Preclinical NF1-PNST Models and Potential Drug Resistance Mechanisms

In this chapter, we aim to discuss the current available in vitro or in vivo models of peripheral nerve sheath tumors (PNSTs). The in vitro models are composed of two-dimensional cell cultures, including the culture of primary cells and cell lines, and three-dimensional tissue cultures, including spheroids, organoids, and induced pluripotent stem cells. The in vivo models are composed of patient-derived xenograft, genetically engineered mice, and other engineered animals. The PNSTs are further divided into benign and malignant parts to better illustrate each model establishment and pros and cons. The choice of appropriate models for preclinical studies can be particularly useful to understand the molecular and cellular mechanisms of the tumoral initiation, progression, malignancy, advanced therapies, and further therapy resistance. It will be eventually critical to improve the outcomes and survival quality in the lifespan of PNST patients.