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G253(P) An audit of adherence to UK Children’s Cancer & Leukaemia Group guidelines for the management of Low and Intermediate risk Neuroblastoma, 2011–2013
  1. F Herd1,
  2. K Wheeler2,
  3. D Tweddle1,3
  1. 1Paediatric Oncology Unit, Great North Childrens Hospital,Newcastle-Upon-Tyne, UK
  2. 2Oxford Childrens Hospital,Oxford University Hospitals,Oxford, UK
  3. 3Northern Institute Cancer Research,Newcastle University, Newcastle-Upon-Tyne, UK

Abstract

Aims To evaluate how children with low and intermediate risk Neuroblastoma in the UK are managed and compare this with the recommendations in the national CCLG guidelines (V1 August 2011) which are based on the current European SIOPEN low & intermediate risk neuroblastoma trial.

Methods Centre neuroblastoma leads at UK CCLG centres were asked if they had followed guidelines for the management of low & intermediate risk Neuroblastoma between 1/8/11 and 31/7/2013. Details collected included number of patients seen, results of genetic testing including MYCN and the presence of segmental chromosomal abnormalities (SCA), and outcome details for particular sub groups.

Results 15 of 21 CCLG centres have replied to date describing 40 cases (25 low risk and 15 intermediate). In principal all centres adhered to the guidelines. Only 2 patients were not biopsied due to other co-morbidity. All biopsied tumours were tested for MYCN amplification and SCA either locally or via the central reference facility in Newcastle.

9/24 low risk tumours had SCA, 17q gain was the commonest. 4 patients had stage 4s disease without life threatening symptoms, 2 with SCA and all 4 received 4–6 cycles of chemotherapy.

5 patients were <18 months of age with localised unresectable (L2) tumours and no SCAs, 4 had chemotherapy followed by surgery in 2 cases, and one surgery alone. 4 patients are alive, and one unexpectedly died from disease progression.

9/15 intermediate risk tumours had SCA, the commonest was 17q gain & 1p loss. 6 patients were >18 months of age with L2, undifferentiated tumours, 5 with SCA. All 6 patients received chemotherapy, 3 surgery, 4 radiotherapy and 2 retinoic acid. One child is still on treatment, one progressed and died and the remainder are in first remission.

Conclusions This study shows that all CCLG centres who replied are broadly following the guidelines, but an in depth analysis of specific subgroups shows adherence is variable. The reasons for this are now being explored. It is important to capture this data accurately as currently in the UK the European trial is not open for this group of patients who account for 50% of all neuroblastoma cases.

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