Treatment modalities among long-term survivors of primary pulmonary NUT carcinoma: A systematic review and meta-analysis.

Abstract

e20782 Background: Primary pulmonary NUT carcinoma (NC) is a rare, highly aggressive malignancy with a reported median overall survival of approximately 6.8–9.2 months. There are no FDA-approved targeted therapies, no consistently effective chemotherapy regimens, and no established standard-of-care or first-line treatment. Long-term survival in this disease is exceptionally rare, and treatment characteristics associated with durable outcomes remain poorly defined. Methods: We performed a systematic review and meta-analysis of published case reports and case series describing adult patients with primary pulmonary NC who achieved long-term survival, defined as survival ≥5 years following diagnosis. A comprehensive literature search of PubMed, Scopus, Embase, and Web of Science was conducted for studies published between January 2014 and March 2025. Extracted variables included age at diagnosis, disease stage, metastatic status, surgical management, radiation therapy, systemic treatment regimens, and survival outcomes. Descriptive statistics were used to summarize treatment patterns among long-term survivors. Results: Thirty-two long-term survivors met inclusion criteria. Median age at diagnosis was 23 years (interquartile range [IQR], 18–44). All patients presented with localized, non-metastatic disease at diagnosis. Definitive surgical resection was performed in all cases, with tumors deemed operable and completely resectable. Adjuvant and/or neoadjuvant radiation therapy was administered in 21 patients (65.6%). Systemic therapy was utilized in 18 patients (56.3%), most commonly in the perioperative setting. Chemotherapy regimens were heterogeneous but predominantly included platinum-based agents (72.2%) and ifosfamide-based combinations (44.4%), reflecting variability in baseline clinical characteristics and institutional practice patterns. Two patients (6.3%) received pembrolizumab as part of systemic therapy and achieved durable long-term survival. No long-term survivor had metastatic disease at initial presentation. Conclusions: Long-term survival in primary pulmonary NUT carcinoma is rarely observed in patients with localized, non-metastatic disease managed with complete surgical resection. Younger age at diagnosis and aggressive local control appear to be common features among long-term survivors. Further research is needed to optimize multimodal treatment strategies and reinforce the necessity of a multidisciplinary approach incorporating surgery, radiation, and systemic therapy in the management of this devastating malignancy.