Laryngeal leiomyosarcoma (LLMS) is an exceptionally rare malignant tumour, accounting for less than 1 % of all laryngeal cancers, which are predominantly epithelial in origin.

A new case report on the topic has now been published in Oncotarget titled “Laryngeal leiomyosarcoma: A rare case report and literature review.”

The study was led by first author Bolat Shalabaev and corresponding author Zhuldyz Kuanysh, both from the National Research Oncology Centre, Astana, Kazakhstan.

In this report, the authors describe a rare case of high-grade laryngeal leiomyosarcoma (LLMS) in a 64-year-old man who presented with progressive dyspnoea and hoarseness caused by a large supraglottic mass.

Laryngeal leiomyosarcoma is an exceptionally uncommon malignant tumour of smooth muscle origin, with fewer than 70 cases reported worldwide since it was first described in 1939.

Because most laryngeal malignancies are epithelial tumours such as squamous cell carcinoma, diagnosis of LLMS can be particularly challenging and requires extensive histopathological and immunohistochemical evaluation.

Imaging studies revealed a heterogeneous laryngeal tumour causing near-complete obstruction of the airway.

Histopathological analysis demonstrated high-grade spindle-cell proliferation with marked pleomorphism and pathological mitoses.

Immunohistochemical testing showed strong expression of smooth muscle actin (SMA) and vimentin, while markers including CD34, myogenin, cytokeratins 5/6 and 7, and p40 were negative, supporting the diagnosis of high-grade pleomorphic leiomyosarcoma.

The patient underwent extended laryngectomy with left neck dissection and formation of a permanent tracheostomy.

Comprehensive staging with CT, MRI, and ultrasound showed no evidence of regional or distant metastases.

Due to the tumour’s aggressive pathological features—including a Ki-67 proliferation index reaching 60%—the multidisciplinary tumour board recommended adjuvant chemotherapy with doxorubicin and ifosfamide following surgery.

“Complete surgical excision remains the cornerstone of therapy, while multidisciplinary-guided adjuvant treatment may benefit selected high-grade or high-risk patients.”

Postoperative pathology confirmed a high-grade pleomorphic leiomyosarcoma classified as pT3N0M0 according to the AJCC 8th edition staging system.

Importantly, surgical margins were negative, and no metastatic involvement was identified in the five examined lymph nodes.

At the most recent follow-up, 12 months after surgery and completion of chemotherapy, the patient remained alive and free of recurrence or metastasis.

The authors also reviewed recently published LLMS cases reported between 2021 and 2024.

Their analysis confirmed persistent male predominance, frequent involvement of the glottic and supraglottic regions, and highly variable clinical outcomes ranging from long-term disease-free survival to rapid metastatic progression.

The report further highlights the central role of immunohistochemistry in differentiating leiomyosarcoma from other spindle-cell neoplasms of the head and neck.

Importantly, the study emphasises that complete surgical resection with histologically negative margins remains the most important factor associated with favourable outcomes.

While the role of chemotherapy in laryngeal leiomyosarcoma remains controversial, the authors note that individualised multidisciplinary treatment approaches may be particularly valuable in patients with high-grade or high-risk disease features.

Overall, this report contributes important clinical insight into one of the rarest malignancies of the larynx.

As the first documented case of laryngeal leiomyosarcoma reported from Central Asia, the study expands the limited global literature on this disease and underscores the importance of coordinated multidisciplinary care, detailed pathological evaluation, and long-term surveillance in optimising patient outcomes.

Source: Impact Journals LLC