{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T06:48:50Z","timestamp":1774594130028,"version":"3.50.1"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T00:00:00Z","timestamp":1774569600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A 23-year-old woman experienced chest pain for ten days and her condition worsened within 3 days. The patient had a known history of neurofibromatosis type 1 (NF1) and exhibited classic caf\u00e9-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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>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 heterogeneous tumor microenvironment composed of distinct functional cell populations, highlighting the tumor\u2019s high degree of heterogeneity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case underscores the importance of considering MTT in young NF1 patients presenting with intrathoracic masses. An individualized, multimodal treatment approach may extend survival. scRNA-seq provides valuable insights into the molecular landscape of MTT and hold promise for guiding precision therapy in the future.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2026.1764543","type":"journal-article","created":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T05:19:46Z","timestamp":1774588786000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Malignant triton tumor with thoracic region as the initial presentation: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Hang","family":"Yin","sequence":"first","affiliation":[{"name":"Department of Thoracic Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University","place":["Shanghai, 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