Clinically, the symptomatology is nonspecific patients often present with systemic signs of tumor fragment embolism, such as limb claudication or mesenteric ischemia.
Primary sarcomas of the aorta are extremely rareĪnd are usually located in the descending thoracic aorta. The local recurrence and the hepatic metastases were reduced after 2 months of treatment and metabolically silent on 18-F–FDG PET imaging.Īfter 1 year, the patient is still alive with persistent paraplegia for which he has started an intensive neurological rehabilitation program. CT-scan revealed a right iliac venous thrombosis extending to the femoral vein, requiring an urgent inferior vena cava filter placement.Īfter 2 months of follow-up another MRI showed a local recurrence and liver metastases, for which the patient started an immunotherapy treatment with pazopanib 800 mg/day. However, he was readmitted due to dyspnea after 2 days.
The patient was discharged on the 10th postoperative day. These signs were associated with a spinal cord injury at the L1/L2 level. The intimal layers of the resected aorta were involved with margins microscopically free.Īt the end of surgery, the patient showed flaccid paraplegia with loss of anal sphincter control and a tactile hypoesthesia. The immunohistochemical staining was positive for ETS-related gene protein and ubiquitin carboxyterminal hydrolase and focally positive for CD 31, factor VIII, and S100. Histopathological analysis confirmed the diagnosis of intimal sarcoma with greater than 50% necrosis, pleomorphism, and a mitotic index exceeding to 10 × 10 power fields. ), positioned with an end-to-side anastomosis above the celiac trunk before that the complete resection of the neoplasm was performed (
Under full heparinization, the descending thoracic aorta was isolated, clamped, resected, and reconstructed with a prosthetic tube of Dacron (18 mm A new MRI (Ĭonsidering the patient characteristics, the clinical investigations, and the poor response to chemotherapy, a multidisciplinary discussion suggested a salvage surgical resection.Ī left thoracoabdominal approach was chosen. The patient underwent neoadjuvant chemotherapy treatment with one cycle of epirubicin and taxolo. The diagnosis was later achieved with a left thoracoscopic surgical biopsy and the final histopathology revealed a primitive sarcoma of the aortic intima. To obtain a histological diagnosis of the tumor, a CT-guided biopsy was attempted without success.