Outcomes for high-risk hepatoblastoma in a resource-challenged setting.

BACKGROUND: Outcomes of high-risk hepatoblastoma have been dismal, especially in resource-challenged countries where access to chemotherapy and paediatric liver transplantation is limited for the underprivileged. This study aimed to assess the results of treatment of high-risk hepatoblastoma in a tertiary centre, including patients who had non-transplant surgical procedures in the form of extended resection. METHODS: A review of patients with high-risk hepatoblastoma treated between January 2012 and May 2018 was carried out. Perioperative data and long-term outcomes were analysed. RESULTS: Of 52 children with hepatoblastoma, 22 were considered to have high-risk hepatoblastoma (8 girls and 14 boys). The mean(s.d.) age at diagnosis was 35(20) months. Of these 22 children, five died without surgery. Of the remaining 17 who underwent surgery, six had a resection (4 right and 2 left trisectionectomies) and 11 underwent living-donor liver transplantation. Median follow-up was 48 (range 12-90) months. Thirteen of the 17 children were alive at last follow-up and four developed disseminated disease (3 had undergone liver transplantation and 1 liver resection). The overall survival rate at 1, 3 and 5 years was 77, 64 and 62 per cent for the whole cohort with high-risk hepatoblastoma. In children who had surgery, 1-, 3- and 5-year survival rates were 91, 82 and 73 per cent for transplantation and 100, 83 and 83 per cent for resection. There was no difference in survival between the two surgical groups. CONCLUSION: Excellent results in the treatment of high-risk hepatoblastoma are possible, even in resource-challenged countries.

ANTECEDENTES: Los resultados del hepatoblastoma de alto riesgo (high risk hepatoblastoma, HRH) han sido pésimos, especialmente en países con recursos limitados, donde el acceso a la quimioterapia y al trasplante hepático pediátrico es limitado para los menos privilegiados. Este estudio tuvo como objetivo evaluar los resultados del HRH en un centro de tercer nivel, incluyendo a los pacientes que se sometieron a procedimientos quirúrgicos diferentes del trasplante en forma de resecciones extendidas. MÉTODOS: Se realizó una revisión de los pacientes con HRH tratados entre enero del 2012 y mayo de 2018. Se analizaron los datos perioperatorios y los resultados a largo plazo. RESULTADOS: De 52 niños con hepatoblastomas, 22 fueron considerados HRH (8 pacientes del sexo femenino/14 del sexo masculino). La edad media al diagnóstico fue de 35 ± 20 meses. De estos 22 pacientes, cinco fallecieron sin haber sido intervenidos quirúrgicamente. De los 17 restantes que se sometieron a cirugía, en seis se realizaron resecciones (4 trisectorectomías derechas, 2 trisectorectomías izquierdas) y 11 se sometieron a un trasplante de hígado de donante vivo. La mediana de seguimiento fue de 48 meses (12-90 meses). Trece de 17 niños estaban vivos en el último seguimiento, y cuatro habían desarrollado enfermedad diseminada (3 habían sido sometidos a trasplante hepático y 1 a resección hepática). La supervivencia global a 1, 3 y 5 años fue del 77,3%, 63,6% y 62% para toda la cohorte de HRH. Entre los que se sometieron a cirugía, las supervivencias a 1, 3 y 5 años fueron del 90,9%, 81,8% y 72,7% para el trasplante y del 100%, 83,3% y 83,3% para la resección. No hubo diferencia en la supervivencia entre los dos grupos sometidos a cirugía. CONCLUSIÓN: En países con recursos limitados es posible obtener excelentes resultados en el tratamiento de HRH.

Single dose rasburicase in the management of tumor lysis syndrome in childhood acute lymphoblastic leukemia: A case series.

Tumor lysis syndrome (TLS) occurs in malignancies with high proliferative potential and tumor burden, such as lymphomas and leukemias. TLS syndrome is an oncologic emergency, requiring prompt intervention. The metabolic derangements cause acute kidney failure and may lead to cardiac arrhythmias, seizures, and death. With the advent of rasburicase, a recombinant urate oxidase, there has been a decline in the TLS-mediated renal failure and the need for dialysis. The recommended regimen and doses pose a heavy financial burden for patients in developing countries like India. With data and studies proving a similar efficacy for the reduced dose and lesser number of rasburicase, we report here a case series of seven children with acute leukemias, whose TLS was managed by a single dose of rasburicase. A retrospective analysis of case records of seven children with acute lymphoblastic leukemia and TLS, admitted to our Pediatric Oncology Unit of our Hospital between the period 2011 and 2013, was done. All our patients responded to a single dose, indicating that in appropriately monitored patients, single dose followed by as-needed dosing can be cost-saving.

Approach to metabolic syndrome in childhood cancer survivors.

The combined effects of optimized chemotherapy, surgery, radiotherapy, stem cell transplantation regimens, and improved supportive care had drastically increased the survival rate of childhood cancer. Hence, the number of adult survivors of childhood cancer is on the raise and this subset of population is gaining more attention due to the late effects of their cancer therapy. There is growing evidence that pediatric cancer survivors are at a greater risk of developing metabolic syndrome (MS) or the MS component traits than the general population. There is currently no drug therapy to treat MS as a whole disease, as it is a cluster of symptoms that present uniquely among different individuals. Given the recent recognition of MS in adult survivors of childhood cancer, there is a scarcity of long-term follow-up studies of this group. Adherence to a healthy lifestyle with both dietary and physical activity is the only most powerful and most useful armor available now against obesity and its metabolic complications.

Hepatic haemangioma of infancy: role of propranolol.

A newborn girl with a haemangioma of the liver failed to respond to cortico-steroid therapy. Ultrasonic evidence of the haemangioma disappeared after 2 months of treatment with propranolol. Propranolol, a non-selective beta-blocker, is a promising therapy in the management of haemangiomas.

Renal enlargement as primary presentation of acute lymphoblastic leukaemia.

Renal enlargement in acute lymphoblastic leukaemia is well reported in literature from Western Countries. However there are very few reports from developing countries. Bilateral symmetrical enlargement of kidneys as a primary presentation of acute lymphoblastic leukaemia is rare. We report a child who had acute lymphoblastic leukaemia presenting with bilateral renal mass.