Epithelioid hemangioendothelioma mimicking aortic intramural haematoma.

Abstract

A 69-year-old man presented with a dull chest pain for 2 months. He denied history of hypertension or trauma. Computed tomography angiography (CTA) indicated ‘intramural haematoma’ in descending aorta (Panel A). Control blood pressure and heart rate were as follows: target 120/70 mm/Hg, 60 beats/min, and the patient was discharged. One month later, to check the progression of aortic intramural haematoma, CTA was done (Panel B). The intramural haematoma area is larger than before. Positron emission tomography/computed tomography (PET/CT) was done for the enlarged area of intermural haematoma without clinical manifestation. PET/CT revealed abnormal intense high metabolic region around aorta (Panel C) and a similar hypermetabolic mass in right lower kidney (Panel D), as well as abdominal lymph node metastasis. For histologic confirmation, biopsy of the renal mass was obtained, and pathological studies supported the diagnosis of epithelioid haemangioendothelioma. The pathologic finding showed irregular nests of atypical epithelioid cells with intracytoplasmic vacuoles (Panel E, arrow). The tumour cells are immunoreactive to ETS-related gene (Panel F). The final diagnosis was made as epithelioid haemangioendothelioma around aortic with multiple metastasis. Our patient died <6 months after the diagnosis was made.