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Aortic sarcomas have not been linked to Lynch syndrome in humans, although other soft tissue malignancies have been. We report the case of a 31-year-old man with Lynch syndrome, who presented with abdominal pain and severe claudication. The clinical and diagnostic workup revealed near occlusion of the infrarenal aorta due to aortic angiosarcoma. En bloc resection of the visceral and infrarenal aorta with right nephrectomy was performed, facilitated by temporary extracorporeal bypass to the visceral arteries. The aorta was reconstructed with a bifurcated Dacron graft. At the 24-month follow-up examination, the patient was free of disease but was experiencing chronic diarrhea. Keywords: Angiosarcoma, Lynch syndrome, Aortic reconstruction, Temporary bypass

Patient: Female, 82-year-old Final Diagnosis: Angiosarcoma of the aorta Symptoms: Fever Medication: — Clinical Procedure: — Specialty: Pathology Objective: Rare disease Background: Primary aortic sarcoma often poses diagnostic challenges for pathologists and clinicians because of a very low incidence and controversy over nomenclature and definition. We report a case of aortic angiosarcoma in association with a graft. We also conducted a clinicopathological review of cases of primary aortic sarcomas associated with implanted grafts. Case Report: The patient was an 82-year-old woman. She underwent thoracic endovascular aneurysm repair (TEVAR) at age 78 because of an aneurysm in the descending aorta. Approximately 4 years after the TEVAR, computed tomography revealed a type II endoleak and expansion of the aneurysm. Her c-reactive protein level rose to 34 mg/dL, and Ga scintigraphy showed 67Ga accumulation at the aneurysm. She had fever up to 39°C, and a stent graft infection was suspected. Despite administration of antibiotics, her condition deteriorated, and she died. Postmortem examination identified epithelioid aortic angiosarcoma at the aorta with aneurysm repair and the graft, and the aortic angiosarcoma invaded the left lower lobe of the lung. Conclusions: Our clinicopathological review revealed that the proper clinical diagnosis was very difficult owing to confusion of aortic sarcoma after the implantation with the infected graft, atypical endoleak, or pseudoaneurysm. The histological diagnosis was ambiguous because immunohistochemical and genetic studies were not adequately conducted. Overall prognosis of aortic sarcoma is poor as most patients die within a year, with no effective treatments. It is hoped that recent projects for genomic medicine will provide useful insights about the diagnosis and treatment of these cancers.

Angiosarcomas are rare vascular connective tissue tumors originating from the tunica intima, media, or adventitia and may arise in large vessels. Here two cases of primary angiosarcoma of the aorta are described that highlight initial manifestations and unique complications from diffuse arterial tumor emboli, which led to delayed cancer diagnosis. In patients with aortic lesions, imaging characteristics must be thoughtfully reconciled with clinical scenarios, particularly in patients with no additional evidence of atherosclerosis or vasculitis. Keywords: Oncology, Neoplasms-Primary, MR-Angiography, Aorta © RSNA, 2021.

Background Aortic thrombosis represents a consequence of atherosclerotic disease. In few cases, it can be secondary to large vessel or infective vasculitis. More rarely, aortic thrombosis is the manifestation of a primary malignant neoplasm of the aortic wall. Aortic angiosarcoma is a rare tumour, its clinical presentation is often non-specific and associated signs and symptoms may vary greatly. An early diagnosis is difficult to reach and the presence of metastatic disease is not uncommon at the time of diagnosis. The prognosis is poor overall. Case summary We report the case of a female patient who presented to her GP because of fatigue, hyporexia, weight-loss, and anaemia. An ultrasound of the abdomen showed two small pancreatic lesions, confirmed and described as benign cystic pancreatic lesions on computed tomography (CT) imaging; an incidental thrombus in the superior mesenteric artery was also found on CT imaging. The thoracic CT identified a large thrombotic lesion in the descending thoracic aorta with significant narrowing of the aortic lumen and confirmed the presence of an osteolytic bone lesion on the VIII right rib, in the absence of atherosclerotic disease. Signs of increased metabolic activity in the aortic lumen and in the VIII posterior right rib were shown at a subsequent positron emission tomography. A CT-guided biopsy of the bone lesion was performed and at histology the diagnosis of metastatic angiosarcoma of the aortic wall was made. Discussion Aortic angiosarcoma is a rare cause of aortic thrombosis, to be taken into consideration in a patient with thrombotic lesions of the aorta in the absence of atherosclerotic disease. The differential diagnosis is difficult because of clinical presentation and radiological features similar to those of inflammatory aortic disease. In our case, the final diagnosis of angiosarcoma was made only by a biopsy of a bone metastatic lesion.

Aortic angiosarcoma is an exceedingly rare clinical entity. A significant delay in diagnosis can occur due to its rareness and lack of specific clinical manifestation. A 71-year-old woman was admitted to the emergency department owing to an acute episode of abdominal pain. A computed tomography (CT) scan showed thoraco-abdominal aortic occlusion and splenic infarction. The patient was initially treated with descending aortic replacement for degenerative atherothrombotic aneurysm. An FDG-positron emission tomography-CT scan and biopsy ultimately confirmed the diagnosis of aortic angiosarcoma. This case highlights the difficulties of making an early diagnosis of aortic angiosarcoma.

Primary cardiac and aortic angiosarcomas are rare and clinically challenging malignant tumors of vascular origin, with >90% arising from the right atrium. Clinical manifestations appear from myocardial ischemia, mass effect, or distant metastases. If origination from the ascending aorta (AAo), angiosarcoma tumors can elude detection by trans-thoracic echocardiogram. We present a case of a 55-year-old male with acute coronary syndrome (ACS) and cardiogenic shock, who was found to have primary aortic angiosarcoma of the AAo with multifocal hemorrhagic brain metastases. Aortic angiosarcoma is an extremely rare and aggressive tumor with poor prognosis, and can be difficult to diagnose. Despite complete surgical resection, cardiac and aortic angiosarcomas carry a high mortality rate, often with recurrence and metastases to lungs, bones, and brain. This report highlights an unusual presentation of a rare disease, and the importance of multimodal diagnostic strategies in complex presentations of ACS.

Paraneoplastic autoimmune encephalopathic syndromes have been described most often in association with small cell lung cancer or breast cancer, tumours of the ovaries, testes, lymphoma and thymoma. Antibodies associated with paraneoplastic encephalopathies are, among others, anti-Hu, anti-Ma2 and, in part, anti-N-methyl-D-aspartate(NMDA)-receptor antibodies. Here, we present the case of a 72-year-old patient hospitalised due to progressive cognitive decline and disorientation. Diagnostic workup revealed paraneoplastic anti-amphiphysin associated limbic encephalitis on the basis of an aortic angiosarcoma with metastases to kidney, muscle and bones. Highly aggressive chemotherapy as well as immunosuppressive therapy and cytoreductive laparoscopic nephrectomy were initiated. However, follow-up revealed further tumour progress and a worsening of neurological symptoms.

Intimal angiosarcoma is a most unexpected cause of aortic occlusion. We present the case of a 74-year-old woman with intimal angiosarcoma that manifested with the triad of congestive heart failure, acute renal failure, and abdominal angina. A review of the literature and discussion of postoperative outcomes follows.

A primary intimal aortic angiosarcoma was diagnosed in a 4-year-old, spayed female German Shepherd that presented for complications of thromboembolic disease because of infarcts in multiple organs. On gross examination, aneurysmal dilatation of the aorta was associated with a friable, necrotic mass attached to the endothelial surface, which partially occluded the aortic lumen. On histologic examination, plump neoplastic spindle cells formed a plaque-like mass arising from the intima that merged with a large accumulation of fibrin and necrotic debris, and projected into the lumen. Neoplastic cells invaded periaortic vessels and were seen in some infarct-associated thromboemboli. Tumor cells expressed vimentin and CD31, with infrequent, patchy staining with factor VIII-related antigen; tumor cells were negative for cytokeratin and smooth-muscle actin. Aortic angiosarcoma is a rare malignancy in humans. This is the first description of a primary intimal aortic angiosarcoma in a dog, with immunohistochemical evidence of endothelial origin.

Purpose We report a technique of finite-element multidimensional modeling that was used to help with the planning of and the resection of an angiosarcoma in a single patient. Description A patient was referred to our department with suspected aortic angiosarcoma. We visualized and reconstructed the computed tomography and magnetic resonance imaging scans of this patient to create finite-element multidimensional models of his diseased aorta. Evaluation This technique and the multidimensional models were very helpful in assessing the tumor size and its extension. It also facilitated preoperative planning of the aortic resection and repair. Conclusion Finite-element multidimensional modeling is a useful technique for preoperative planning of aortic operations in patients with angiosarcoma.

Primary angiosarcoma of the aorta is a rare tumor. The symptoms resemble those of atherosclerotic occlusive disease, and the radiomorphologic pattern is often nonspecific. In most published cases, the malignant vascular obstruction was diagnosed histopathologically after surgical vascular reconstruction. We report on interventional and CT-angiographic features of an abdominal aortic angiosarcoma, observed in a 71-year-old patient. The polyploid intimal alteration is clearly depicted on CT images. Morphology and the segmental obstruction of the aortic lumen without aneurysmal or extensive atherosclerotic mural changes should lead to the differential diagnosis of an intravascular malignancy.

Primary aortic tumors are rare, difficult to diagnose, and often fatal. This case and a review of the literature identified aortic tumors as a potential source of emboli leading to acute mesenteric insufficiency. The case showed the efficacy of magnetic resonance imaging in diagnosing and determining the extent and location of an aortic tumor.

Although extremely rare, the group of primary malignant tumors of the aorta (PMTA) exhibits enormous histologic heterogeneity. In most cases, diagnosis is established late in the course of the disease; the median survival time is only a few months. We present the case of a 75-year-old patient with an intimal angiosarcoma of the infrarenal abdominal aorta and discuss the clinical presentation, histopathologic diagnosis, and classification of primary aortic sarcomas. A critical review of the diagnostic and therapeutic management in this case revealed that the atypical aortic thrombus should have prompted a comprehensive preoperative diagnostic work-up, specifically with magnetic resonance tomography of the aorta and bone scintigraphy.

Primary malignant tumors of the thoracic and abdominal aortas are extremely rare. In most cases, a diagnosis is established either postmortem or after an emergency operation for embolic complications. We present the case of a thoracic aortic angiosarcoma in a 71-year-old man who initially was seen with peripheral embolization. The management of these tumors and the world literature are reviewed. (J Vasc Surg 1998;28:732-7.)

Aortic angiosarcoma is an exceedingly rare clinical entity. Significant delay in diagnosis can occur due to a low index of suspicion on the part of the clinician. We report a case of aortic angiosarcoma masquerading as a descending thoracic aneurysm arising from a penetrating ulcer. The patient was initially treated with an endovascular stent graft for rapid growth, but the lesion continued to enlarge despite angiographic exclusion. FDG-PET CT scan and biopsy ultimately confirmed the diagnosis of aortic angiosarcoma. This case highlights some of the difficulties of making the early diagnosis of aortic angiosarcoma.