ID #1024 Multicentre, case series on Paediatric Pineal Parenchymal Tumours of Intermediate Differentiation (PPTID)
Jay Sharma, Karen Tsui, Marty Campbell, Dong Anh Khuong Quang, Nick Gottardo, Jason Dyke, Santosh ValviAbstract
Introduction
Pineal parenchymal tumours of intermediate differentiation (PPTID) are extremely rare brain tumours that arise from pinealocytes, the parenchymal cells of the pineal gland. Pineal parenchymal tumours include pineocytomas (WHO grade I), PPTIDs (WHO grade II-III), and pineoblastomas (WHO grade IV), representing a spectrum from benign to highly malignant tumours. In children PPTIDs are exceedingly rare, the optimal treatment strategy remains undefined and paediatric management is largely based on adult experience.
Methods
We conducted a retrospective, multicentre case series analysis across multiple tertiary paediatric neuro-oncology centres in Australia and New Zealand (NZ). The primary objective was to retrospectively analyse outcomes of paediatric patients with PPTID treated across Australia and NZ. Secondary objectives were to compare treatment approaches and survival outcomes with historical paediatric cohorts and with an adult PPTID cohort reported from a tertiary neuro-oncology centre. Children and adolescents aged <18 years diagnosed with PPTID between 2010 and 2024 were eligible. Histopathological confirmation of PPTID (WHO 2021 criteria) was required for inclusion. Clinical, radiological, pathological, treatment, and outcome data were extracted from institutional medical records, and neuro-oncology databases using a standardised data collection sheet.
Results
We identified 4 children diagnosed with PPTID across 11 paediatric oncology centres in Australia and NZ over a period of 20 years. Patients with PPTID were older than 10 years with headache being the most common presenting symptom. Recurrent alterations in KBTBD4 were the differentiating features compared to pineoblastomas. All patients were treated with multimodality therapy including surgery, radiation therapy and chemotherapy and are alive at 1-15 years since diagnosis. Analysis of the adult cohort and outcome comparison with the paediatric patient is ongoing.
Conclusions
PPTIDs are rare paediatric brain tumours with good survival with multimodality therapy. A larger, multicentre analysis may inform the optimal therapy for this disease.