Establishment and characterization of a novel alveolar rhabdomyosarcoma cell line, NCC-aRMS1-C1

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CELL LINE

Establishment and characterization of a novel alveolar rhabdomyosarcoma cell line, NCC‑aRMS1‑C1 Yooksil Sin1 · Yuki Yoshimatsu1 · Rei Noguchi1 · Ryuto Tsuchiya1,2 · Akane Sei1 · Takuya Ono1 · Shunichi Toki3 · Eisuke Kobayashi3 · Ayumu Arakawa4 · Masanaka Sugiyama4 · Akihiko Yoshida5 · Akira Kawai3 · Tadashi Kondo1  Received: 6 July 2020 / Accepted: 17 July 2020 © Japan Human Cell Society 2020

Abstract Alveolar rhabdomyosarcoma (aRMS) is a histological subtype of RMS, which is the most common pediatric and adolescent soft-tissue sarcoma, accounting for 3–4% of all pediatric malignancies. Patient-derived cells are essential tools for understanding the molecular mechanisms of poor prognosis and developing novel anti-cancer drugs. However, only a limited number of well-characterized cell lines for rhabdomyosarcoma from public cell banks is available. Therefore, we aimed to establish a novel cell line of aRMS from the tumor tissue of a patient with aRMS. The cell line was established from surgically resected tumor tissue from a 4-year-old male patient diagnosed with stage III, T2bN1M0 aRMS and was named as NCC-aRMS1-C1. The cells were maintained for more than 3 months under tissue culture conditions and passaged more than 20 times. We confirmed the presence of identical fusion gene such as PAX7-FOXO1 in both the original tumor and NCC-aRMS1-C1. The cells exhibited spheroid formation and invasion. We found that docetaxel, vincristine, ifosfamide, dacarbazine, and romidepsin showed remarkable growth-suppressive effects on the NCC-aRMS1-C1 cells. In conclusion, the NCC-aRMS1-C1 cell line exhibited characteristics that may correspond to the lymph node metastasis in aRMS and mirror its less aggressive features. Thus, it may be useful for innovative seeds for novel therapeutic strategies. Keywords  Alveolar rhabdomyosarcoma · Primary culture cells · Cell line · Chemotherapy · Drug response

Introduction

Electronic supplementary material  The online version of this article (https​://doi.org/10.1007/s1357​7-020-00403​-0) contains supplementary material, which is available to authorized users. * Tadashi Kondo [email protected] 1



Division of Rare Cancer Research, National Cancer Center Research Institute, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan

2



Department of Orthopaedic Surgery, Graduate School of Medicine, Chiba University, 1‑8‑1, Inohana, Chuo‑ku, Chiba 260‑8670, Japan

3

Department of Musculoskeletal Oncology, National Cancer Center Hospital, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan

4

Department of Pediatric Oncology, National Cancer Center Hospital, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan

5

Department of Diagnosis Pathology, National Cancer Center Hospital, 5‑1‑1 Tsukiji, Chuo‑ku, Tokyo 104‑0045, Japan





Alveolar rhabdomyosarcoma (aRMS) is a histological subtype of RMS, which is the most common pediatric soft-tissue sarcoma, accounting for 3–4% of all pediatric malignancies [1]. RMS exhibits skeletal muscle phenotypes and diverse histology, with four histological subtypes, na