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1469 20 Years Case Review on Hepatoblastoma in Northern Ireland
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  1. VK Sundarajan1,
  2. A McCarthy2
  1. 1Specialty Registrar in Paediatrics
  2. 2Paediatric Oncologist, Childrens Haematology/Oncology Unit, Royal Belfast Hospital for Sick Children, Belfast, UK

Abstract

Aims To look at the incidence, management and outcome of Hepatoblastoma over 20 years period in Northern Ireland.

Methods A retrospective audit conducted in December 2011. Data collected from medical notes of all children diagnosed with Hepatoblastoma from Jan 1991 to Nov 2011 (14 patients), using an Audit proforma sheet.

Results Age at presentation: < 2 years-11 patients (79%), 2 to 15 years-3 patients (21%).

AFP level: High in all patients ranging from 12,589 to 3,140,000 KU/L.

Platelet count: Abnormally high-8 patients (57%), Normal-6 patients (43%). LFT: Deranged-11 patients (79%), Normal-3 patients(21%).

Clotting screen: Normal-11 patients (79%), Deranged-2 patients (15%), Not performed-1 patient (7%).

Staging: Pretext II-7 patients (51%), Pretext III-3 patients (21%), Pretext IV-3 patients (21%), Awaited-1 patient (7%).

Diagnosis: Non Metastatic Hepatoblastoma-Standard risk 8 patients (58%) and High risk 3 patients (21%),Metastatic Hepatoblastoma 3 patients (21%).

Pre-op chemotherapy: SIOPEL one-1 patient (7%),SIOPEL three-8 patients (57%), SIOPEL four-5 patients (36%).

Chemotherapy treatment: On time-11 patients (79%), Delayed-3 patients (21%).

Timing of surgery from initial diagnosis: 10 patients (70%) 2–4 months, 2 patients (15%) 4–5 months, 2 patients (15%) awaiting.

Type of Surgery: Orthotopic liver transplant-3 patients (21%), Partial hepatectomy-9 patients (64%), Awaiting-2 patients (15%).

Associated syndrome: Present (Beckwith-Wiedemann syndrome, Fanconi syndrome and 2 parents had familial polyposis coli)-4 patients (30%), Absent-10 patients (70%).

Post-op chemotherapy: SIOPEL one-1 patient (7%), SIOPEL three-7 patients (50%), SIOPEL four-3 patients (21%), Not applicable-1 patient (7%), Awaited-2 patients (15%).

Complications of chemotherapy: Yes (Hearing loss)-3 patients (21%), Nil-11 patients (79%).

Outcome: Remission-10 patients (70%), Died-2 patients (15%), Undergoing treatment-2 patients (15%).

Conclusions Incidence Overall 0.02 per 100,000 versus 2 per 100,000 worldwide.

Platelet count: Abnormally high in 57% patients.

Survival rate: Overall 83.5% (10/12) versus 70% worldwide.

https://doi.org/10.1136/archdischild-2012-302724.1469

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