A case of primary aortic sarcoma with tumor infarction after stent graft placement

Primary aortic sarcoma is a very rare disease, and most primary aortic tumors are malignant mesenchymal tumors. We present the case of a 62-year-old man with sudden epigastric and back pain. Contrast-enhanced computed tomography (CT) revealed a mass lesion about 33.8 mm in diameter, in contact with the left side of the abdominal aorta. Impending rupture of an abdominal aortic aneurysm was suspected, so cardiovascular surgery for stent graft placement was performed the same day. Symptoms immediately improved and CT at 3 months postoperatively showed a marked decrease in lesion size, but the lesion subsequently grew again. Fluorodeoxyglucose (FDG)-positron emission tomography/CT was performed due to the possibility of malignant solid tumor, revealing markedly increased FDG accumulation (maximum standardized uptake value, 36.95) in the mass lesion. Primary aortic sarcoma was diagnosed from thoracoscopic biopsy. Here, we report a primary aortic sarcoma that shrank due to tumor infarction after stent graft placement, followed by tumor regrowth.

Diagnostic Enigma: Spindle Cell Sarcoma of the Aorta Presenting as Pulmonary Embolism and Chronic Anaemia

Primary aortic sarcoma is a rare and aggressive malignancy with only approximately 190 cases reported in the literature. While angiosarcoma and intimal sarcomas represent an estimated 67.7% of malignant aortic tumours, spindle cell sarcomas are even more exclusive, consisting of only 0.9% of malignant aortic tumours. Differentiated from other malignant aortic tumours, spindle cell sarcomas are of mesenchymal origin and usually express vimentin and osteopontin. Clinical presentations are variable and nonspecific, ranging from back pain, abdominal pain or elevated blood pressure, misleading to differentials like pulmonary emboli or aortic aneurysms such as in our case here. In this article, we discuss the finding of an extremely rare aortic sarcoma masquerading as a pulmonary embolism. The patient underwent surgical resection; however, the course was complicated by the development of brain metastases and intracranial haemorrhage. The literature is expanding regarding the evolution of adjuvant chemotherapy and radiation therapy in the treatment of these patients. The exact pathogenesis of spindle cell sarcomas is unknown but thought to be related to the MDM2-p53 pathway. The development of spindle cell sarcomas may be related to Li-Fraumeni syndrome, which should be on the differential for these patients. This case highlights the importance of identifying aortic sarcomas in patients who present with signs and symptoms of peripheral embolization as the diagnosis can be easily misconstrued for thrombus or aortic aneurysm, leading to a delay in proper and timely management. We herein emphasize that aortic sarcomas should be included in the clinician’s working differential due to the poor prognosis and outcomes that these aggressive tumours carry.

Manifestation of Pleomorphic Undifferentiated Aortic Sarcoma with Splenic Infarction: A Case Report

Aortic spindle cell sarcoma is a rare neoplasm with poor prognosis that is often found incidentally due to its adverse effects. CT and MRI with contrast are useful imaging modalities, but a tissue biopsy is the gold standard for diagnosis. Tumor resection is the ultimate treatment followed by chemotherapy. Our case was an adult female who presented mainly for shortness of breath, and further imaging workup demonstrated a soft tumor juxtaposed to a major vein with compressive effect. The patient’s tumor was resected, and the pathology result confirmed undifferentiated aortic sarcoma. The patient’s condition improved and she was discharged with outpatient oncology follow-up and possible treatment.

Imaging, Surgical, and Pathology Findings of a Myxoid Aortic Sarcoma: When Distal Embolization Uncovers a Bigger Problem

Aortic sarcomas are a very rare condition typically characterized by a deceiving presentation. Making a correct diagnosis is based on the application of an algorithm which allows to identify the primary disease site and to obtain a tissue diagnosis. Surgical aortic resection with adjuvant therapy offers the best palliation, particularly in cases of well-differentiated tumors with no evidence of diffuse metastatic spread.