Anti-angiogenic therapy for advanced primary pulmonary lymphoepithelioma-like carcinoma: a retrospective multicenter study

Abstract

Purpose Primary pulmonary lympho-epithelioma-like carcinoma (PPLELC) is a rare subtype of primary non-small cell lung cancer (NSCLC). Currently, there is still lack of research data on anti-angiogenic therapy of advanced PPLELC. The purpose of this study was to investigate the efficacy and safety of anti-angiogenic therapy combined with chemotherapy compared with traditional chemotherapy for these patients. Methods Advanced PPLELC patients admitted to six grade A hospitals from January 2013 to January 2021 were selected. The patients received anti-angiogenic therapy combined with chemotherapy (AT group) or chemotherapy (CT group) alone. Results A total of 65 patients were included in this study, including 31 patients in the AT group treated with anti-angiogenic therapy combined with chemotherapy and 34 patients in the CT group treated with chemotherapy alone. As of October 1, 2021, the median progression-free survival (PFS) in the AT group was 11.2 months [95% confidence interval (CI), 5.9–16.5]. The median PFS in the CT group was 7.0 months [95%CI, 5.1–8.9] [Hazard Ratio (HR), 0.49; 95%CI, 0.29–0.83; P = 0.008]. The 1-year PFS rates were 41.9% and 17.6%, respectively. The overall response rates (ORR) of two groups were 45.2% (95% CI, 0.27–0.64), 38.2% (95% CI, 0.21–0.56), (P = 0.571). The disease control rates (DCR) of two groups were 93.5% (95% CI, 0.84–1.03), 88.2% (95% CI, 0.77–1.00), (P = 0.756). Conclusion Among patients with advanced PPLELC, the PFS of patients with anti-angiogenic therapy combined with chemotherapy is better than that of patients with chemotherapy alone. Anti-angiogenic therapy combined with chemotherapy is an optional treatment scheme.

Pathogenesis and therapeutic implications of EBV-associated epithelial cancers

“…Because PPLELC is uncommon, the adoption of standardized treatments has been challenging. Existing treatment regimes are adapted from those used for stage IV NPC, which involves gemcitabine-based chemotherapy ( 45 ), taxanes ( 244 ), anti-angiogenic therapy ( 245 ), and anti-PD1/PD-L1 therapy ( 246 ). The favorable response of PPLELC to NPC treatments is evident of the therapeutic implications of molecular similarities between the two cancer types, and the substantial overlap in their molecular characteristics provides an explanation for the effective response of PPLELC to chemotherapies typically used in NPC.…”

“…In one study, small molecule multi-targeted TKI, inhibiting VEGFR, platelet derived growth factor (PDGFR), FGFR, and c-KIT was used to treat a patient with FGFR3-TACC3 fusion, and response was favorable. While studies are limited, this suggests that anti-angiogenic therapy can potentially be employed in the treatment of PPLELC ( 245 ).…”