Pineoblastoma are rare, highly malignant WHO Grade IV tumors of the pineal gland that primarily
affect young children. They are characterized by aggressive growth, frequent recurrence, and poor
survival despite intensive multimodal treatment.
Most pineoblastoma arise sporadically, but may occasionally occur in patients with inherited cancer
predisposition syndromes such as germline mutations in DICER1 and RB1 (Pfaff et al., 2021). Further,
it has been shown, that a high number of these tumors show an OTX2b up-regulation and/or a
DROSHA down-regulation. However, the genetic and molecular drivers of pineoblastoma still remain
poorly understood.
The aim of my project is to investigate the functional roles of the potentially tumor-
regulated genes DROSHA, CCNK, CD276, FZD7 und HDAC1 in the development of
pineoblastoma using our transgenic Tg(Pinealis:GAL4_5UAS:tdTom-p2A-ZfOtx2b) zebrafish model.
Additionally, I want to uncover possible therapeutic effects by in vivo specific drug testing of
Parthenolide and Valproatic acid on our otx2b over-expressing zebrafish tumor model – both these
HDAC1 targeting drugs were successfully tested in pineoblastoma cell-culture already. 

RG Odermatt — anatomy