Abstract 433P
Background
Lung adenoid cystic carcinoma (LACC) and tracheal adenoid cystic carcinoma (TACC) are considered rare thoracic malignancies with low incidence rates. LACC is more aggressive due to its higher metastatic potential compared to TACC. However, due to the rarity of these tumors, limited data exists on their clinicopathological and molecular characteristics. The current study cohort aimed to examine the clinicopathologic features and outcomes of these malignancies in a single-center study.
Methods
We retrospectively analyzed all histologically confirmed cases of LACC and TACC diagnosed at Gustave Roussy. Clinical, pathological, and molecular data were collected via REDCap database and vital status was reviewed as of December 31, 2024. Descriptive analyses, progression free survival (PFS) and overall survival (OS)were evaluated. Survival outcomes were and analyzed using the Kaplan-Meier method and compared via the log-rank test.
Results
From June 1989 to October 2024 34 patients were enrolled, n=18 (52.9%) with LACC and n=16 (47.1%) with TACC. The median age was 46 years (range 18–82),19 (56%) patients being female. At baseline, all TACC cases presented with localized disease, while n=2 (11%) of LACC cases were metastatic (pleural and lung metastases). Overall, 94% of patients underwent local treatment. After a median follow-up of 5.7 years, n=19 (56%) of patients experienced disease progression. The most common metastatic sites were the lung (24.5%) followed by bone (10.2%), liver (10.2%), brain (6,1%), lymph nodes (6,1%), pleural (4,1%), meningeal (2%) and renal (2%). The median progression-free survival (PFS) for the LACC was 88.7 months (95% CI: 20.29–157.23) and 120 months (95% CI: 13.271–226.742) for TACC (long rank test: x2=2.455, df=1, p=0.117). Median overall survival (mOS) for LACC was 140.7 months (95% CI: 46.082–231.023), while TACC OS was not reached. For two patients with metastatic LACC, median PFS was 30.35 months (immature data for OS).
Conclusions
LACC and TACC are rare thoracic malignancies typically diagnosed in young patients. Despite the aggressiveness of LACC, localized cases demonstrate a survival exceeding 10 years, emphasizing the importance of early detection and surgical intervention.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.