Adamantiomatious Craniopharyngioma (ACP) is a benign intracranial tumor that is thought to arise from embryonic remnants of Rathke’s pouch. Though histologically benign, the clinical manifestation of the disease is profound due to the location of the tumor in the sellar/parasellar region around critical brain structures as the hypothalamus and the pituitary gland. Our understanding of the disease pathology is limited and currently, there are no targeted drug therapeutics for the disease. The lack of reliable cell line models for ACP has limited our ability to utilize standard in vitro techniques to study disease mechanism and drug interactions. Some attempts have been made in the isolation and culture of ACP primary cell lines. However, due to the heterogeneous nature of the tumor and low proliferation rate in ACP tumor cells, the resultant culture often leads to overgrowth and confounding by non-ACP tumor cells such as the stromal fibroblasts.
In this study, we propose to characterize and validate the new ACP tumor cell lines developed by the CBTN. After the robustness of the cell line is satisfactorily validated, we will then utilize the in vitro model to conduct experiments to dissect the unique mechanism of ACP pathology.
What are the goals of this project?
The goal of this project is to characterize and validate the cell line model for use as reliable in vitro model of ACP.
What is the impact of this project?
Experimental studies and drug interaction studies for ACP has been very limited due to the lack of reliable cell line models. This project to systematically characterize and validate the ACP cell line will allow us to establish a reliable in vitro model for ACP. Once established, this model can be utilized to great extent in the laboratory to explore disease specific mechanisms and to study the effect of various targeted drugs.
Why is the CBTN request important to this project?
The cell lines requested from CBTN are very important for this project because of the lack of such comparable cell lines from another source. Additionally, the access to matched samples and curated database of clinical, histological, and molecular information associated with these cell lines that is available from the CBTN is very critical for the success of this project
The Children's Brain Tumor Network is contributing to this project by providing cell lines.
Todd Hankinson, MD
My research interests focus in 3 areas:1. Pediatric Brain Tumors - I assembled and lead the only multi center consortium in North America that focuses on the improvement of therapies for pediatric craniopharyngioma: Advancing Treatment for Pediatric Craniopharyngioma. We use tissue and clinical data
Children’s Hospital of Philadelphia
Children’s Hospital of PhiladelphiaJoined on
Operations Center for the Children’s Brain Tumor Tissue Consortium, the Children’s Hospital of Philadelphia (CHOP) is currently ranked 1st nationally for their Pediatric Cancer Program by U.S. News & World Report. CHOP’s Biobank is home to the CBTTC’s pediatric brain and CNS tumor biorepository; the
Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).Craniopharyngiomas are usually part solid mass and