Abstract
Primary mediastinal sarcoma is a rare tumour that usually presents with nonspecific symptoms such as hoarseness, dyspnoea, and chest pain. Superior vena cava (SVC) syndrome is an extremely uncommon complication that is caused by the compression, invasion, and thrombosis of the SVC or brachiocephalic veins. SVC syndrome can present as asymptomatic cases or as rare life-threatening emergencies with upper airway obstruction and increased intracranial pressure. This report describes the case of a 58-year-old female who presented with swelling of the face, neck, and upper limbs associated with dyspnoea on exertion. The radiological investigations revealed a large well-defined central necrotic peripherally enhancing lesion in the superior mediastinum extending anteriorly with the compression of brachiocephalic veins. A histopathological examination detected spindle cells arranged in fascicles with nuclear atypia with immunohistochemistry positive for creatine kinase (CK), smooth muscle actin (SMA), desmin and CD99. These findings established the diagnosis of a mediastinal monophasic synovial sarcoma with SVC obstruction. The patient was initiated on palliative radiotherapy for the management of the SVC, followed by systemic biological treatment with the tyrosine kinase inhibitor pazopanib, and was clinically improved. It is essential to promptly diagnose and treat this condition, especially when SVC syndrome manifests.
Reference14 articles.
1. Pulmonary and thoracic sarcomas;Curr. Diagn. Pathol.,2006
2. Salah, S., Al-Ibraheem, A., Daboor, A., and Al-Hussaini, M. (2013). Synovial sarcoma presenting with huge mediastinal mass: A case report and review of literature. BMC Res. Notes, 6.
3. Mediastinal Synovial Sarcoma: Clinicopathologic Analysis of 21 Cases With Molecular Confirmation;Am. J. Surg. Pathol.,2018
4. Poorly differentiated monophasic synovial sarcoma of the mediastinum;Indian J. Pathol. Microbiol.,2011
5. Primary synovial sarcomas of the mediastinum: A clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases;Am. J. Surg. Pathol.,2005