Abstract Neurofibromatosis type 1 (NF1) is a common genetic disorder that predisposes
affected individuals to tumours. The NF1 gene encodes a RAS GTPase-activating protein …

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that
comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients …

Wnts and R-spondins (RSPOs) support intestinal homeostasis by regulating crypt cell
proliferation and differentiation. Ex vivo, Wnts secreted by Paneth cells in organoids can …

Learning Objectives Explain the characteristics and treatment of malignant peripheral nerve
sheath tumors, both in relation to neurofibromatosis type I and otherwise. Cite the unique …

Sarcomas are complex mesenchymal neoplasms with a poor prognosis. Their clinical
management is highly challenging due to their heterogeneity and insensitivity to current …

Aberrant activation of the Wnt/β-catenin pathway is frequently found in various cancers, often
through mutations of downstream components. Inhibiting β-catenin signaling in tumors with …

Cardiac glycosides (CGs) are a diverse family of naturally derived compounds having a
steroid and glycone moiety in their structures. CG molecules inhibit the α-subunit of …

Abstract Polycomb repressive complex 2 (PRC2) has oncogenic and tumor-suppressive
roles in cancer. There is clinical success of targeting this complex in PRC2-dependent …

Deregulation of the β-catenin signaling has long been associated with cancer. Intracellular
components of this pathway, including axin, APC, and β-catenin, are frequently mutated in a …

Abstract Neurofibromatosis type 1 (NF1) is an autosomal dominant tumour predisposition
syndrome that is caused through loss of function mutations of a tumour suppressor gene …