Case Reports:

ABSTRACT

Background: Laryngeal leiomyosarcoma (LLMS) is an exceptionally rare malignant tumor, accounting for less than 1 % of all laryngeal cancers, which are predominantly epithelial in origin. Since the first description in 1939, fewer than 70 cases have been reported worldwide. In the largest pooled analysis reported a 5-year overall survival rate of 64%, with distant metastasis identified as the main adverse prognostic factor.

Case presentation: We report a 64-year-old male who presented with progressive dyspnea and hoarseness caused by a supraglottic mass. Preoperative biopsy revealed spindle-cell proliferation consistent with leiomyosarcoma (SMA +, Vimentin +, Ki-67 60 %). Comprehensive staging with CT, MRI, and ultrasound excluded regional and distant metastases. The patient underwent extended laryngectomy with R0 margins and left neck dissection (levels II–IV). Based on multidisciplinary tumor-board discussion, four cycles of adjuvant doxorubicin and ifosfamide were administered according to national sarcoma guidelines. At 12-month follow-up, the patient remains alive and free of disease.

Conclusions: This report, representing the first documented case of LLMS from Central Asia, contributes to the limited global experience with this rare tumor. Our review identified four additional LLMS cases published between 2021 and 2024, totaling five recent reports including the present case. Collectively, these demonstrate persistent male predominance, glottic and supraglottic predilection, and survival outcomes consistent with previous observations. Complete surgical excision remains the cornerstone of therapy, while multidisciplinary-guided adjuvant treatment may benefit selected high-grade or high-risk patients. Continued accumulation and molecular characterization of cases are needed to refine prognostic assessment and optimize management strategies.