Popliteal Angiosarcoma after Bypass with Autologous Saphenous Vein.

Angiosarcoma (AS) is a type of malignancy within sarcomas affecting soft tissue and bone. It presents a very heterogeneous distribution in the human body, and it can appear in multiple locations, from breast to liver or skin. The incidence of this type of tumor is low; however, it is aggressive and requires an early diagnosis to initiate the treatment as soon as possible. In the present article, we report the case of a male patient who was diagnosed with epithelioid AS of popliteal artery after an aneurysm popliteal surgery, as well as its treatment and evolution. This kind of tumors has controversial treatment and poor survival rates in the early years.

Angiosarcoma of a Thrombosed Arteriovenous Fistula in a Renal Transplant Recipient.

Angiosarcomas developing in nonfunctioning arteriovenous fistulas in renal transplant recipients are quite rare clinical entities with very poor prognosis. Herein we present a 60-year-old male who developed an angiosarcoma in a thrombosed radiocephalic fistula 6 years after renal transplantation. The patient presented with pain and swelling at the site of a previously asymptomatic fistula. The fistula was excised and diagnosis was made by histology. Despite radical surgery with an above-elbow amputation, the patient died of metastatic disease 6 months later.

Development of Angiosarcoma in a Saphenous Vein Graft after Femoral to Above-Knee Popliteal Artery Bypass.

Angiosarcoma is an exceedingly rare and aggressive neoplasm. Owing to early metastasis even in low-grade tumors, it is associated with poor survival. We report a late development of an angiosarcoma in a saphenous vein graft after a femoral artery to above-knee popliteal artery bypass performed for disabling claudication. Chronic inflammation, lymphatic disruption, and thrombosis of the graft may have contributed to the malignant transformation, similar to cases described as occurring after arteriovenous fistulae creation. This case illustrates that angiosarcoma may be a rare, late complication of autogenous vascular bypass.

Intravascular lymphoma forming massive aortic tumors complicated with sarcoidosis and focal segmental glomerulosclerosis: a case report and literature review.

BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by proliferation of B cells within small vessels. Herein, we report a case of a 77-year-old man who presented with IVLBCL and massive tumor formation on the aortic wall who was previously diagnosed with sarcoidosis and focal segmental glomerulosclerosis (FSGS). To our knowledge, this is the first reported case of an IVLBCL with aortic tumor formation. CASE PRESENTATION: A 77-year-old ambulatory man with sarcoidosis and FSGS had neurological symptoms for nine months. The patient presented to the emergency department with sudden left leg pain, and was diagnosed with acute femoral artery occlusion. Emergency thrombectomy was performed subsequently. Pathological evaluation of the thrombi revealed that its surface was filled with large atypical B cells. Bone marrow biopsy showed infiltration of large atypical B cells within the small vessels. IVLBCL was suspected and further examination was planned, but the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. Autopsy revealed intravascular tumors adherent to the aortic arch, left ventricle, and the abdominal aorta. All enlarged lymph nodes and the ventricular septum of the heart showed hyalinized lesions with granular formation consistent with sarcoidosis. The patient was diagnosed with IVLBCL with aortic tumor formation complicated with sarcoidosis and FSGS. CONCLUSIONS: IVLBCL may present with tumor formation on the aortic wall. Although the cause of its affinity to the aortic wall is yet unknown, autopsy findings imply that arteriosclerosis may have contributed to the tumor formation. The literature suggests that T-cell abnormalities could possibly be the common etiology of intravascular lymphoma, sarcoidosis, and FSGS.

Perigraft hygroma - a rare cause of post-EVAR aneurysm growth.

Development of perigraft hygromas following repair of abdominal aortic aneurysms is extremely rare. A case is presented of a patient who was found to have a large hygroma despite two re-interventions on a previous EVAR.

"Caval Reconstruction for Lower-Extremity Sarcoma Metastasis Trapped within Inferior Vena Cava Filter".

This report describes the management of a hematogenously spread metastasis from a lower-extremity sarcoma found trapped within an inferior vena cava (IVC) filter. Although endovascular techniques for treating thrombosed IVC filters are successful in a majority of cases, the malignant nature of this lesion required a novel approach. In this unique case, the segment of infrarenal IVC with the thrombosed filter was resected and reconstruction performed with an interposition prosthetic graft. There were no early or late complications, and the patient remains clinically free of recurrence at 24-month follow-up.

Aortic Epithelioid Angiosarcoma after Endovascular Aneurysm Repair.

We report a case of epithelioid angiosarcoma of the abdominal aortic wall after endovascular treatment for abdominal aortic aneurysm (EVAR). A 60-year-old male, treated 7 years before with EVAR, presented with abdominal back pain, general fatigue, and fever. It was assumed to be a graft infection with periaortic tissue compatible with an inflammatory reaction. The endograft was therefore completely removed and a Dacron silver aorto-bisiliac graft was implanted. After a few days the patient worsened, the angio-computed tomography scan showed a progressive increase of the periaortic mass and numerous small nodules in the abdomen were also detected. The patient was again brought to surgery, an axillo-bifemoral bypass was performed, and the aorto-bisiliac graft was removed but the patient died after surgery. The histological examination showed an aortic epithelioid angiosarcoma with peritoneal metastasis.

Intravascular Myopericytoma of the Plantar Region: Case Report and Discussion of the Probable Origin From a Cutaneous Vascular Malformation.

Myopericytoma is a perivascular myoid neoplasm of skin and soft tissues characterized by numerous thin-walled blood vessels surrounded concentrically by round to spindle myoid tumor cells, which shows α-smooth muscle actin and h-caldesmon coexpression and commonly negativity for desmin. These tumors arise predominantly in extremities of adult patients with benign clinical course. Based on the architectural pattern, there are various histologic variants as classical-solid myopericytoma, hemangiopericytoma-like myopericytoma, angioleiomyoma-like myopericytoma, myofibroma-like myopericytoma, hipocelular fibroma-like myopericytoma, intravascular myopericytoma, cellular immature myopericytoma, and malignant myopericytoma. The authors report a case that fully satisfies the morphological and immunohistochemical criteria for intravascular myopericytoma, which plantar location is not previously described in the literature. In addition, the authors discuss about its possible development from a preexistent cutaneous vascular malformation.

Implication of microscopic and macroscopic vascular invasion for liver resection in patients with hepatocellular carcinoma.

BACKGROUND AND AIM: Long-term data after liver resection for hepatocellular carcinoma (HCC) with vascular invasion are rare for non-Asian patients. The aim of the present study was to analyze the long-term outcome of liver resection for HCC with vascular invasion and to compare the results of subgroups with micro- and macrovascular invasion. METHODS: From January 2000 to September 2010, 288 patients without extrahepatic metastases underwent liver resection for HCC. In 107 out of 288 patients (37%), vascular invasion was found in the final pathological analysis. The long-term outcome as well as the perioperative course of these patients was analyzed using a prospective database. RESULTS: The 1-, 3- and 5-year cumulative survival rate of HCC patients with vascular invasion was 64.3, 41.4 and 23.9%, respectively. The median survival was 19 months. In the multivariate analysis, the overall survival was not influenced by the type of vascular invasion (micro- vs. macrovascular invasion), however overall survival was significantly impaired in case of lymphatic vessel invasion, intraoperative blood transfusions, need of fresh frozen plasma application, prolonged ICU stay and elevated preoperative bilirubin levels. CONCLUSIONS: Acceptable survival rates can be achieved in selected patients with macrovascular invasion after surgical resection, which is not markedly different from those with microvascular invasion. In view of an otherwise poor prognosis, liver resection seems to be justified for selected HCC patients with macrovascular invasion, although this stands in contrast with the BCLC recommendations. However, it is in accordance for example with the guidelines of the Asia-Pacific Association for the Study of the Liver.

Angiosarcoma arising in an arteriovenous fistula in a patient without kidney transplant.

Angiosarcomas are relatively rare and account for only 1% of all sarcomas. They arise from endothelial cells of blood or lymph vessels. They are usually highly aggressive and long term outcome is poor with an overall 5-year survival rate of 10-20%. We report the case of a 80-year old man with an angiosarcoma arising in a non-functioning arteriovenous fistula. Angiosarcomas arising in an arteriovenous fistula are very rare and only eleven cases were found in the literature. In nine cases (82%) chronic immunosuppression, taken for renal transplant, was one of the causing factors. Our patient however did not receive a kidney transplant and was not on immunosuppressive therapy. Clinicians should be aware that an angiosarcoma can arise in an arteriovenous fistula even without chronic immunosuppression.

Unusual myoid, perivascular, and postradiation lesions, with emphasis on atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelial tumors, myopericytoma, and perivascular epithelioid cell tumor.

In recent years, a number of new soft tissue tumor entities have been described that occur in the skin only, or that also occur in other sites but form clinically and pathologically distinct subsets when arising in the skin and subcutaneous tissue. These include a variety of mesenchymal lineages and have variable malignant potential, although superficial malignant soft tissue tumors generally have a more favorable outcome than their more deeply located counterparts. This article reviews the clinical and pathologic features and differential diagnoses of atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelioma, myopericytoma, and perivascular epithelioid cell tumor.

A case of intravascular epithelioid hemangioendothelioma occurring 14 years after coil embolization for an extracranial internal carotid artery aneurysm.

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm originating from various organs. The clinical outcome mostly depends on surgical resectability. The authors report an EHE of the extracranial internal carotid artery developed in a 59-year-old male patient 14 years after the intravascular coil embolization for a carotid aneurysm at the same site. Because the lesion was initially diagnosed as regrowth of the thrombosed aneurysm, decision for radical resection was delayed, and the patient died from rapid tumor progression. Differential diagnosis of atypical vascular mass lesions should include neoplasm, because initial radical resection may be the key to achieve a better prognosis.

Intimal sarcoma in an inflammatory aneurysm after endovascular aneurysm repair.

Long-term outcomes after endovascular aneurysm repair (EVAR) for inflammatory aneurysms are unknown. We present a young patient with new-onset back pain and failure to thrive 6 years after EVAR for an inflammatory abdominal aortic aneurysm (AAA). Endograft explanation was performed with a presumed diagnosis of infection. Pathology revealed intimal sarcoma in the excluded aneurysm sac with liver metastasis. This report presents a detailed review of literature regarding potential association of prosthetic implantation and carcinogenesis.

Disseminated angiosarcoma of the dialysis fistula in 2 patients without kidney transplants.

Angiosarcoma of the dialysis fistula is a rare occurrence. Of the 8 cases of angiosarcoma of the dialysis fistula reported in the literature, all occurred after kidney transplant and long-term immunosuppression therapy. We report 2 cases of disseminated angiosarcoma of the dialysis fistula in hemodialysis patients without concurrent kidney transplants or immunosuppression. Both patients presented with symptoms of pain and bleeding at the site of the thrombosed fistula. Clinicians should be aware that angiosarcoma of the dialysis fistula can occur in patients without kidney transplants.

A natural history of aortic aneurysm hygroma.

A 56-year-old man with a family history of aortic aneurysm underwent routine repair in 2003. A postoperative computed tomography scan showed a 6-cm perigraft hygroma. Sudden onset of abdominal pain 12 months later revealed a larger hygroma, with an additional anterior fluid collection suggestive of contained rupture. The bilobed hygroma remained stable until 2010, when he presented with chills and severe abdominal pain. A computed tomography scan demonstrated free rupture of the sister hygroma, with air pockets observed within the sac. Conservative management was elected. Air pockets as well as the hygroma eventually resolved, and the patient remains well.

Angiosarcoma developing in a non-functioning arteriovenous fistula post-renal transplant.

BACKGROUND: Angiosarcomas comprise less than 1% of all sarcomas, arising from endothelial cells of blood or lymph vessels. Chronic immunosuppression increases the risk of many malignancies and an association between the development of angiosarcoma with an immunosuppressed state is established. A few cases have been reported of angiosarcomas arising in the post-renal transplant patient. Specifically, there have been six cases of an angiosarcoma arising in arteriovenous (AV) fistulae in this patient population. We describe a further case and review the relevant literature with specific emphasis on a possible mechanism for the development of angiosarcoma in the post-transplant patient. CASE PRESENTATION: We report the case of a 48-year-old male who developed an angiosarcoma in a ligated native AV fistula. The lesion arose on the background of immunosuppression following a successful ABO-incompatible renal transplant for chronic renal failure. CONCLUSION: Angiosarcomas are extremely rare tumours but should be considered as a differential diagnosis for an evolving mass near the site of an AV fistula. Diagnosis relies on an index of suspicion and obtaining a definitive histological diagnosis. Both clinicians and patients should be aware that an evolving mass within or around an AV fistula should prompt urgent biopsy.

Polyoma middle T-induced vascular tumor formation: the role of the plasminogen activator/plasmin system.

The middle T antigen of murine Polyomavirus (PymT) rapidly transforms endothelial cells, leading to the formation of vascular tumors in newborn mice. Transformed endothelial (End.) cell lines established from such tumors exhibit altered proteolytic activity as a result of increased expression of urokinase-type plasminogen activator (uPA) and are capable of inducing vascular tumors efficiently when injected into adult mice. In this study we have used mice lacking components of the PA/plasmin system to analyze the role of this system in the transformation process and in tumor growth. We found that the proteolytic status of the host is not a critical determinant for PymT-induced vascular tumor formation. In addition, the lack of either uPA or tissue-type PA (tPA) activity is not limiting for the establishment and proliferation of End. cells in vitro, although the combined loss of both PA activities leads to a marked reduction in proliferation rates. Furthermore, the in vitro morphogenetic properties of mutant End. cells in fibrin gels could only be correlated with an altered proteolytic status in cells lacking both uPA and tPA. However, in contrast with tumors induced by PymT itself, the tumorigenic potential of mutant and wild-type End. cell lines was found to be highly dependent on the proteolytic status of both the tumor cells and the host. Thus, genetic alterations in the PA/plasmin system affect vascular tumor development, indicating that this system is a causal component in PymTmediated oncogenesis.

Intravascular lymphoma masquerading as multiembolic stroke developing after coronary artery by-pass surgery.

BACKGROUND: Intravascular lymphoma (IVL) is a very rare non-Hodgkin type lymphoproliferative disorder characterized by neoplastic growth of lymphoid cells within the lumen of capillaries, small veins, and arterioles. The neoplastic cells cannot reach the parenchyma because of the loss of adhesion molecules during malignant transformation. Multifocal vascular occlusions caused by proliferation of malignant lymphocytes in the lumen result in diffuse thrombosis and tissue infarction. The clinical symptoms of the disease are dependent on the specific organ involvement which most often includes the central nervous system and skin. Neurologic presentation includes focal sensory or motor deficits, altered sensorium, rapidly progressive dementia, seizures, ataxia, and vertigo. CASE REPORT: We report a patient with IVL whose symptoms developed on the second postoperative day of coronary artery-bypass surgery imitating a multiembolic stroke. Magnetic resonance imaging showed widespread ischemic subcortical lesions. The patient's clinical status worsened irrespective of supportive medical treatment. The diagnosis was established by autopsy. CONCLUSION: IVL may mimic ischemic stroke. IVL is not often diagnosed before death because of the intravascular growth pattern of the tumor cells and a fulminant clinical course. IVL may be considered in the differential diagnosis of ischemic stroke patients with progressive worsening despite medical management.