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Updated: Jun 4, 2025

Defining Gene Functions in Tumorigenesis by Ex vivo Ablation of Floxed Alleles in Malignant Peripheral Nerve Sheath Tumor Cells
Published on: August 25, 2021
Multicellular model of neuroblastoma proposes unconventional therapy based on multiple roles of p53
Kenneth Y Wertheim1,2,3,4, Robert Chisholm2, Paul Richmond2
1Insigneo Institute for in Silico Medicine, University of Sheffield, Sheffield, United Kingdom.
Abstract:
Neuroblastoma is the most common extra-cranial solid tumour in children. Over half of all high-risk cases are expected to succumb to the disease even after chemotherapy, surgery, and immunotherapy. Although the importance of MYCN amplification in this disease is indisputable, the mechanistic details remain enigmatic. Here, we present a multicellular model of neuroblastoma comprising a continuous automaton, discrete cell agents, and a centre-based mechanical model, as well as the simulation results we obtained with it. The continuous automaton represents the tumour microenvironment as a grid-like structure, where each voxel is associated with continuous variables such as the oxygen level therein. Each discrete cell agent is defined by several attributes, including its cell cycle position, mutations, gene expression pattern, and more with behaviours such as cell cycling and cell death being stochastically dependent on these attributes. The centre-based mechanical model represents the properties of these agents as physical objects, describing how they repel each other as soft spheres. By implementing a stochastic simulation algorithm on modern GPUs, we simulated the dynamics of over one million neuroblastoma cells over a period of months. Specifically, we set up 1200 heterogeneous tumours and tracked the MYCN-amplified clone's dynamics in each, revealed the conditions that favour its growth, and tested its responses to 5000 drug combinations. Our results are in agreement with those reported in the literature and add new insights into how the MYCN-amplified clone's reproductive advantage in a tumour, its gene expression profile, the tumour's other clones (with different mutations), and the tumour's microenvironment are inter-related. Based on the results, we formulated a hypothesis, which argues that there are two distinct populations of neuroblastoma cells in the tumour; the p53 protein is pro-survival in one and pro-apoptosis in the other. It follows that alternating between inhibiting MDM2 to restore p53 activity and inhibiting ARF to attenuate p53 activity is a promising, if unorthodox, therapeutic strategy. The multicellular model has the advantages of modularity, high resolution, and scalability, making it a potential foundation for creating digital twins of neuroblastoma patients.
Insights
We developed a novel multicellular model to simulate neuroblastoma tumor growth and drug responses. This model reveals conditions favoring MYCN-amplified tumors and suggests a new therapeutic strategy involving p53 modulation.
Area of Science:
- Computational Biology and Oncology
- Pediatric Cancer Research
- Systems Biology
Background:
- Neuroblastoma is a common pediatric cancer with poor prognosis in high-risk cases.
- MYCN amplification is critical in neuroblastoma, but its mechanistic role requires further elucidation.
- Current treatments often fail, necessitating novel therapeutic strategies.
Purpose of the Study:
- To develop and utilize a sophisticated multicellular model for simulating neuroblastoma dynamics.
- To investigate the growth conditions and drug responses of MYCN-amplified neuroblastoma clones.
- To identify potential new therapeutic strategies based on simulation insights.
Main Methods:
- A multicellular model integrating a continuous automaton for the microenvironment, discrete cell agents with stochastic behaviors, and a center-based mechanical model.
- Stochastic simulation algorithm implemented on GPUs to model over one million neuroblastoma cells.
- Simulation of 1200 heterogeneous tumors, tracking MYCN-amplified clones and testing 5000 drug combinations.
Main Results:
- Identified key factors influencing the growth advantage of MYCN-amplified clones within the tumor microenvironment.
- Revealed interdependencies between MYCN-amplified clone dynamics, gene expression, other tumor clones, and the microenvironment.
- Generated results consistent with existing literature while providing novel mechanistic insights.
Conclusions:
- Proposed a hypothesis of two distinct neuroblastoma cell populations with opposing p53 protein roles (pro-survival vs. pro-apoptosis).
- Suggested a novel therapeutic strategy involving alternating inhibition of MDM2 and ARF to modulate p53 activity.
- The developed multicellular model offers modularity, high resolution, and scalability, serving as a foundation for patient-specific digital twins.
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