A 66 year old woman, with no significant medical history, presented to her general practitioner with progressive general malaise that had lasted 6 months. She intentionally lost 10 kilograms because she was overweight. For 1 week, she had suffered from fever, night sweats, and fatigue in the lower limbs. She had no history of tuberculosis and, although living in an endemic Q-fever area, she had had no contact with sheep or goats. After 2 weeks she was referred to a rheumatologist who found an increased white cell count of 25.2 x 109/L (normal range 4-11 x 109/L), an increased C-reactive protein (CRP) level of 42 mg/L (normal range <10), and an increased erythrocyte sediment rate of 48 mm/hour (normal range <30 mm/hour). With the differential diagnosis of an unknown autoimmune disease she was treated with prednisone. As the symptoms persisted after 3 weeks, she was admitted to the department of internal medicine at a rural hospital. The initial differential diagnosis included an unknown generalized infection. Therefore, blood cultures were taken, which were negative and remained negative. The blood levels at that time were: an increased CRP of 106 mg/L, an increased white cell count of 20.6 x 109/L, and negative Q fever and lues. A computerized tomography (CT) scan showed a filling defect of the descending aorta and the coeliac trunk (Fig. 1), which was interpreted as a possibly infected thrombosis and was treated with gentamicin and vancomycin. To prevent further growth of a possible thrombus, the patient was treated with therapeutic Nadroparin (Aspen Pharma Trading, Dublin, Ireland). The CT findings suggested positron emission tomography (PET), which showed a hotspot at the filling defect of the descending aorta and a small hotspot in the right tibia, considered to be septic emboli. A magnetic resonance imaging (MRI) scan of the tibia was performed. The differential diagnosis of the lesion in the tibia was inflammation or a malignancy.
The patient was referred to the department of vascular surgery of a tertiary referral hospital. Here the antibiotic was discontinued. On admission, no abdominal abnormalities were found on physical examination. The symptoms of the patient were persistent, but unchanged from the initial presentation. A multidisciplinary treatment team, including a cardiothoracic surgeon, a vascular surgeon, and a microbiologist recommended a magnetic resonance angiogram (MRA). This showed a non-enhancing lesion in the descending aorta without vessel wall involvement, concluding that the filling defect of the descending aorta was less suspicious of malignancy. Seven days after the referral, the patient developed acute abdominal pain. A CT scan was performed, which showed complete occlusion of the celiac trunk as well as infarctions in the left lobe of the liver, spleen, and both kidneys. To prevent further embolization and to limit any ischemia-reperfusion injury, urgent surgical intervention was performed. Because the MRA showed a non-enhancing lesion without vessel wall involvement, the defect was considered to be resectable, therefore endovascular recanalization and stenting was not considered before surgery.
A left thoracophrenic-laparotomy was performed using left heart bypass with a Biomedicus pump (Medtronic inc. Minneapolis, MN, USA). The distal part of the thoraco-abdominal aorta, from the 8th thoracic vertebrae down to the celiac trunk, was resected (Fig. 2A). An embolectomy of the celiac trunk was performed. An interposition graft (Intergard prosthesis o 22 mm, Maquet Getinge group, Intervascular, Athelia, La Ciotat, France) was inserted between the two ends of the aorta with an oblique anastomosis at the celiac trunk (Fig. 2B). The tumor appeared to have spread beyond the vascular wall into some of the intercostal arteries. After 4 days on intensive care, the patient returned to the ward, recovered without complications, and was discharged in good health 10 days after the operation. The total hospital stay was approximately 5 weeks.
Histological examination revealed an epithelioid angiosarcoma. The tumor was present at the proximal resection edge and the embolus was shown to be a malignant tumor of the same kind. The tumor was staged pT4N0M1. Treatment was taken over by an oncologist, who conducted new PET, CT, and MRI scans to determine the course of treatment. In the PET/CT, a few weeks after the surgery, new abnormalities were found in the right femur and os ilium, suspicious of metastasis. The patient underwent palliative treatment.