Objective:

Laryngeal leiomyosarcoma is a rare malignant tumor of the head and neck region. This systematic review examines how patient demographics, presentation of symptoms, tumor characteristics, treatment, and survival for leiomyosarcoma of the larynx compare across the existing literature.

Methods:

Following PRISMA guidelines, this review was registered on the PROSPERO database. Search terms were optimized by a librarian specializing in health science, and articles from the following databases were pooled: PubMed, EMBASE, SCOPUS, Cochrane, and Web of Science. Duplicates were removed, and a blinded review was conducted by three reviewers using an online artificial intelligence program called Rayyan. All individuals diagnosed with primary leiomyosarcoma of the larynx from English-based literature, as well as animal studies, were included. Metastasis of leiomyosarcoma to the larynx, non-English-based literature, articles without patient data, as well as articles that were out of scope, were excluded. Patient demographics such as gender and age, co-morbidities, history of smoking and alcohol use, presenting symptoms, as well as tumor characteristics, treatment and survival data were collected. Data analysis was performed using SPSS software.

Results:

57 articles met inclusion criteria out of the 270 articles collected across databases. This yielded 61 cases of primary leiomyosarcoma of the larynx between 1987 and 2023. There were no animal studies reported. The average age at diagnosis was 60 years (SD +/- 17) with a range between 9 and 87 years. There was a 30:1 male predominance, and the most commonly reported symptom was dysphonia. There were no significant correlations between alcohol and/or tobacco use and tumor grade or survival. Treatment type, including total laryngectomy, also did not correlate significantly with survival. From available data, Pearson correlation revealed that patients who survived at least 2 years were more likely to survive at least 5 years (r =.821, N =15, P<.001). Patients who underwent repeat interventions were less likely to survive at least two years (r =-.73, N =14, P<.01), as well as five years. (r = -.63, N =14, P<.05). The cases were also grouped based on year where early cases were compared to more recent cases. Group 1 included 16 cases from 1987 to 2001 and Group 2 included cases 18 from 2018 to 2023. Recent cases, on average, presented 60 days sooner to the clinic compared to early cases. However, the follow-up periods for recent cases were shorter, which limited comparisons with earlier cases.

Conclusion:

Laryngeal leiomyosarcoma is a rare soft tissue malignancy. The type of intervention, surgical or non-surgical, did not confer a significant survival benefit. Timely identification and effective interventions for this disease process may improve survival outcomes. Thorough data reporting and longer-term survival data is recommended for future case reports.