.010). The 2-year EFS of treatment with NB84 was 37% compared to OPEC/OJEC (35%) and rCOJEC (18%) (p = 0.008). OPEC/OJEC had the least treatment-related deaths (1.6%) compared to rCOJEC (7.1%) and NB84 (7.5%) (p = 0.037). On multivariate analysis LDH (p = 0.023), ferritin (p = 0.002) and INSS stage (p = 0.006) were identified as significant prognostic factors for OS. The induction chemotherapy was not significant for OS (p = 0.18), but significant for EFS (p = 0.08) Treatment with NB84 achieved better mCR, OS and EFS, while OPEC/OJEC had the least treatment-related deaths. In resource-constrained settings, OPEC/OJEC is advised as induction chemotherapy in HR-NB due to less toxicity as reflected in less treatment-related deaths.

Overall survival for neuroblastoma in South Africa between 2000 and 2014.

BACKGROUND: Outcome data for neuroblastoma in sub-Saharan Africa are minimal, whereas poor outcome is reported in low- and middle-income countries. A multi-institutional retrospective study across South Africa was undertaken to determine outcome. METHODS: Patients treated between January 2000 and December 2014 in nine South African pediatric oncology units were included. Kaplan-Meier curves and Cox regression models were employed to determine two-year survival rates and to identify prognostic factors. RESULTS: Data from 390 patients were analyzed. The median age was 39.9 months (range, 0-201 months). The majority presented with stage 4 disease (70%). The main chemotherapy regimens were OPEC/OJEC (44.8%), St Jude NB84 protocol (28.96%), and Rapid COJEC (22.17%). Only 44.4% had surgery across all risk groups, whereas only 16.5% of high-risk patients received radiotherapy. The two-year overall survival (OS) for the whole cohort was 37.6%: 94.1%, 81.6%, and 66.7%, respectively, for the very-low-risk, low-risk, and intermediate-risk groups and 27.6% for the high-risk group (P < 0.001, 95% CI). The median survival time for the whole group was 13 months (mean, 41.9 months; range, 0.1-209 months). MYCN-nonamplified patients had a superior two-year OS of 51.3% in comparison with MYCN-amplified patients at 37.3% (P = 0.002, 95% CI). CONCLUSIONS: Limited disease had an OS comparable with high-income countries, but advanced disease had a poor OS. South Africa should focus on early diagnosis and implementation of a national protocol with equitable access to treatment.

Nutritional status of children with Wilms' tumour on admission to a South African hospital and its influence on outcome.

BACKGROUND: In developing countries up to 77% of children with cancer have been shown to be malnourished on admission. High rates of malnutrition occur due to factors such as poverty and advanced disease. Weight can be an inaccurate parameter for nutritional assessment of children with solid tumours as it is influenced by tumour mass. This study aimed to assess the prevalence of malnutrition amongst children with Wilms tumour (WT), the level of nutritional support received on admission and the influence of nutritional status on outcome. METHODS: Seventy-six children diagnosed with WT and admitted to Inkosi Albert Luthuli Central Hospital between 2004 and 2012 were studied prospectively. Nutritional assessment was conducted using weight, height, mid-upper arm circumference (MUAC) and triceps skinfold thickness (TSFT) prior to initiating treatment. Outcome was determined 2 years after admission. Time until commencement of nutritional resuscitation and nature, thereof, were recorded. RESULTS: Stunting and wasting was evident in 12% and 15% of patients, respectively. The prevalence of malnutrition was 66% when MUAC, TSFT and albumin were used. Malnutrition was not a predictor of poor outcome and did not predict advanced disease. The majority of patients (84%) received nutritional resuscitation within 2 weeks of admission. CONCLUSIONS: When classifying nutritional status in children with WT, the utilisation of weight and height in isolation can lead to an underestimation of the prevalence of malnutrition. Nutritional assessment of children with WT should also include MUAC and TSFT. Early aggressive nutritional resuscitation is recommended.

Age is not a prognostic factor in children with Wilms tumor beyond stage I in Africa.

BACKGROUND: Patients under age 4 with stage I favorable histology (FH) Wilms tumor have a reported survival advantage. Among children above 10 years, a poorer prognosis has been associated with a higher prevalence of diffuse anaplasia. PURPOSE: To determine if, in our practice, patients with Wilms tumors >8 years of age (stage II-V) have a poorer prognosis than those aged <8 years or <4 years. PROCEDURE: Case-control study of 19 patients >8 years with Wilms tumor stages II-V who were identified from a cohort of 192 new patients (2002-2012). For each patient two controls were chosen matched for stage and histology, one 0-3 years and one 4-7 years. Neo-adjuvant chemotherapy was offered to all, combined with intensive supportive care. Postoperative treatment was determined by local stage and histology. OS and EFS at 5 years for the different age groups were compared. RESULTS: Each age group contained 19 patients, of whom 6 had stage II tumors, 3 stage III, 8 stage IV, and 2 stage V. Histology was intermediate risk (IR) in 17 and high risk (HR) in 2. OS at 5 years was 80.8% and EFS was 79.2% for the whole group. No significant difference in outcome could be shown between age groups. Loss to follow up was 6/57 (11%). CONCLUSIONS: The survival advantage of young age (<4 years) associated with stage I FH could not be demonstrated in higher stages. Age had no significant impact on prognosis although a trend to better outcome was seen in children <4 years.

Bilateral Wilms' tumour in a developing country: a descriptive study.

PURPOSE: To present our experience of 20 children with bilateral Wilms' tumour seen in a resource-challenged environment over a 10-year period. METHOD: All patients with a diagnosis of bilateral synchronous Wilms' tumour were identified and recruited. RESULTS: Study patients represented 11 % of a cohort of 177 new patients with Wilms' tumour seen over the same period. Three patients had a syndromic predisposition to Wilms' tumour. Metastatic disease was seen at presentation in four patients (20 %) and three children presented with unilateral tumour rupture. One patient presented with paraplegia and one with obstruction of the duodenum. All children received neoadjuvant chemotherapy. One HIV-infected child died of IRIS after neoadjuvant treatment, but before surgery. One child died of progressive disease after unilateral nephrectomy. Nephron-sparing surgery was performed in 22 kidneys and 15 kidneys were removed in toto. Following enucleation of tumours, three children had positive margins. Discordant histopathology was seen in 53 % of patients. Overall survival at 2 years is 85 %. CONCLUSION: Despite significant co-morbidity and advanced disease, bilateral Wilms' tumour is a treatable disease in a resource-constrained environment.

The results of treatment of children with metastatic Wilms tumours (WT) in an African setting: do liver metastases have a negative impact on survival?

BACKGROUND: From Africa, where socio-economic circumstances differ from the developed world, there are no data regarding the influence of liver metastases on survival of children with Wilms tumour. PROCEDURE: One hundred fifty new patients with WT were seen between 2002 and 2010, 45 (30%) had metastases at diagnosis. Seven patients had bilateral disease with additional visceral metastases. Nine patients who developed liver metastases during treatment were excluded. The site of metastases and the results of pretreatment biopsies were retrieved. Neo-adjuvant chemotherapy was combined with nutritional resuscitation, and aggressive supportive care. Post-operative treatment was determined by stage and histology. RESULTS: Liver metastases were present in 19 (42%) patients but were the sole metastatic site in only 4 (9%). Overall survival at 5 years was 58.5%. Event Free Survival was 54%. Thirty-three (73%) had favourable histology, nine unfavourable and undetermined in three. No influence of histology on outcome was evident. Three patients had resection of persistent liver metastases. The pattern of metastatic disease had no influence on outcome. Despite aggressive supportive care two patients (4%) died within a week of presentation. Two patients died of chemotoxicity and two of complications following biopsy. Eight patients (17%) were lost to follow-up of whom five were on palliative treatment only. CONCLUSIONS: In Africa liver metastases do not appear to worsen the prognosis of children with Stage IV WT. Despite the poor socio-economic circumstances survival is comparable to other countries.

Pediatric non-Wilms' renal tumors: a third world experience.

BACKGROUND: Pediatric non-Wilms' renal tumors (NWRT) are poorly understood owing to their heterogeneity and relative rarity. This study aimed at auditing the outcome of the management of NWRT in a tertiary hospital in the Third World. METHODS: Records of all patients (n = 68) treated for NWRT over a 32-year period (1978-2010) were reviewed retrospectively. RESULTS: The major histological groups included clear cell sarcoma of the kidney (CCSK) (33.8%), mesoblastic nephroma (17.6%), cystic partially differentiated nephroblastoma (CPDN) (17.6%), intrarenal neuroblastoma (8.8%), malignant rhabdoid tumor (MRT) (7.4%), and renal cell carcinoma (RCC) (5.9%). Sixteen (69.7%) patients with CCSK and 11 (91.7%) with CPDN were aged 1-4 years. Ten (83.3%) patients with mesoblastic nephroma were aged <1 year and three (60.0%) with RCC were aged 10-14 years. Ten (43.5%) patients with CCSK and four (80.0%) with RCC had metastases at diagnosis. The sensitivity of a pretreatment Tru-Cut biopsy was 100% for MRT. All the patients with CCSK, mesoblastic nephroma, CPDN, and RCC had radical nephrectomy. Only eight (34.8%) patients with CCSK received radiotherapy. The overall 1-10-year survival rates were 52.2%, 91.7%, 75.0%, 40.0% and 0.0% for CCSK, mesoblastic nephroma, CPDN, RCC, and MRT, respectively. The overall 1-10-year survival for the entire cohort was 51.5%. CONCLUSIONS: The demography and clinical presentation of pediatric NWRT, which comprises 13.6% of pediatric renal tumors in the Third World, were similar to those in the Developed World. The overall 1-10-year survival for pediatric NWRT was low.

Dermal Epstein Barr virus--associated leiomyosarcoma: tocsin of acquired immunodeficiency syndrome in two children.

Although rare in childhood, a relatively high incidence of smooth muscle tumors are recognized in patients with AIDS, mainly in association with Epstein Barr virus (EBV) infection. Although EBV-associated smooth muscle tumors have been documented rarely in the subcutis of AIDS patients, dermal involvement has not been described to date. This report describes dermal EBV-associated leiomyosarcomas (EBV-LMS) with a nodular but superficial plaque-like appearance on the lower limbs of 2 males, 9 and 12 years old. Histopathological assessment of the excised lesions demonstrated hypercellular mitotically active dermal tumors with hyperchromatic spindle and round cells, arranged in short fascicles and sheets, with microfoci of necrosis. A smooth muscle immunophenotype, including prominent desmin immunopositivity, and positive EBV-encoded RNA in situ hybridization investigation confirmed a diagnosis of EBV-LMS. Subsequent HIV seropositivity and AIDS were confirmed in both patients. Both patients also had pulmonary tuberculosis and received antituberculous therapy. Patient 1 had a 3 cm re-excision of the prior tumor site. He received highly active antiretroviral therapy, completed 6 months of antituberculous therapy, achieved immune reconstitution and viral suppression and is tumor-free 2 years after tumor excision. Patient 2 died before further therapy. The immune status, presence, and appropriate therapy of co-existent systemic infection and highly active antiretroviral therapy in AIDS patients with EBV-LMS are crucial to a favorable outcome.

e-Education in paediatric surgery: a role for recorded seminars in areas of low bandwidth in sub-Saharan Africa.

BACKGROUND: There is a shortage of surgeons in Africa, and this shortage is particularly acute in paediatric surgery with most paediatric patients being cared for by general surgeons. The use of information technology to augment teaching in paediatric surgery in Africa is appealing but often unsuccessful due to the costs involved and a lack of bandwidth. A simple solution is needed to allow sharing of teaching sessions that are normally conducted by videoconference in areas in which bandwidth is adequate. METHOD: Weekly paediatric surgical interactive seminars that are regularly shared by videoconference by three centres in South Africa have been recorded onto DVDs. These have been bundled into monthly packages and couriered to four medical schools in East and Central Africa. Recipients have been at liberty to use them as they saw fit. After 6 months, a survey was conducted to determine the usefulness of the exercise. RESULTS: At all recipient sites, the seminars were used as part of the teaching of general surgical trainees and paediatric surgical fellows at those medical schools with appropriate training programmes. Three of the four schools used the seminars in undergraduate courses. All regarded the seminars as useful and all but one as of adequate visual and sound quality. CONCLUSION: The in-house teaching at the medical school in Durban is now shared by over 140 surgical trainees and students in four countries in which a lack of bandwidth precludes videoconferencing, and this low-tech low-cost solution has proved effective in resource-poor settings.

Intestinal inflammatory myofibroblastic tumour.

BACKGROUND: Inflammatory myofibroblastic tumours (IMFTs) are rare tumours characterised by nosologic, histogenetic and aetiopathogenetic controversy and variable clinicopathological features. We report our experience with intestinal-IMFTs (I-IMFTs) that have been reported mainly as single case reports to date. METHODS: Five patients with I-IMFTs, identified between 2005 and 2008, formed the study cohort. The clinicopathological features were obtained from departmental and hospital records. RESULTS: The median patient age was 13 years. While 4 patients presented with symptoms and signs of intestinal obstruction, one IMFT was an incidental finding at laparotomy for trauma. Three I-IMFTs were located in the small bowel and 2 in the colon. Complete resection with end-to-end anastomoses was performed. The gross morphology included 1 polypoid myxoid tumour that served as a lead point for an intussusception, 3 multinodular whorled masses and 1 firm circumferential, infiltrative tumour. Microscopically, all tumours had typical features of IMFT with variable expression of ALK-1, a low proliferation index and tumour-free resection margins. All patients had an uneventful recovery. One patient was lost to further follow-up. Four patients were well, without local recurrence or metastases at 6 months to 3 years. CONCLUSIONS: Surgery with tumour-free resection margins is the gold standard of care of adult and paediatric I-IMFTs. Heightened recognition of I-IMFT, albeit rare, as a cause of intestinal obstruction, including intussusception, is necessary for pre-operative suspicion of I-IMFT.

Retrospective surveillance of intussusception in South Africa, 1998-2003.

BACKGROUND: Intussusception is a common gastrointestinal emergency in children and appears to have a somewhat different clinical spectrum in developing countries. Its etiology is still unclear, but a link to infective agents and viruses has been highlighted. This study aimed to assess the clinical spectrum and prevalence of intussusception in children from the diverse South African population. METHODS: Retrospective data were obtained from 9 participating pediatric referral units on the occurrence of intussusception in South African children (<14 years old) during a 6-year period (1998-2003). Results were correlated with national population statistics. Intussusception was anatomically classified into ileoileal, ileocolic, and colocolic types. The clinical features, management, outcome, and possible causes were examined. RESULTS: We reviewed the occurrence and clinical spectrum of intussusception in 423 children (age, 0-14 years) presenting with acute intussusception to 9 pediatric surgical centers. The mean duration of symptoms was 1.5 days, but a delayed presentation was common (median delay, 2.3 days). Intussusception occurred throughout the year, with a peak in the summer months. The majority of patients (89%) were <2 years old, and 78% presented at age 3-18 months of age. Crude population estimates indicate an occurrence of 1 case per 3123 population <2 years old. Only 11% of patients presented after 2 years of age, and the age at presentation was significantly lower (P < .05) in black African patients. All ethnic groups were affected. In 84% of patients, intussusception occurred at the ileocolic region junction, in 7% it was ileoileal, and in 9% it was colocolic. Colocolic intussusception appeared more common in black African patients, and associated pathologic conditions (polyps and Burkitt's lymphoma) occurred mainly in older children. Surgical intervention was required in 81% of patients and involved resection of gangrenous bowel in 40%. CONCLUSION: Intussusception appears to be a relatively frequent occurrence in children in South Africa. Although the clinical spectrum appears to vary, there is an apparent link to intestinal infection, which requires further investigation. A collaborative approach is required to ascertain the relationship of intussusception to preventable infections and to improve its diagnosis and management.

Spinal cord compression in children with Wilms' tumour.

PURPOSE: To document the clinical, imaging and histopathological features of five children with paraplegia due to Wilms' tumour (WT), highlighting therapeutic options and patient outcome in a developing country. METHODS: Patients with WT and paraplegia seen at the Department of Paediatric Surgery since 1984 form the study cohort. Patient demographics, duration of neurological symptoms, stage of primary tumour, therapeutic intervention and outcome were recorded. Histology of the primary tumours and paraspinal or epidural biopsies were reviewed. RESULTS: Five patients with WT and paraplegia were identified. Imaging showed epidural masses with paraspinal disease, cord displacement and compression. Four patients have died. Of the two patients with neurological recovery, one relapsed 4 months later. Histology revealed triphasic WT with one case showing anaplasia. Paraspinal or epidural biopsies confirmed WT with post-treatment changes. Three biopsies showed lymphovascular, perineurial and intraneural tumour invasion and one showed epidural venous invasion. CONCLUSION: Although rare, WT-associated spinal disease may cause permanent neurological deficit, adding considerably to the burden of disease. In developing countries where patients present late, the prognosis is poor, however surgery may provide immediate relief of compression symptoms and biopsy material. The treatment of choice will depend on the facilities available and the clinical circumstances.

Clear cell sarcoma of the kidney in children: experience in a developing country.

INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is a rare tumour comprising 4% of primary renal tumours in children. It has a unique constellation of chromosomal and molecular features and should no longer be viewed as an unfavourable histological variant of Wilms tumour. Little is known of its clinical presentation and pathological profile in children living in a developing country. AIM: To describe the clinical and pathological features of CCSK in children in our practice and to identify factors contributing to poor patient outcomes. METHOD: A retrospective review of patients with a confirmed diagnosis of CCSK who presented for treatment at a single institution between 1990 and 2008. RESULTS: 14 patients fulfilled the inception criteria. They represented 4% of 356 patients presenting with primary renal tumours during the review period. Clinical and radiological features were indistinguishable from Wilms tumour. Tumours were large (Mean mass 1.4 kg; median 0.9 kg) and metastases were common (42%). Lung and lymph node metastases were more common than skeletal disease. Co-morbidity, particularly hypertension (64%) was common. Initial diagnosis by needle biopsy was correct in only two of seven patients (29%) leading to inappropriate neoadjuvant chemotherapy. Overall survival is poor with 57% of patients alive and disease free from 1 to 7 years off treatment. CONCLUSION: In a developing country, CCSK is rare and clinically and radiologically indistinguishable from Wilms tumour. Associated hypertension is common. Pretreatment diagnosis is difficult and sampling errors using needle biopsies may be unavoidable. Treatment results are poor and, given the propensity for late recurrence in CCSK, may not be sustained.

The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country.

PURPOSE: To describe the management of cavoatrial extension of nephroblastoma in a developing country. PATIENTS AND METHODS: Of 406 consecutive children with Wilms tumour, 40 (10%) presented with tumour extension into the inferior vena cava (IVC) (30), right atrium or right ventricle and form the inception cohort. Of this group, 17 (43%) had visceral metastases and two had bilateral synchronous tumours. RESULTS: Neoadjuvant chemotherapy appropriate to the pre-operative stage caused reduction in intravascular tumour in 18 (45%) patients, allowing 3 children who had atrial tumour at presentation to be surgically managed without recourse to cardiopulmonary bypass. Neoadjuvant chemotherapy also provided a window of opportunity to assess and manage co-morbidity especially malnutrition. Of the inception cohort, 31 (78%) underwent surgical resection; 23 had simple cavotomy, one caval resection and seven resection under cardiopulmonary bypass. There was one post-operative death. 77% of resected specimen contained viable malignant cells despite one or more cycles of neoadjuvant chemotherapy. Retrograde extension into caval tributaries threatened the completeness of resection in all patients necessitating the addition of post-operative radiotherapy. Of nine patients who did not undergo resection, five died pre-operatively. Three of these children died of chemotherapy induced neutropaenic sepsis. Four patients refused surgical treatment. Twenty-three patients completed the post-operative treatment protocol (1,203 characters).

Paediatric intussusception caused by acquired immunodeficiency syndrome-associated Kaposi sarcoma.

PURPOSE: To document the clinicopathological features of paediatric intussusception caused by acquired immunodeficiency syndrome (AIDS)-associated Kaposi sarcoma (KS). METHODS: Clinicopathological features of six patients with AIDS-KS-associated intussusception were obtained retrospectively from departmental and hospital records. RESULTS: Six debilitated male children, without cutaneous KS, were presented with abdominal pain and vomiting for >1 week. Intussusception was the sentinel of HIV infection in five patients. One patient had been on HAART for 13 months. Three patients each had ileal and ileocolic intussusceptions; two had recurrent intussusception. Bowel resection was performed because of failed reduction, infarction and polypoid lead points in all patients, in addition to perforation and peritonitis in three. Five patients died, the immediate cause being massive hematochezia from anorectal KS and/or septic shock. One patient, who received post-surgical chemotherapy and HAART, is currently in remission. Pathologic examination confirmed intussusception due to KS. CONCLUSION: AIDS-KS-associated intussusception occurred without cutaneous KS. Resection of the infarcted segment may relieve the presenting obstruction, but recurrent intussusception may occur because every elevated KS is a potential lead point. AIDS-KS-I is rare but fatal in children, unless timely surgical intervention, optimal histopathological diagnosis, and appropriate medical management, including HAART and chemotherapy, are facilitated.

Gastroschisis: a third world perspective.

PURPOSE: The incidence of gastroschisis is rising worldwide. In developed countries advances in neonatal intensive care (NICU) and the availability of total parenteral nutrition have improved survival for such patients, but in the third world mortality rates remain high. The aim of this study was to evaluate the impact of modern intensive care facilities on the mortality of babies with gastroschisis in Africa. METHODS: A retrospective review of all neonates admitted with a diagnosis of gastroschisis at Inkosi Albert Luthuli Central Hospital in Durban over a 6-year period (2002-2007) was conducted. RESULTS: A total of 106 babies with gastroschisis presented during the review period. The prevalence of gastroschisis amongst neonatal surgical admissions increased from 6% in 2003 to 15% in 2007. 72% of patients weighed less than 2.5 kg at birth and 64% were premature (<37 weeks gestation). 91% were "outborn" with 71% delivered vaginally. Median maternal age was 22.6 years and 57% of mothers were primiparous. Primary abdominal wall closure was possible in 74% of patients. The overall mortality was 43% with sepsis being the leading cause. Staged closure was associated with a higher mortality than primary closure. CONCLUSION: The prevalence of gastroschisis amongst neonatal surgical admissions appears to be increasing. Most babies were "outborn" resulting in delays in diagnosis and referral for surgical management. Despite the availability of NICU and total parenteral nutrition the mortality remains high. Reduction in mortality will depend upon improvements in antenatal diagnosis, primary care and transportation, as well as a reduction in postsurgical sepsis.

Malignant solid tumour, HIV infection and tuberculosis in children: an unholy triad.

INTRODUCTION: Co-morbidities introduce confounding variables into investigational protocols and complicate both diagnosis and management of children with malignant solid tumours. Such patients who are coincidentally HIV infected and who also have pulmonary or abdominal tuberculosis pose a particular challenge. AIM: The purpose of this report is to describe the diagnostic and management difficulties encountered in a small cadre of 18 HIV-infected children with solid tumours presenting to the Department of Paediatric Surgery. METHOD: A retrospective descriptive study of HIV-infected children with malignant solid tumours. RESULTS: 18 HIV-infected children were identified with a variety of primary tumours. 11 children had confirmed pulmonary or abdominal tuberculosis and in 4 the diagnosis was suspected. Neoadjuvant chemotherapy was used when possible to provide a window of opportunity to investigate co-morbidity and improve health status, inter alia, improving the patients' nutritional status. FDG-PET scanning proved unreliable in discriminating between malignant and inflammatory pathology. Overall survival was 33%. CONCLUSION: The coincidence of malignancy, HIV infection and tuberculosis carries a high mortality independent of the primary tumour type.