Decade-long Disease Control in Metastatic Epithelioid Angiosarcoma of the Thoracic Spine Following Surgery and Consolidation Radiotherapy: A Case Report and Review of the Literature

Abstract

Background: Epithelioid angiosarcoma (EAS) is a rare, high-grade vascular malignancy with epithelioid morphology and considerable diagnostic overlap with poorly differentiated carcinoma. Primary spinal involvement is exceptionally uncommon, and metastatic disease at presentation is generally associated with short survival in the published literature. Case Presentation: We report the case of a 32-year-old man who presented with progressive paraparesis secondary to a D9 vertebral tumour causing spinal cord compression. He underwent decompressive laminectomy with posterior instrumentation. Histopathological and immunohistochemical assessment supported a diagnosis of EAS. Staging computed tomography identified four small pulmonary micronodules that were interpreted as metastatic disease. Owing to microscopically incomplete resection, low-volume pulmonary involvement and preserved performance status, the multidisciplinary tumour board recommended consolidation radiotherapy to the operative spinal region at 20 Gy in five fractions, followed by clinical and radiological surveillance without systemic chemotherapy. During more than ten years of follow-up, the residual vertebral lesion and pulmonary micronodules remained radiologically stable, and the patient remained clinically well with no further oncological intervention. Conclusion: This case documents an unusual long-term stable course in spinal EAS with suspected synchronous pulmonary metastases. It supports the need for careful pathological diagnosis, multidisciplinary assessment and cautious individualisation of management in selected patients with low-volume, clinically silent disease.