A novel patient-derived immortalised cell line of myxofibrosarcoma: a tool for preclinical drugs testing and the generation of near-patient models

一种新型患者来源的粘液纤维肉瘤永生化细胞系:一种用于临床前药物测试和近患者模型生成的工具

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作者:Ania Naila Guerrieri #, Chiara Bellotti #, Marianna Penzo, Marta Columbaro, Micaela Pannella, Alessandro De Vita, Marco Gambarotti, Laura Mercatali, Roberta Laranga, Barbara Dozza, Silvia Vanni, Serena Corsini, Tommaso Frisoni, Giacomo Miserocchi, Toni Ibrahim, Enrico Lucarelli

Background

Myxofibrosarcoma is a rare malignant soft tissue sarcoma characterised by multiple local recurrence and can become of higher grade with each recurrence. Consequently, myxofibrosarcoma represents a burden for patients, a challenge for clinicians, and an interesting disease to study tumour progression. Currently, few myxofibrosarcoma preclinical models are available.

Conclusions

In conclusion, we established a novel patient-derived myxofibrosarcoma cell line that, together with the few others available, could serve as an important model for studying the molecular pathogenesis of myxofibrosarcoma and for testing new drugs and therapeutic strategies in diverse experimental settings.

Methods

In this paper, we present a spontaneously immortalised myxofibrosarcoma patient-derived cell line (MF-R 3). We performed phenotypic characterization through multiple biological assays and analyses: proliferation, clonogenic potential, anchorage-independent growth and colony formation, migration, invasion, AgNOR staining, and ultrastructural evaluation.

Results

MF-R 3 cells match morphologic and phenotypic characteristics of the original tumour as 2D cultures, 3D aggregates, and on the chorioallantoic membrane of chick embryos. Overall results show a clear neoplastic potential of this cell line. Finally, we tested MF-R 3 sensitivity to anthracyclines in 2D and 3D conditions finding a good response to these drugs. Conclusions: In

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