Laryngeal Synovial Sarcoma: a Rare Clinical Entity
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CASE REPORT
Laryngeal Synovial Sarcoma: a Rare Clinical Entity Sravan Kumar 1 & Srijan Shukla 1 & S. Avinash 1 & Daphne Fonseca 2 & Hemant Nemade 1 & LMCS Rao 1 & T. Subramanyeswara Rao 1 Received: 23 October 2019 / Accepted: 7 May 2020 # Indian Association of Surgical Oncology 2020
Abstract Synovial sarcomas in head and neck region are extremely rare and have an aggressive nature and an unpredictable prognosis. The principles of management are still controversial, and a multidisciplinary approach is essential in managing Synovial sarcomas. We present a case report here for its rarity along with a brief review of literature.
Introduction Head and neck sarcomas amount to less than 10% of all sarcomas in the body. Of these, Synovial sarcomas account for 8 to 10% [1]. Synovial sarcoma arises from pluripotent mesenchymal cells found in periarticular areas [2]. Synovial sarcoma in head and neck region is extremely rare and has an aggressive nature and an unpredictable prognosis. This case report is being presented here for its rarity along with a brief review of literature.
Case Report A 28-year-old female presented with difficulty in swallowing (for both solids and liquids) and change in voice for 2 months. She underwent endoscopic assessment of the larynx and pharynx which showed proliferative growth involving the right vocal cord and right aryepiglottic fold with impaired mobility. Biopsy of the lesion was done which showed poorly differentiated neoplasm; further, immunohistochemical analysis showed strong positivity for TLE-1, suggesting Synovial sarcoma as the diagnosis. Contrast-enhanced CT of the neck showed heterogeneous enhancing soft tissue density (20 × 19 mm) involving the right * Sravan Kumar [email protected] 1
Department of Surgical Oncology, Basavatarakam Indo American Cancer Hospital and Research Institute, Banjara Hills Road No 10, Hyderabad, Telangana 500034, India
2
Department of Pathology, Basavatarakam Indo American Cancer Hospital and Research Institute, Banjara Hills Road No 10, Hyderabad, Telangana 500034, India
pyriform fossa and minimal extension to posterior pharyngeal wall medially abutting the right paraglottic space and right vocal cord. No significant cervical lymph nodes were seen. The details of the case were discussed in multidisciplinary tumor board, and she underwent LASER-assisted partial pharyngectomy. Histopathology showed lesion of size 2.2 × 1 × 0.8 cm with adequate margins, routine H&E sections showed spindle cells arranged in plump fascicles with hyalinization and focal whorling, on IHC, Cytokeratin 7 was positive, Pancytokeratin was positive, Calponin was positive, CD117 was negative, Cytokeratin 5/6 was negative, and TLE1 was positive, hence suggestive of Synovial sarcoma. After discussing again in multidisciplinary tumor board, she was kept on close follow-up. She was on regular followup for 2 years, during which the endoscopic evaluations of the larynx and hypopharynx were normal. Later on she developed dysphasia and visible neck mass in the right paratracheal re
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