%0 Journal Article
%T Laryngeal Synovial Sarcoma: A Rare Clinical Entity
%A Clair Saxby
%A Ron Bova
%A Melanie Edwards
%J Case Reports in Otolaryngology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/578606
%X Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the left supraglottic larynx and an emergency tracheostomy was performed. A diagnosis of biphasic synovial sarcoma was formed. A total laryngectomy and left hemithyroidectomy was performed in conjunction with a left modified radical neck dissection. The patient received adjuvant chemotherapy followed by a course of radiotherapy and remains alive and disease free at 18£¿months after treatment. Discussion. Prognosis for patients with SS is related to primary tumour extent, grade, and size. The presence of the diagnostic translocation, t(X;18), is being targeted and hopefully will lead to the development of new therapeutics (Guadagnolo et al., 2007). Conclusion. Laryngeal SS remains a rare and poorly understood entity. A multidisciplinary approach to treatment is essential and long-term followup is imperative. 1. Introduction Sarcomas represent 1% of all head and neck malignancies [1]. Only 10% of soft tissue sarcomas are synovial in type. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells and usually involve large joints within the lower extremities [1]. Only 3% of cases arise in the head and neck [2]. The most common site is the hypopharynx with the larynx being the least common site [3]. The name SS arises from the histological appearance which resembles a synovial membrane [4]. There have been very few cases of laryngeal SS reported in the literature. 2. Case Report An otherwise fit and well nonsmoking 20-year-old male presented with an acute onset of respiratory stridor associated with a sore throat and odynophagia. Flexible nasendoscopy revealed a large well circumscribed mass in the left aryepiglottic fold which was causing some degree of laryngeal inlet obstruction. Computer tomography (CT) scanning confirmed a large cystic mass measuring 7.5£¿cm ¡Á 3.6£¿cm arising from the left supraglottic larynx extending through the cricothyroid membrane into the left thyroid lobe (Figure 1). There was associated laryngotracheal deviation (Figure 2). An emergency tracheostomy was performed and an open biopsy of the left thyroid mass was obtained. Histopathological examination revealed a high grade biphasic tumour composed of nests and ribbons of epithelioid and plump spindle cells with areas of
%U http://www.hindawi.com/journals/criot/2013/578606/