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Learning Modern Laryngeal Surgery in a Dissection Laboratory
Published on: March 18, 2020
A Rare Case of Primary Laryngeal Synovial Sarcoma, Challenges in Diagnosis and Management in a Resource-Limited
Mikiyas Olani1, Mesele Bogale1, Suleman Essa2
1Department of Otolaryngology-Head and Neck Surgery, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.
Abstract:
Introduction: Sarcomas account for less than 1% of malignant tumors of the larynx. Synovial sarcomas account for 5%-7% of all soft tissue sarcomas and 0.1% of sarcomas in the head and neck region. Clinical Report: A 45-year-old patient presented with hoarseness of voice and breathing difficulty. Contrast-enhanced computed tomography showed a well-defined mass originating from the laryngeal surface of the epiglottis, obstructing the supraglottis, for which emergency tracheostomy was done. Pathology confirmed monophasic synovial sarcoma. He underwent a supraglottic partial laryngectomy with complete removal of the tumor. The patient is on a 6-month postoperative course, with monthly follow-up, and there is no sign of recurrence. Discussion: Clinical diagnosis can be challenging, as patients often present with ill-defined symptoms in the throat and larynx, which may delay diagnosis. The current approach relies on immunohistochemistry analysis for diagnostic purposes, and imaging is generally used to define the tumor location and extent and to rule out other tumors. Most reported studies put surgery as a first-line mode of management, and adjuvant radiotherapy is currently advocated, as it is associated with better overall survival. Synovial sarcoma is considered a high-grade tumor, although head and neck subsites tend to have a slightly better prognosis. Tumor size of more than 5 cm and higher tumor stage are associated with poor overall survival. Conclusion: Synovial sarcoma of the larynx is a rare clinical entity, and every case should be examined individually.
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