A case report of vocal cord leiomyosarcoma

Abstract

Background: Head and neck soft tissue sarcomas are rare and leiomyosarcoma accounts for only 4%^[1]. There have only been 50 cases of glottic leiomyosarcomas reported in scientific literature to date. Immunohistochemistry is pivotal in establishing diagnosis. Surgery remains mainstay of treatment for glottic leiomyosarcomas. We report a case of glottic leiomyosarcoma. Case Description: A 53-year-old gentleman with no comorbidities, presented in January 2017 with progressive hoarseness of voice over six months. There was no dysphagia, odynophagia, dyspnoea, cough, or constitutional symptoms. Clinical examination of the neck was unremarkable. Flexible scope showed a pedunculated mass at subglottic, anterior commissure, and left vocal cord. Subsequent direct laryngoscopy revealed a mass arising from anterior two-thirds of left vocal cord extending to anterior commissure and subglottic area. Excisional biopsy on 13/1/2017 reported histopathological finding of leiomyosarcoma. Immunohistochemistry showed positivity for SMA, vimentin and EMA. Computed tomography on 28/2/2017 revealed a glottic soft tissue lesion measuring 0.8 × 0.8 cm with neither involvement of the surrounding structures nor distant metastasis. Patient subsequently underwent total laryngectomy. Intraoperatively, a mass was seen at the anterior commissure (left > right) extending 5 mm below vocal cord with levels II, III right cervical lymphadenopathy. The histopathological report revealed a firm, whitish 1.3 × 1.0 × 1.0 cm leiomyosarcoma, FNCLCC Grade 2. Resected lymph nodes were negative and resection margins were clear (closest margin 3cm). Conclusion: Due to rarity of cases, survival statistics and treatment options in vocal cord leiomyosarcoma are not well established. Treatment principles are extrapolated from sarcomas of other sites, with extirpative surgery with wide margins is preferred, whenever feasible. Adjuvant radiotherapy may be recommended for high grade tumours, especially those with close/involved margins for better local control. The role of adjuvant chemotherapy remains uncertain but may be considered in fit patients with high risk of recurrence. More case studies are required to consolidate the management of this rare malignancy.