Laryngeal Synovial Sarcoma: A Rare Clinical Entity

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 lef...

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Main Authors: Clair Saxby, Ron Bova, Melanie Edwards
Format: Article
Language:English
Published: Hindawi Limited 2013-01-01
Series:Case Reports in Otolaryngology
Online Access:http://dx.doi.org/10.1155/2013/578606
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spelling doaj-a22728aded334d428915ba81c83fa14a2020-11-25T00:45:17ZengHindawi LimitedCase Reports in Otolaryngology2090-67652090-67732013-01-01201310.1155/2013/578606578606Laryngeal Synovial Sarcoma: A Rare Clinical EntityClair Saxby0Ron Bova1Melanie Edwards2St Vincents Hospital, Sydney, NSW 2010, AustraliaSt Vincents Clinic, Sydney, NSW 2010, AustraliaSt Vincents Hospital, Sydney, NSW 2010, AustraliaIntroduction. 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.http://dx.doi.org/10.1155/2013/578606
collection DOAJ
language English
format Article
sources DOAJ
author Clair Saxby
Ron Bova
Melanie Edwards
spellingShingle Clair Saxby
Ron Bova
Melanie Edwards
Laryngeal Synovial Sarcoma: A Rare Clinical Entity
Case Reports in Otolaryngology
author_facet Clair Saxby
Ron Bova
Melanie Edwards
author_sort Clair Saxby
title Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_short Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_full Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_fullStr Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_full_unstemmed Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_sort laryngeal synovial sarcoma: a rare clinical entity
publisher Hindawi Limited
series Case Reports in Otolaryngology
issn 2090-6765
2090-6773
publishDate 2013-01-01
description 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.
url http://dx.doi.org/10.1155/2013/578606
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AT ronbova laryngealsynovialsarcomaarareclinicalentity
AT melanieedwards laryngealsynovialsarcomaarareclinicalentity
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