Hepatoblastoma(HB) is the most common pediatric malignant liver tumour. However, none of the present prognostic indicators are targetable or clinically actionable.
This study is to assess the relationship between anti-TERT expression in hepatoblastoma tumour tissue and recurrence.
Retrospective single centre pilot study of patients treated for hepatoblastoma from January 2010 to December 2018. Patients of HB with preserved tumour specimen were included. Demographic, clinical details, disease characteristics were recorded. Slides were prepared from blocks and stained with anti TERT antibody.
Thirteen patients were included. Mean age of presentation was 26.1months (5-90 months). Staging was PRETEXT-I in 1/13 (7.7%), PRETEXT-II in 8/13 (61.5%), PRETEXT-III in 4/13 (30.8%) and no PRETEXT-IV patients. High Risk disease was observed in 5/13 (38.5%) patients, among them 3/5 had metastatic disease at presentation. One patient had inoperable disease after chemotherapy while remaining twelve patients had undergone resection with clear margins The histological types were embryonal and mixed embryonal/foetal subtype in seven patients, pure fetal in one patient and mixed epithelial and mesenchymal in five. Mean follow up duration was 33.4 months(12-60 months). The 5-year overall survival was 59.3%. Three patients developed recurrence.
Nuclear staining by TERT was positive in four patients. Of this three developed recurrence and expired. Strong correlation (p<0.01) between nuclear staining and recurrence was observed. No significant association between nuclear staining or recurrence with other parameters.
Although complete resection could be achieved in majority of patients with hepatoblastoma, recurrence was seen in 23% with poor outcome. Anti-TERT could be used to identify such patients who are likely to have cancer recurrence requiring aggressive chemoadjuvant therapy, close follow up or in the future, telomerase- targeted therapy. Further studies are needed to validate it.