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Primary angiosarcomas of the aorta are rare, highly aggressive malignant tumors for which there have been limited documented cases in the medical literature. Diagnosis is challenging due to nonspecific symptomatology and imitation of common aortic pathologies. A subset of these primary aortic angiosarcoma cases is seen in association with endovascular aneurysm repair, or EVAR; however, the connection between the 2 entities is not completely understood. Here, we present 18F-FDG PET/CT imaging findings of an angiosarcoma of the aorta in a 69-year-old man status post EVAR.

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.

In a 72-year-old patient with anemia (Hb 7 g/dl), duodenoscopy exhibited an area of polypous irregular-looking mucosa. Histology revealed duodenal infiltration by epithelioid tumor cells, immunhistochemically positive for endothelial cell markers (CD34, CD31, ERG). Ultrasonography showed thrombotic material in the otherwise unremarkable abdominal aorta with some uptake of contrast bubbles on CEUS. Histological and clinical diagnosis assumed an epithelioid angiosarcoma of the thoraco-abdominal aorta at the level of the visceral and renal arteries with duodenal metastases.To prevent further shedding of tumor cells and aortic rupture, a branched stent-graft was placed into the thoraco-abdominal aorta. Palliative chemotherapy with gemcitabine and docetaxel was started, leading to a partial remission after 6 cycles. Three months later, however, there was a progress of the duodenal masses with new pulmonal and osseous metastases. Thirteen months after the initial diagnosis, death occured due to a hemorrhagic shock caused by a hematothorax.Aortic tumors are exceedingly rare, with only slightly more than 220 cases reported so far. In most cases, diagnosis is made either at autopsy or after an emergency operation for embolic complications like embolic intestinal ischemia. With an overall median survival of 8 months, prognosis is very poor.This case sensitizes for the correct sonographic interpretation of aortic "thrombi" in an otherwise normally appearing aorta, possibly with the aid of CEUS. Besides, it demonstrates the relatively early and uncommon diagnosis of an aortic angiosarcoma by the combination of endoscopy, immunohistochemistry, and ultrasonography.Bei einem 72 Jahre alten anämischen Patienten (Hb 7 g/dl) fand sich duodenoskopisch ein Bereich mit polypöser, unregelmäßiger Schleimhaut. Histologisch lag eine Duodenalinfiltration durch epithelioide Tumorzellen vor, immunhistochemisch positiv für endotheliale Marker (CD34, CD31, ERG). Sonografisch zeigte sich thrombotisches Material in der ansonsten unauffälligen Bauchaorta mit einer leichten Kontrastaufnahme in der CEUS. Histologisch und klinisch wurde die Diagnose eines duodenal metastasierten epithelioiden Angiosarkoms des thorako-abdominellen Aortenabschnitts auf Höhe der Viszeral- und Nierenarterien gestellt.Um ein weiteres Ablösen von Tumorzellen zu verhindern, wurde eine Aortenstentprothese mit 4 Seitenarmen in die thorako-abdominelle Aorta implantiert. Eine palliative Chemotherapie (Gemcitabin/Docetaxel) führte nach 6 Zyklen zu einer partiellen Remission. 3 Monate später kam es jedoch zu einem Progress im Duodenum mit pulmonalen und knöchernen Filiae. Der Patient starb 13 Monate nach der Erstdiagnose an einem hämorrhagischen Schock bei Hämatothorax.Mit nur etwas mehr als 220 beschriebenen Fällen sind Aortentumoren sehr selten. Die Diagnose erfolgt meist autoptisch oder nach einer Notfalloperation bei embolischen Komplikationen. Die Prognose ist bei einem medianen Überleben von 8 Monaten sehr schlecht.Dieser Fall sensibilisiert für die korrekte sonografische Interpretation von Aorten-„Thromben“ in einer ansonsten normal erscheinenden Aorta, möglicherweise mithilfe von CEUS. Außerdem beschreibt er die relativ frühe und ungewöhnliche Diagnose eines aortalen Angiosarkoms durch die Kombination von Endoskopie, Immunhistochemie und Sonografie.

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.

Primary malignant tumours of the aorta are rare. They present with aneurysm formation, arterial occlusion, and embolic phenomenon. We report the case of a 56-year-old man whose initial presentation and investigations lead to emergency endovascular stenting of a descending thoracic aneurysm with a contained leak. Initial response was favourable, yet the patient presented again with worsening symptoms. The circum-aortic haematoma expanded by 50% on subsequent imaging, but no endoleak was identified. When altered bone marrow signal was identified on magnetic resonance imaging, the possibility of malignancy was considered. A metastatic skin lesion was then biopsied, which demonstrated morphological and immunohistochemical features consistent with metastases from a pleomorphic sarcoma of the aorta.

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.

Primary aortic tumors are rare and include a variety of histologic types, including aortic angiosarcomas, with less than 100 cases being reported since the first description of these tumors in 1873. The diagnosis of aortic angiosarcoma is usually confirmed by postoperative histopathologic examination or postmortem study. We present a case of primary aortic angiosarcoma presenting with intermittent claudication and radiologic findings of aortoiliac atherosclerotic disease treated initially with stenting; lower extremity embolic skin metastases developed during follow-up that prompted resection of the aortic bifurcation and restoration of the arterial continuity with a bypass. Despite postresection recurrence, a 3-year survival was achieved with aggressive multidisciplinary management.

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.

Aortic angiosarcoma is a rare, malignant neoplasm of the vasculature, with 24 case reports in the literature. Patients usually present with either aneurysmal or occlusive disease. Treatment consists of en bloc resection of the vessel and contiguous structures with postoperative chemoradiation therapy. Despite surgery and adjunctive measures, prognosis remains dismal as a result of early metastasis and late diagnosis. We report a case of aortic angiosarcoma that presented with distal thromboembolic phenomenon.

The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. Survival at 5 years was 8%. Survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection.

Aortic angiosarcoma is a rare malignancy. Clinical diagnosis is difficult, as the presenting symptoms mimic more common aortic lesions, particularly atherosclerosis. Dermatologists and dermatopathologists play a critical role in recognizing cutaneous metastases as a manifestation of this life-threatening tumor. We describe the fourth case of aortic angiosarcoma in the literature with initial presentation in the skin.

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.

A 62-year-old woman was referred for general fatigue, left lower limb pain, and increased inflammatory blood markers. Computed tomography (CT) demonstrated an intraluminal tumor in the distal portion of the thoracic aorta, which was confirmed by magnetic resonance imaging (MRI) with mild en

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.

Fewer than 140 cases of aortic sarcoma have been reported with only 34 classified as angiosarcoma. These rare malignancies most often mimic aortoiliac occlusive or aneurysmal arteriosclerotic disease both clinically and on imaging studies, and the large majority are unexpected and diagnosed pathologically on a surgical specimen or at autopsy [1–5]. A 42-year-old woman who presented with low back pain and claudication was shown by CT and angiography to have infrarenal aortic occlusion. Angiosarcoma was an unexpected pathologic diagnosis based on tissue removed during aortobifemoral bypass surgery. Pre and postoperative CT and angiography are presented along with a review of the literature.

We report two patients in whom angiosarcoma of the aorta (ASA) with distal emboli and skin metastases was initially clinically misdiagnosed as polyarteritis nodosa (PAN). Both presented with features highly suggestive of systemic necrotizing vasculitis, including constitutional symptoms, orchitis (in one), leg pain with sensory neuropathy, livedo, focal areas of skin necrosis, painful cutaneous nodules demonstrating vasculitis or inconspicuous changes on biopsy, raised acute phase reactants, and transient improvement with systemic corticosteroids. Repeated skin biopsies revealed an atypical endovascular cell proliferation with strong reactivity for factor VIII. Magnetic resonance angiography (MRA) demonstrated an intra-luminal aortic tumour at the infra-renal level. In retrospect, the (PAN)-like features appeared to involve exclusively the lower part of the body in these patients.

Primary aortic tumors are rare; only 86 cases of primary aortic angiosarcoma have been reported since its first description in 1873. The diagnosis of aortic angiosarcoma is usually confirmed by postoperative pathologic examination or postmortem study. The authors present a case of primary aortic angiosarcoma with postresection recurrence.

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.

Aortic aneurysms and stenosis are widely treated with endovascular procedures in the current era. In this report, we present endovascular stent grafting for symptomatic treatment of severe thoracic aortic stenosis caused by an aortic sarcomatoid carcinoma in a 73-year-old female. The peri- and post-operative courses were complicated with tumor embolisms that were managed by both endovascular and surgical measures.

Primary angiosarcoma of the aorta is a rare malignancy that is characterized by rapid proliferation and propensity for metastasis. It has been reported only 35 times in the surgical literature. This case report presents a 66-year-old man diagnosed with angiosarcoma of his native aorta 7 years after endograft repair of an abdominal aortic aneurysm. We then reviewed the world surgical literature for occurrence, tumorigenic studies, prognosis, and management of aortic angiosarcoma. Because native aortic tissue is retained after endovascular repair of an abdominal aortic aneurysm, the treating physician should have an awareness of this pathology and entertain the diagnosis as appropriate.

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.

A 46-year-old woman died from massive bowel infarction. At autopsy, a primary sarcoma was found growing along the intimal surface of the aorta at the level of the celiac axis. Tumor emboli were found in distal aortic branches and most abdominal organs. Immunoperoxidase for Factor VIII and electron microscopy (EM) did not support an endothelial origin. EM showed myofibroblastic differentiation. Review of the literature yields an array of diagnostic histologic terms for these tumors, hampering case comparison. The literature does suggest, however, that the clinical presentation of these rare neoplasms correlates nicely with the location and gross morphology of the lesion. We therefore propose a clinicopathologic classification, categorizing the lesions as intimal (obstructive and nonobstructive) and mural. The former are typically pleomorphic sarcomas and are probably of myofibroblastic origin, whereas the latter are usually leiomyosarcomas or fibrosarcomas that probably originate in the media or adventitia.