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CASE REPORT article

Front. Oncol.

Sec. Thoracic Oncology

Malignant Triton Tumor with Thoracic Region as the Initial Presentation: A Case Report

Provisionally accepted
Hang YinHang Yin1Xindi ZhangXindi Zhang2Zhan HuangZhan Huang3Xiaotian ZhangXiaotian Zhang3Qian LiQian Li3Tao HuTao Hu3Xiaoliang ShiXiaoliang Shi3Changbin ZhuChangbin Zhu3Huabing WeiHuabing Wei1*
  • 1Department of thoracic surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
  • 2Department of thoracic surgery, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China
  • 3Department of Translational Medicine, Amoy Diagnostics Co Ltd, Xiamen, China

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Abstract Background: Malignant Triton Tumor (MTT) is a rare, highly aggressive peripheral nerve sheath tumor characterized by rhabdomyoblastic differentiation, seldom occurring primarily in the thoracic cavity. This report presents a rare case of MTT initially manifesting as chest pain, and reviews relevant literature to summarize its clinical features and therapeutic strategies. Methods: A 23-year-old woman experienced chest pain for ten days and and her condition worsened within 3 days. The patient had a known history of neurofibromatosis type 1 (NF1) and exhibited classic café-au-lait spots on physical examination. Diagnosis was confirmed via chest CTA, PET-CT, bronchoscopy, ultrasound, and histopathology. After surgical resection, the tumor recurred rapidly, prompting multiple treatments including chemotherapy, targeted therapy, and combination immunotherapy. Results: Pathology after right lower lobectomy and chest wall resection confirmed MTT (S-100+/Desmin+/Myogenin+). Recurrence in the mediastinum was detected two months postoperatively. Disease stabilization was achieved using cadonilimab, apatinib, and ifosfamide/etoposide. Notably, the first application of single-cell RNA sequencing (scRNA-seq) in MTT, combined with the CopyKAT algorithm, distinguished malignant from non-malignant cells and revealed a

Keywords: case report, Immunotherapy, Malignant triton tumor, neurofibromatosis type 1, single-cell sequencing, Thoracic tumor

Received: 10 Dec 2025; Accepted: 03 Feb 2026.

Copyright: © 2026 Yin, Zhang, Huang, Zhang, Li, Hu, Shi, Zhu and Wei. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Huabing Wei

Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Supplementary Material