Establishment and characterization of NCC-GCTB10-C1: a novel cell line derived from a patient with recurrent giant cell tumor of bone.

Abstract

Giant cell tumor of bone (GCTB) is a rare osteolytic tumor composed of mononuclear stromal cells, macrophages, and osteoclast-like giant cells. While generally benign, GCTB has a high risk of local recurrence and can occasionally undergo malignant transformation or metastasis, posing significant clinical challenges. The primary treatment is complete surgical resection; however, effective management strategies for recurrent or advanced GCTB remain elusive, underscoring the need for further preclinical research. This study reports the establishment of a novel cell line, NCC-GCTB10-C1, derived from a recurrent GCTB lesion. NCC-GCTB10-C1 retains the characteristic H3-3A G34W mutation, which is central to the tumor's pathogenesis, and demonstrates significant growth potential, spheroid formation capability, and invasive properties. Extensive drug screening of NCC-GCTB10-C1, along with nine previously established GCTB cell lines, revealed a distinct drug response profile, with the cell line showing resistance to many previously effective agents. However, doxorubicin, foretinib, and ceritinib were identified as promising therapeutic candidates due to their low IC₅₀ values in NCC-GCTB10-C1. The establishment of NCC-GCTB10-C1 offers a critical resource for further research into GCTB, especially in the context of recurrent disease, and holds potential for the development of more effective treatment strategies.