Sequential multimodal management for recurrent pulmonary NUT carcinoma: a case report

Background Pulmonary NUT carcinoma is an ultra-rare and highly aggressive malignancy associated with extremely poor survival, especially in thoracic primary tumors. Clinically meaningful prolonged survival after postoperative locoregional recurrence without distant metastasis, particularly in a child or adolescent with primary pulmonary NUT carcinoma, has rarely been described. Here, we report a notable case managed with sequential multimodal treatment across multiple lines, resulting in 26 months of post-recurrence survival. Case description We report a 13-year-old male initially diagnosed with stage IB primary pulmonary NUT carcinoma and treated with complete surgical resection. Twenty-two months later, he developed intrathoracic recurrence without distant metastasis. He subsequently received sequential multimodal treatment, including locoregional therapy, chemotherapy plus immunotherapy, and later anti-angiogenic therapy combined with immunotherapy and salvage chemotherapy, with transient disease control achieved at multiple time points. Molecular and immunohistochemical analyses of the primary tumor confirmed NUT positivity, PD-L1 negativity, and MTAP loss. The patient ultimately died from multiple organ failure. The post-recurrence survival, defined as the interval from confirmed postoperative recurrence to death, was 26 months, exceeding historically reported survival for thoracic NUT carcinoma. Conclusion This case suggests that prolonged survival in recurrent pulmonary NUT carcinoma may be achievable through individualized multimodal management. Early complete resection, aggressive locoregional control, and the integration of systemic therapies (including immunotherapy-based combinations) may contribute to extended disease control. In our patient, immune checkpoint inhibitors combined with chemotherapy and multi-target inhibitor therapy provided periods of disease stabilization, highlighting the potential role of immunotherapy as part of a multimodal treatment strategy. Comprehensive molecular profiling, including assessment of MTAP status, may further inform future therapeutic options in this rare malignancy.