Poster Presentation Clinical Oncology Society of Australia Annual Scientific Meeting 2023

Primary thoracic aorta angiosarcoma presenting with thromboembolism and progressive claudication despite anticoagulation (#220)

Jane McKenzie 1 , Sarat Chander 1 2 , Jeremy Lewin 1 3 4
  1. Peter MacCallum Cancer Centre, Melbourne, VICTORIA, Australia
  2. Honorary, Department of Pathology, University of Melbourne, Parkville, Victoria, Australia
  3. ONTrac at Peter Mac, Victorian Adolescent & Young Adult Cancer Service, Melbourne, Victoria, Australia
  4. Sir Peter MacCallum Department of Oncology, The University of Melbourne, Parkville, Victoria, Australia

Introduction

Angiosarcomas are aggressive cancers arising from lymphatic or vascular endothelium, comprising 1% of all soft tissue sarcomas. Primary aortic angiosarcomas are a rare subtype of angiosarcoma, frequently diagnosed in advanced stages due to initial misdiagnosis. Early surgical resection offers the best chance of survival, and despite use of palliative radiotherapy and chemotherapy for locally advanced or metastatic angiosarcoma, survival remains poor.

 

Case

A 67-year-old woman initially presented with a distal thoracic aorta thrombus and symptomatic bilateral popliteal emboli, underwent right popliteal artery thrombectomy and left popliteal vein patch, and was commenced on warfarin. Histology revealed bland thrombus and thrombophilia screen was unremarkable. Over subsequent months she experienced progressive lower limb pain and intermittent claudication. Surveillance ultrasound showed occluded popliteal arteries with good collateralisation and lower limb symptoms were attributed to known degenerative spinal canal stenosis. 12 months following initial presentation, she re-presented with constitutional symptoms, 20kg loss of weight, progressive lower limb claudication and melaena. CT abdomen and pelvis revealed a new solid right renal lesion and a persistent distal thoracic aorta lesion now causing 90% luminal stenosis. Subsequent MRI favoured primary malignancy rather than bland thrombus and PET revealed FDG-avid bilateral renal and soft tissue metastatic deposits. Renal biopsy was diagnostic for metastatic angiosarcoma. She commenced palliative radiotherapy to the primary aortic lesion for symptom control with evidence of response, however died following embolic complications with small bowel ischemia.

 

Conclusion

Primary aortic angiosarcoma is an aggressive malignancy where early recognition is vital to improve outcomes. Suspicion should be raised in the case of thrombus in unusual segments (e.g. thoracic aorta) or progressive course despite anticoagulation. Multimodal imaging including PET is useful to distinguish from benign aetiologies.