Laryngeal Synovial Sarcoma: A Rare Clinical Entity

Abstract

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.

Out-of-the-box soft tissue tumor: pediatric synovial sarcoma of larynx

“…3 Most individuals with laryngeal SS are male and are diagnosed in their third decade of life. 4 Morphologically, as per WHO classification, SS can be biphasic or monophasic with variable morphology showing spindle and/or epithelial cellular characteristics. Both types are characterized by monomorphic spindle cells with tapering nuclei and poorly defined cytoplasm, often arranged in fascicles within a collagenous stroma.…”

“…Both types are characterized by monomorphic spindle cells with tapering nuclei and poorly defined cytoplasm, often arranged in fascicles within a collagenous stroma. 4 The biphasic SS has the additive features of discernable glandular or solid epithelial structures. 7 Both, however, often contain some extent of stromal calcification.…”

“…7 Both, however, often contain some extent of stromal calcification. 4 Due to its rarity, recognition of this laryngeal SS is often very difficult, and diagnosis relies heavily on immunohistochemistry and cytogenetics. 1 Eighty percent of SS have a specific t(x;18) (p11.2; q11.2) chromosomal abnormality, leading to fusion of the SSX gene with the SYT gene, which contributes to morphology and outcome.…”

“…1 Eighty percent of SS have a specific t(x;18) (p11.2; q11.2) chromosomal abnormality, leading to fusion of the SSX gene with the SYT gene, which contributes to morphology and outcome. 3,4 Third of cases are monophasic or SSX2, 2/3 of cases are biphasic or SSX1, and rare cases are SSX4. Local recurrence is 45% of adults' head and neck SS, 33% metastasize, and 50% survive for 2 years.…”

Synovial Sarcoma of the Larynx: Report of a Case and Review of Literature

“…Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature (Table 1 [5–23]). Synovial sarcoma is an aggressive malignant soft tissue tumor that is thought to arise from pluripotent mesenchymal cells and usually involve the lower extremities [24].…”