Laryngeal Clear Cell Carcinoma: A Systematic Review

Objective Laryngeal clear cell carcinoma (LCCC) is an exceedingly rare tumor. Current literature on LCCC is limited to case reports with little comprehensive data available. This systematic review aims to analyze existing literature to better characterize LCCC and to identify trends in presentation, treatment, and survival. Data Sources A literature search of PubMed, MedLine, and Embase was conducted. Review Methods A systematic review of LCCC cases from 1976 to 2024 was performed. Data extraction followed PRISMA guidelines. Included studies were those describing adult or pediatric patients pathologically diagnosed with LCCC. Excluded studies were those describing nonclear cell pathology, nonlaryngeal location, or nonprimary clear cell carcinoma. Results In total, 7 studies (n = 9 patients) were included in the analysis. Males (77.7%) were more commonly affected, with an average patient age of 56.5 years. The supraglottis was the most frequently involved subsite (66.6%). Most patients presented with advanced locoregional disease. Surgery alone was the most common treatment (66.6%), followed by chemoradiation (22.2%) and surgery with adjuvant chemoradiation (11.1%). Disease recurrence occurred in 55.5% of cases. Four patients (44.4%) died due to the disease, with an average survival of 9.3 months. Conclusion LCCC is a rare tumor often presenting as a supraglottic mass with cervical metastasis. Patients underwent various treatments with surgery, chemoradiation, or a combination of both. Reported survival was generally poor, emphasizing the aggressive nature of this disease.