WCLC 2025 - Posters & ePosters-EP.14.31 Incidence Trends and Survival Outcomes of Pulmonary Langerhans Cell Histiocytosis: A National Cancer Study Using SEER

EP.14.31 Incidence Trends and Survival Outcomes of Pulmonary Langerhans Cell Histiocytosis: A National Cancer Study Using SEER

Back to course

Pdf Summary

This national cancer study utilized SEER Research Plus data from 2010 to 2021 to analyze incidence trends and survival outcomes of pulmonary Langerhans cell histiocytosis (PLCH), a rare smoking-related neoplasm characterized by clonal proliferation of dendritic cells often driven by BRAF V600E mutations. The cohort included 253 patients (57% female), predominantly White (73%), with the majority at localized stage (73%). <br /><br />Key findings showed an overall age-adjusted incidence rate (IR) of 0.0132 per 100,000, peaking in 2016 before declining. Females exhibited a 33% higher incidence than males. The highest incidence occurred in the 55–59 age group and in nonmetropolitan counties, indicating demographic and geographic disparities. Black patients had a slightly higher incidence than White patients, though not statistically significant.<br /><br />Survival outcomes revealed an excellent cancer-specific survival (CSS) with 5-year CSS at 96.3% and 10-year CSS at 93.4%, highlighting the low mortality directly attributable to PLCH. However, overall survival (OS) was moderate, with 5-year OS at 84.4% and 10-year OS at 60.1%, reflecting the impact of comorbidities or late diagnosis. Age over 60 years significantly worsened OS, with a median survival of 81 months and hazard ratio of 4.19.<br /><br />The study emphasizes the importance of early detection and smoking cessation, given that PLCH can stabilize or regress following smoking cessation, distinguishing it from other lung neoplasms. It also underscores the need for further research into molecular pathways and targeted therapies, particularly BRAF inhibitors, to improve management. In conclusion, while PLCH is rare with favorable cancer-specific prognosis, moderate overall survival points to the relevance of comorbidity management and timely intervention.

Asset Subtitle

Janani Arunachalam

Meta Tag

Speaker Janani Arunachalam

Topic Mesothelioma, Thymoma, and Other Thoracic Tumors

Keywords

Pulmonary Langerhans cell histiocytosis

PLCH

SEER Research Plus

incidence trends

survival outcomes

BRAF V600E mutation

smoking-related neoplasm

cancer-specific survival

overall survival

targeted therapies