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 × 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
References
[1]
E. M. Sturgis and B. O. Potter, “Sarcomas of the head and neck region,” Current Opinion in Oncology, vol. 15, no. 3, pp. 239–252, 2003.
[2]
S. Pai, R. F. Chinoy, S. A. Pradhan, A. K. D'Cruz, S. V. Kane, and J. N. Yadav, “Head and neck synovial sarcomas,” Journal of Surgical Oncology, vol. 54, no. 2, pp. 82–86, 1993.
[3]
A. P. Dei Tos, R. Sciot, C. Giannini et al., “Synovial sarcoma of the larynx and hypopharynx,” Annals of Otology, Rhinology and Laryngology, vol. 107, no. 12, pp. 1080–1085, 1998.
[4]
S. Kusuma, D. J. Skarupa, K. A. Ely, A. J. Cmelak, and B. B. Burkey, “Synovial sarcoma of the head and neck: a review of its diagnosis and management and a report of a rare case of orbital involvement,” Ear, Nose and Throat Journal, vol. 89, no. 6, pp. 280–283, 2010.
[5]
American Cancer Society, Cancer Facts & Figures, American Cancer Society, Atlanta, Ga, USA, 2012.
[6]
M. F. Okcu, M. Munsell, J. Treuner et al., “Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome,” Journal of Clinical Oncology, vol. 21, no. 8, pp. 1602–1611, 2003.
A. Al-Nemer and M. A. El-Shawarby, “Laryngeal synovial sarcoma: case report and Literature review,” Gulf Journal of Oncology, vol. 9, pp. 52–56, 2011.
[9]
Diagnostic Histopathology of Tumors, Churchill Livingstone Elsevier, 3rd edition, 2007.
[10]
P. Bergh, J. M. Meis-Kindblom, F. Gherlinzoni, et al., “Synovial sarcoma: identification of low and high risk groups,” Cancer, vol. 85, pp. 2596–2607, 1999.
[11]
R. L. Randall, K. L. S. Schabel, Y. Hitchcock, D. E. Joyner, and K. H. Albritton, “Diagnosis and management of synovial sarcoma,” Current Treatment Options in Oncology, vol. 6, no. 6, pp. 449–459, 2005.
[12]
J. F. Tierney, “Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data,” The Lancet, vol. 350, no. 9092, pp. 1647–1654, 1997.
[13]
B. A. Guadagnolo, G. K. Zagars, M. T. Ballo et al., “Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy,” International Journal of Radiation Oncology Biology Physics, vol. 69, no. 4, pp. 1173–1180, 2007.
[14]
W. J. Harb, M. A. Luna, S. R. Patel, M. T. Ballo, D. B. Roberts, and E. M. Sturgis, “Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension,” Head and Neck, vol. 29, no. 8, pp. 731–740, 2007.
[15]
S. Kawaguchi, T. Wada, K. Ida et al., “Phase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovial sarcoma,” Journal of Translational Medicine, vol. 3, article 1, 2005.
[16]
V. Balakrishnan, S. Flatman, B. J. Dixon, and B. Lyons, “Synovial sarcoma of the pharynx causing airway obstruction,” Medical Journal of Australia, vol. 196, no. 1, pp. 72–73, 2012.
