Pulmonary carcinoma with rhabdoid features: cases report and literature review

ABSTRACT Background Non–small-cell lung cancers (NSCLC) with rhabdoid features are rare, accounting for <1% of lung malignancies. They are characterized by aggressive clinical behavior and poor outcomes. Most reported cases predate the immunotherapy era and lack PD-L1 evaluation or molecular profiling, leaving the role of immune checkpoint inhibitors (ICIs) unknown. Methods We performed a retrospective, single-institution review of patients with histologically confirmed NSCLC with rhabdoid features. Clinicopathologic and molecular data were extracted from institutional records. A literature search was conducted. Results Two patients with metastatic NSCLC with rhabdoid features were identified. Patient 1, a 53-year-old man with KRAS G12D mutation and PD-L1 expression over 90%, achieved a complete response on pembrolizumab monotherapy, sustained for 7 years. Patient 2, a 64-year-old man with KRAS G12C mutation and a PD-L1 status of 100%, attained a complete response with carboplatin–paclitaxel–pembrolizumab, with ongoing remission at 3.5 years. The literature review identified 44 previously published cases, resulting in 46 total. Most patients presented with advanced disease and experienced poor survival: 11 died ≤3 months, 8 survived 4–12 months, and 5 survived 1–3 years; only 3 survived >3 years. No prior patients received ICIs. Conclusion These cases represent the first durable complete responses to ICIs in NSCLC with rhabdoid features, suggesting a potential role for immunotherapy—particularly in tumors with high PD-L1 expression and KRAS mutations. Further molecular characterization is warranted.