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Program Number: B166
Session Name: Poster Session

Laryngeal Clear Cell Carcinoma: A Systematic Review

Gabriele Noreikaite, DO¹; Savannah Nicks, DO¹; Daniel Lofgren, DO²; Kerolos Shenouda, MD³; ¹McLaren Oakland Hospital; ²Vanderbilt University; ³Trinity Oakland Hospital

Introduction: Primary clear cell carcinoma (CCC) is an uncommon type of cancer which mainly develops within the renal system, lungs, liver, pancreas, and female reproductive organs. In the head and neck, CCC has been described as a minor salivary gland tumor that can affect the entire aerodigestive tract. Laryngeal involvement is exceedingly rare, making up only 0.6% of head and neck CCC cases. The current literature on CCC of the larynx is composed of individual observational studies with little comprehensive data available on demographics, treatment and survival. The goal of this qualitative systematic review is to examine the available literature on laryngeal clear cell carcinoma (LCCC) to better characterize the disease and determine trends in presentation, treatment, and patient outcomes. 

Methods: A systematic review of LCCC from 1976 to 2024 was conducted using the following databases: Pubmed, MedLine, Embase. The following MesH search terms were used in article identification: “clear cell carcinoma larynx” OR “laryngeal clear cell carcinoma” OR “supraglottic clear cell carcinoma” OR “supraglottis clear cell carcinoma” OR “glottic clear cell carcinoma” OR “glottis clear cell carcinoma” OR “subglottic clear cell carcinoma” OR “subglottis clear cell carcinoma” NOT “squamous”. Data was extracted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. 

Results: After review of 371 studies, seven studies were included with a total of nine patients. Males were more commonly affected than females with a total of seven (77.7%) and two (22.2%) patients, respectively. Average age in years was 56.5. The supraglottis was the most affected laryngeal subsite noted in six (66.6%) patients. Two (22.2%) patients had glottic involvement, and one (11.1%) patient had subglottic involvement. The most common stage at presentation was advanced locoregional disease with extension to the cervical lymph nodes (66.6%). Six (66.6%) patients received surgical treatment alone. Two (22.2%) patients were treated with chemoradiation and one (11.1%) patient was treated with surgery and adjuvant chemoradiation. Five patients (55.5%) had recurrence of disease after treatment. The average disease free months prior to recurrence was 6.6. Of the five patients with recurrence, two (40%) underwent salvage therapy. Regardless of salvage therapy, no patients with disease recurrence survived. Overall, four (44.4%) of the patients with LCCC died secondary to the disease. Average months survived was 9.3.

Conclusion: LCCC is a rare and aggressive minor salivary gland tumor which commonly presents as a supraglottic mass with cervical lymph node metastasis. Treatment options include surgery, chemoradiation and surgery with adjuvant chemoradiation. Patients managed surgically with partial/total laryngectomy with or without neck dissection were more likely to experience disease recurrence. Patients treated with chemoradiation alone or surgery with adjuvant chemoradiation showed lower rates of disease recurrence and greater months survived. Data is limited by the rarity of the disease process and reported cases. Nonetheless, this systematic review provides valuable information on presentation, treatment and survival of patients with LCCC in hopes of aiding otolaryngologists in the management of future LCCC cases.

 

 

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