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EP-001. CENTRAL NERVOUS SYSTEM TUMORS IN CHINESE CHILDREN UNDER THE AGE OF THREE: A POPULATION STUDY BY THE HONG KONG PAEDIATRIC HAEMATOLOGY/ ONCOLOGY STUDY GROUP Anthony Pak-Yin Liu1, Matthew Ming-Kong Shing2, Hui-Leung Yuen3, Chak-Ho Li4, Siu-Cheung Ling5, Chung-Wing Luk3, Shau-Yin Ha1, Chi-Kong Li2, and Godfrey Chi-Fung Chan1; Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong; Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, Hong Kong; Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, Hong Kong; Department of Paediatics and Adolescent Medicine, Tuen Mun Hospital, Hong Kong, Hong Kong; Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong, Hong Kong

Background Children with Down syndrome (DS) are at higher risk of developing acute leukemia. Treatment continues to evolve as we accumulate better understanding of the distinctive clinical and biological features of acute leukemia in DS patients. Procedure A retrospective review of the clinical features, treatment outcomes, and survival of DS children with acute leukemia in Hong Kong from 1993 to 2013 was conducted. Patients were identified from the registry of the Hong Kong Pediatric Hematology and Oncology study group. Results This cohort included a total of 29 patients with DS. Ten were diagnosed with acute lymphoblastic leukemia and 19 had acute myeloid leukemia (AML). The mean follow-up duration was 8.3 years (range, 0.6 mo to 18.1 y). The 5-year overall survival and event-free survival for DS-acute lymphoblastic leukemia and DS-AML were 65.6%, 54.9%, 89.5%, and 89.5%, respectively. Conclusions The clinical characteristics and treatment outcomes of DS patients with acute leukemia in Hong Kong were comparable with results from other international study groups. Patients with DS-AML had a better prognosis.

OBJECTIVES To review the clinical features, pathology and survivals of infants with brain tumours. METHODS A retrospective review of the clinical findings, pathology, treatment and survival outcome in infants with brain tumours. RESULTS From 1999 to 2018, there were 507 children ( CONCLUSION Infants with brain tumours have different pathology and inferior outcome.

METHOD: This clinical trial started from January 2008 and ended in June 2014. Patients who were suspected to have germinoma received a biopsy, followed by chemotherapy with carboplatin (600 mg per m(2) on day 1) and etoposide (150 mg per m(2) per day, day 1 to 3), alternating with ifosfamide (1.8 mg per m(2) per day and etoposide 150 mg per m(2) per day, day 1 to 5). They were assessed with MRI every 2 cycles. A total of 4 cycles of chemotherapy were given, followed by radiotherapy. The dose and volume of radiotherapy depend on the response and site of involvement. Patients with non-germinomatous germ cell tumours received operation to remove the tumour (if possible), chemotherapy as above for 6 cycles, reassessed by MRI once in two cycles, and cranio-spinal radiotherapy. The patients may need a second look operation if necessary. RESULTS: The data were reviewed on 31 May 2017. There were 45 patients, male to female ratio: 2.8:1, median age: 12.5 years of age (0.4 to 18.7). The pathology were germinoma (n=24) and non-germinomatous germ cell tumours (n=21). The locations of the tumours were suprasellar/sellar(n=13), pineal gland (n=20), suprasellar & pineal gland (n=7) and basal ganglia (n=4) and left frontal lobe (n=1). Five patients had metastatic diseases at presentation. Two patient had relapses. The median follow-up time was 6.75 years (2.43 to 9.54). PFS and OS at 5 years were 95.5% and 100% respectively. CONCLUSION: With a multi-disciplinary approach, a satisfactory outcome is achieved.

METHOD: Retrospective cohort for patients age 0 to 18 years old from January 1999 to Aug 2016 diagnosed to have low grade glioma. RESULT: Fifty-five patients diagnosed with pLGG with n=50 (91%) got a tissue diagnosis all showing astrocytoma WHO grade 1. Sites included cerebellum (n=29, 53%), cerebrum (n=10, 18%), brainstem (n=7, 13%), optic pathway (n=8, 15%) and spinal (n=1, 2%). Four patients (7%) have multi-focal tumours. Thirty-three (60%) patients received surgery alone and remained alive. Chemotherapy adopted including CCNU/CDDP/VCR, VCR/CPM/CDDP/VP-16, BLE/VP-16/CDDP, PCZ/CCNU/VCR, TMZ, CBDCA/VCR. Six (11%) received focal radiotherapy. One had NF-1 with thalamic lesion treated by surgery alone. One had TSC treated with mTOR inhibitor. Seven (13%) had progressive disease after chemotherapy and six remained alive. One had refractory brainstem tumour despite varies chemotherapy. The tumour had BRAF-KIAA1549 fusion and patient achieved stable disease after MEK-inhibitor (currently only 3 months). Three patients (5%) died. The first had parietal tumour at 12 years old received CCV and focal radiotherapy 54Gy, died from disease progression 5.8 years later. The second presented with diencephalic syndrome at 8 months old, received VCR/CPM/CDDP/VP-16 with stable disease, then died from status epilepticus 1.7 years later. The third had optic pathway glioma diagnosed at 2 months old, died from pneumonia 5.6 years from diagnosis. Median follow-up was 7.2 years (0.4-18). OS at 5 years was 94.1%. CONCLUSION: pLGG had excellent prognosis. Many patients treated successfully with surgery alone. New novel therapy from advanced molecular study might provide better prognosis and spare some patients from radiotherapy.

Background and aim We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. Methods Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. Results Sixty patients were enrolled with median age at diagnosis of 1.1 years and median follow-up time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients. Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. Conclusions Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients.

A population-based multicentre study for childhood acute lymphoblastic leukemia (ALL) was conducted in Hong Kong from 1993 to 1997. One hundred and forty-five newly diagnosed ALL patients were treated by the HKALL 93 protocol. Patients were stratified into three risk groups according to age, presenting white cell count, immunophenotyping and cytogenetic study. The patients received the same induction and early and late intensification at week 5 and week 20. Fifty-eight standard risk (SR) patients received regular intrathecal methotrexate as CNS preventive therapy, while 49 intermediate risk (IR) patients received high dose intravenous methotrexate and regular intrathecal methotrexate. Thirty-eight high risk (HR) patients were treated with prophylactic cranial irradiation and an additional intensification block at week 35. The induction remission rate was 97.2% with 2% induction death. Two patients died during first complete remission. Relapse occurred in 20.7, 42.9 and 42.1% of SR, IR and HR patients respectively. By multivariate logistic regression, age≥10 years and white cell count≥100×109/l were the two significant variables accounting for mortality. The 5-year overall and event-free survival of the whole group was 81.3 and 62.6% respectively. According to risk groups, the event-free survival was 79, 49 and 61% for SR, IR and HR patients respectively, while the overall survival was 96, 73 and 68% for SR, IR and HR patients respectively. In conclusion, the treatment protocol had low treatment-related mortality but was associated with a rather high relapse rate, especially in IR patients. Salvage therapy achieved sustained second remission in some patients. More intensive treatment especially a late intensification is required to improve the outcome. Copyright © 2002 John Wiley & Sons, Ltd.