Establishment and characterization of NCC-PS2-C1: a novel cell line of high-grade pleomorphic spindle cell sarcoma, most consistent with myxofibrosarcoma.

Abstract

Pleomorphic sarcoma (PS) is a heterogeneous group of malignant mesenchymal tumors that lack specific histological differentiation. PS is characterized by genetic instability and diversity and unique histological features such as pronounced morphologic pleomorphism. PS is one of the most common soft tissue sarcomas. Complete surgical resection remains the only curative treatment and is often combined with neoadjuvant radiotherapy. Effective systemic chemotherapy is yet to be established, and PS frequently recurs locally and metastasizes to the lungs. Patient-derived cancer cell lines are invaluable tools for basic and preclinical research for developing novel chemotherapies. Herein, we report a high-grade pleomorphic spindle cell sarcoma, most consistent with myxofibrosarcoma cell line, NCC-PS2-C1, which was derived from a primary tumor specimen. NCC-PS2-C1 cells exhibited a range of copy number alterations. This cell line demonstrated consistent proliferation, spheroid formation, and invasive capabilities in vitro. Drug screening using NCC-PS2-C1 cells revealed that cobimetinib, crenolanib, and ixazomib were effective against PS. In conclusion, we established NCC-PS2-C1 cells from primary tumors of PS. This cell line is a valuable resource for developing novel chemotherapies.