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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., (Copyright © 2018. Published by Elsevier Inc.)

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., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: Lymphovascular invasion (LVI) is an important step in bladder cancer cell dissemination. We aimed to perform a systematic review and meta-analysis of the literature to assess the prognostic value of LVI in radical cystectomy (RC) specimens., Patients and Methods: A systematic review and meta-analysis of the last 10 years was performed using the MEDLINE, EMBASE, and the Cochrane libraries in July 2017. The analyses were performed in accordance with the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement., Results: We retrieved 65 studies (including 78,107 patients) evaluating the effect of LVI on oncologic outcomes in patients treated with RC. LVI was reported in 35.4% of patients. LVI was associated with disease recurrence (pooled hazard ratio [HR] = 1.57; 95% CI: 1.45-1.70) and cancer-specific mortality (CSM) (pooled HR = 1.59; 95% CI: 1.48-1.73) in all studies regardless of tumor stage and node status (pT1-4 pN0-2). LVI was associated with recurrence and CSM in patients with node-negative bladder cancer (BC). In patients with node-negative BC, LVI rate increased and was associated with worse oncologic outcome. LVI had a lower but still significant association with disease recurrence and CSM in node-positive BC., Conclusions: LVI is a strong prognostic factor of worse prognosis in patients treated with RC for bladder cancer. This association is strongest in node-negative BC, but it is also in node-positive BC. LVI should be part of all pathological reporting and could provide additional information for treatment-decision making regarding adjuvant therapy after RC., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Vascular tumors associated with Kasabach-Merritt phenomenon (KMP) are life-threatening and the mortality is as high as 10-30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid-resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid-resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side-effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants., (© 2018 The Authors. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)

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.

Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) have recently been associated with primary and secondary immunodeficiencies. They are broadly divided into 3 subgroups: HIV-related, posttransplant, and congenital immunodeficiency. Subsequent to organ transplantation and acquired immunosuppression, a few cases of EBV-associated SMTs have been described in the liver, respiratory tract, and gastrointestinal system. To the authors' knowledge, intracranial involvement after peripheral blood stem cell transplantation has never been reported previously. The authors describe the case of a 65-year-old woman who presented with recent-onset painful ophthalmoplegia. She had a prior history of acute myelogenous leukemia requiring allogenic peripheral blood stem cell transplantation 2 years earlier, but she was in a remission phase. Imaging revealed a T1/T2 isointense, homogeneously enhancing lesion of the left cavernous sinus. A presumptive diagnosis of Tolosa-Hunt syndrome was made, and she was treated with steroids; however, her symptoms progressed quickly and repeat imaging revealed that the lesion was growing. To rule out leukemic deposits, a minimally invasive lateral orbitotomy extradural transcavernous approach was performed for biopsy sampling and debulking of the lesion. The biopsied tumor tissue was found to be infiltrative, grayish, firm, and moderately vascular. The final pathology results indicated an EBV-associated SMT of the cavernous sinus. Subsequently, the patient's steroid treatment was stopped and she had obtained partial symptomatic relief at her last follow-up visit, 3 months after surgery. EBV-associated SMT should be included in the differential diagnosis for intracranial and dural-based central nervous system lesions, especially in immunocompromised patients. Paradoxical response to steroids with worsening of symptoms is a hallmark of EBV-associated SMTs.

Context: Overall, 4-10% of patients with renal cell carcinoma (RCC) present with venous tumour thrombus. It is uncertain which surgical technique is best for these patients. Appraisal of outcomes with differing techniques would guide practice., Objective: To systematically review relevant literature comparing the outcomes of different surgical therapies and approaches in treating vena caval thrombus (VCT) from nonmetastatic RCC., Evidence Acquisition: Relevant databases (Medline, Embase, and the Cochrane Library) were searched to identify relevant comparative studies. Risk of bias and confounding assessments were performed. A narrative synthesis of the evidence was presented., Evidence Synthesis: The literature search identified 824 articles. Fourteen studies reporting on 2262 patients were included. No distinct surgical method was superior for the excision of VCT, although the method appeared to be dependent on tumour thrombus level. Minimal access techniques appeared to have better perioperative and recovery outcomes than traditional median sternotomy, but the impact on oncologic outcomes is unknown. Preoperative renal artery embolisation did not offer any oncologic benefits and instead resulted in significantly worse perioperative and recovery outcomes, including possibly higher perioperative mortality. The comparison of cardiopulmonary bypass versus no cardiopulmonary bypass showed no differences in oncologic outcomes. Overall, there were high risks of bias and confounding., Conclusions: The evidence base, although derived from retrospective case series and complemented by expert opinion, suggests that patients with nonmetastatic RCC and VCT and acceptable performance status should be considered for surgical intervention. Despite a robust review, the findings were associated with uncertainty due to the poor quality of primary studies available. The most efficacious surgical technique remains unclear., Patient Summary: We examined the literature on the benefits of surgery to remove kidney cancers that have spread to neighbouring veins. The results suggest such surgery, although challenging and associated with high risk of complications, appears to be feasible and effective and should be contemplated for suitable patients if possible; however, many uncertainties remain due to the poor quality of the data., (Copyright © 2015 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

Masson tumor (MT, papillary endothelial hyperplasia) is an exaggerated form of thrombus reorganization rarely occurring in the central nervous system (CNS), where it presents as a mass or hemorrhage in parenchyma, meninges, or venous sinuses. MT is subclassified as type 1 arising within a histologically normal vessel, type 2 associated with a ruptured vascular malformation, and extravascular. Limited reports of CNS MT after radiosurgery, or especially external radiation therapy, have emerged. We searched our databases for cases reported from 2008 to present. Nine cases were identified, 6 of which were associated with receipt of therapeutic radiation for known lesions, with intervals of 1 to 25+ years to MT development (4 neoplasms=external beam radiation; 1 neoplasm=external beam radiation+radiosurgery, 1 arteriovenous malformation=radiosurgery). MTs were coassociated with radiation-induced vascular malformations (1 cavernoma-like, 1 massive) only in 2 of 6 irradiated patients, whereas the other 4 had MTs only. The 3 MTs in nonirradiated patients were extravascular, with 1 spontaneously developing in a hemangioblastoma. Seven of 9 MTs were intracerebral, 1 was within the spinal cord, and 1 was subdural. Papillary MT architecture was best appreciated by CD31 or CD34 immunohistochemistry, although ERG verified the endothelial monolayer population. Most CNS MTs at our institution have arisen in patients who have received therapeutic cranial radiation, many of whom received only external beam radiation. Although MTs could conceivably represent early, severe phases in radiation-induced cavernoma development, most were not found coassociated with the latter. This study further extends our knowledge of types of radiation-induced CNS vascular abnormalities.

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The saphenous nerve is the terminal branch of the femoral nerve and a pure sensory nerve that provide sensation to medial leg. Injury to saphanous nerve following trauma or surgery of the knee can result in formation of a painful neuroma along its distribution. We present a case of saphenous neuroma following use of an ankle-foot orthosis (AFO) in a patient with paraplegia. A 36-year-old patient with paraplegia who was capable of walking independently with his AFO presented to our department with a 3-month history of pain in his left calf. Examination revealed tenderness, paresthesias and positive Tinel sign over the anteromedial aspect of the calf. Ultrasonographic examination of the painful area showed a mass with heterogenous echogenity which was consistent with a saphenous neuroma at the site where fastener band of AFO compressed to skin. We performed a nerve block with steroid and local anesthetic injection under ultrasound guidance to the neuroma. The patient reported pain relief following injection. The use of the AFO may cause a painful saphenous neuroma which is an unusual cause of extremity pain in patients with paraplegia. Ultrasound may be a beneficial diagnostic tool and a guidance for the therapeutic interventions in this condition.

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.

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.

Primary vascular neoplasms are rare entities. They were first described as arising spontaneously in the aorta and other vessels. However, in the past several decades, a number of systemic artery-derived vascular neoplasms, mostly sarcomas, have been reported as arising in intimate association with synthetic grafts. We describe two additional cases of intimal sarcoma seen at our institution. The first is an invasive intimal sarcoma detected in a thoracoabdominal aortic aneurysm at the time of surgical intervention. The second is a superficial spreading intimal sarcoma associated with a Dacron-coated graft, in place for 9 years, detected when the graft was replaced. When the patient died 3 months later, a metastatic subcutaneous sarcomatous lesion was detected at autopsy. In these cases, we studied selective molecular pathways that may be involved in the transformation of benign endothelium to malignant endothelium, with implications for possible therapeutic targets. These cases are presented in order to contribute additional data to the literature involving these vascular neoplasms and to potentially provide a spectrum of disease seen in the vasculature tissues that may arise spontaneously or after placement of a synthetic graft., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Total hip arthroplasty with metal-on-polyethylene implants and metal-on-metal articulations have been an area of recent interest. This article describes a patient with a persistent small soft tissue mass on the anterior groin following total hip arthroplasty with negative findings on infection workup, including blood work and aspiration. Subsequent biopsy findings were consistent with organizing thrombus and necrosis. It then developed into an expansile soft tissue groin mass with significant femoral and pelvic bony destruction and soft tissue infiltration. Metallosis, the formation of a pseudocapsule, and aseptic lymphocyte vasculitis-associated lesions have also been an area of interest following adult hip reconstruction. Occasionally, the formation of subsequent lesions make revision surgery impossible to perform due to bony destruction and soft tissue limitations. Although few in number, most studies or cases that have linked metal-on-metal implants to pseudotumors have been treated with revision surgery and replacement of articulating surfaces. This case highlights a metal-on-polyethylene total hip arthroplasty with a mass presenting with pseudotumor-like characteristics. However, final histology demonstrated fibroadipose tissue with fat necrosis and fibrosis, thickened vascular vessels, and diffuse chronic inflammation with lymphocytic infiltrates, which are more consistent with an atypical inflammatory vascular tumor. The current case illustrates treatment difficulties and provides surgical options for when this event occurs. Although aseptic lymphocyte vasculitis-associated lesions have been observed with metal-on-polyethylene articulations, the multiorgan involvement in this case is unique. In this case, radical excision with hemipelvectomy and complex flap closure was a last resort treatment solution that staved off the prospect of patient mortality., (Copyright 2013, SLACK Incorporated.)

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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Infantile hemangiomas (IHs) are the most common vascular tumors of childhood, affecting ~5% of all infants. Although most lesions proliferate and then involute with minimal consequence, a significant minority can be disfiguring, functionally significant, or, rarely, life-threatening. Recent discoveries concerning hemangioma pathogenesis provide both an improved understanding and more optimal approach to workup and management. Important detrimental associations can be seen with IH, such as significant structural anomalies associated with segmental IH. Standards of care have dramatically changed evaluation and management of hemangiomas. The goal of timely recognition and therapy is to minimize or eliminate long-term sequelae. New modalities, such as oral propranolol, provide the caregiver with better therapeutic options, which can prevent or minimize medical risk or scarring, but the side effect profile and risk-benefit ratio of such interventions must always be evaluated before instituting therapy.

Soft tissue tumors in very young children pose diagnostic and therapeutic challenges. Vascular tumors are the most prevalent soft tissue neoplasms in the neonatal period. They are generally benign tumors, but may exhibit aggressive behaviour and cause life-threatening complications. Fibroblastic tumors of intermediate prognosis, more prevalent in very young children (especially infantile fibrosarcoma), are locally aggressive. Since metastases are unusual in this group of tumors, complete surgical resection is generally curative. However, these tumors often present a therapeutic challenge because of the location which makes complete surgical resection difficult. Among the malignant soft tissue tumors, rhabdomyosarcoma is most frequent. It is an aggressive high-grade tumor, with local invasiveness and a propensity to metastasize. These tumors respond to chemotherapy and radiotherapy. Neonates with rhabdomyosarcoma seem to have a worse prognosis than in older age groups. This may be a result of inappropriate dosing of chemotherapeutic agents and decreased use of radiation therapy among other factors., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

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., (Copyright © 2012 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

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., (Copyright © 2012 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

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., (Copyright © 2011 Society for Vascular Surgery. All rights reserved.)

We report a 58-year-old man who had a multi-lobulated pseudomyxomatous lesion in his ascending aorta 6 months after his root and ascending aorta was replaced by a Dacron graft., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2011 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

A hallmark of infantile hemangioma, the most common tumor of infancy, is its dramatic growth after birth, by diffuse proliferation of immature endothelial cells, followed by spontaneous regression. The growth and involution of infantile hemangioma is quite different from other vascular anomalies, which do not regress and can occur at any time during life. Some hemangioma lesions can be extremely disfiguring and destructive to normal tissue and may even be life-threatening. Unfortunately, existing therapeutic approaches have limited success and significant adverse effects of some treatment modalities limit their use. Better understanding of the pathogenesis of hemangioma will enable the development of better therapeutic strategies. Herein, we review recent studies and new hypotheses on the pathogenesis of the tumor. Detailed mechanisms of activated vascular endothelial growth factor (VEGF) signaling in tumor cells, identification of their origin and characterization of multipotent stem cells that can give rise to infantile hemangioma are shedding new light on this intriguing vascular tumor., (© 2010 Japanese Dermatological Association.)

Giant cell arteritis, Takayasu arteritis, and Horton disease are rare, idiopathic diseases that cause chronic inflammation and obliteration of large arteries, mainly the aorta and its major branches. Histological examination reveals multinucleated giants cells and clinical presentation is characterized by general symptoms and/or symptoms related to stenosis or occlusion of vessels. A case of a 50-year-old woman with neurological symptoms, cervicothoracic tumour with severe stenosis of the right subclavian artery and complete occlusion of common carotid artery is presented.

Angiosarcoma is a rare neoplasm comprising 1 to 2% of soft tissue sarcoma. This tumor has been associated with previous irradiation, exposure to toxins and the presence of foreign bodies. A case report of an epithelioid angiosarcoma that developed at the site of an arterial femoro-popliteal bypass using autologous vein is described. The initial presentation looked like a painful popliteal cyst. Chronic fibrosis secondary to the thrombosis could play a role in the tumorogenesis of this uncommon tumor. This case illustrates that sarcoma may be a late complication of vascular bypass and may have a rheumatologic presentation., (Copyright 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Leiomyosarcoma of the renal vein is a rare and malignant tumor difficult to diagnose. No standard treatment has been defined and prognosis is poor. We described the case of a 68-year-old woman with rheumatoid arthritis treated by methotrexate who developed a leiomyosarcoma of the left renal vein with a fatal outcome in less than 1 year. Association of a leiomyosarcoma and rheumatoid arthritis raises the question of a casual association or of a predisposing factor since studies have shown increased risk of cancer with this rheumatism.

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., ((c) 2010 Wiley-Liss, Inc.)

A 72-year-old man was referred to our department with an incidentally diagnosed bronchogenic carcinoma of the right upper lobe. Positron emission tomography (PET) combined with computed tomography (PET-CT) revealed an unexpected hot spot in the ventral wall of the infrarenal segment of the inferior vena cava (IVC). Diagnostic biopsy of this lesion was performed under CT guidance with semiautomated 20G fine-needle aspiration (FNA) through a 19G coaxial needle. Cytology revealed few carcinoma cells, which led to the remarkable diagnosis of a distant metastasis to the IVC wall. Both the immediate postinterventional CT control and the further surveillance period of the patient were unremarkable; in particular, no signs of bleeding complications were detected. We conclude that coaxial FNA of an IVC wall lesion is technically feasible and may even help diagnose distant metastasis.

Essential thrombocythemia is frequently associated with abdominal thrombotic complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype. In the presented case report, the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism. The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.

Racemose hemangioma of the bronchial arteries is a rare abnormality and is characterized by enlarged and convoluted bronchial arteries arranged segmentally along the longitudinal axis of bronchus. Primary racemose hemangioma may arise from inborn malformation, and secondary one may develop following primary inflammatory, stenosing or deforming diseases of the bronchus, the peribronchial tissues and the surrounding lung tissues. The symptom at onset is usually hemoptysis. Although the typical treatments have not been established, various treatments, like embolization or ligation of the bronchial arteries and surgical resection of the involved area of lung, were reported. However the term 'Racemose hemangioma' seems to be used only in Japan, so it is favorable to make a consensus of its definition among countries.

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.

Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and soft tissue. Gastrointestinal and genitourinary vascular malformations in Klippel- Trenaunay syndrome may present with lower gastrointestinal bleeding and hematuria. The majority of patients with splenic hemangiomatosis are asymptomatic. We herein report a case admitted to the Gastroenterology Clinic with life-threatening hematochezia and symptomatic iron deficiency anemia. The patient's history was remarkable for subtotal cystectomy and enterocystoplasty in December 2002 for vascular malformation, located in the bladder, which presented with hematuria. Although the patient was also diagnosed with colonic varices and splenic hemangiomas at that time, due to the asymptomatic mild intermittent hematochezia and splenic hemangiomas, the patient did not seek any help for rectal bleeding until her admittance to our department for evaluation of massive lower gastrointestinal bleeding. Endoscopy revealed vascular malformations starting from the transitional zone in the rectum extending up to the descending colon. Due to this extensive involvement of the rectum and sigmoid colon, no interventional endoscopic procedure was attempted and she was referred to surgery. A very low anterior resection with double stapling technique was done. Postoperative follow-up has been uneventful for six months since the operation. To the best of our knowledge, this is the first Klippel-Trenaunay syndrome case presenting with lower gastrointestinal bleeding, hematuria and splenic hemangiomas. The literature on the evaluation and management of lower gastrointestinal and genitourinary bleeding in Klippel-Trenaunay syndrome is reviewed.

Vascular smooth muscle tumors are very rare. They can be benign or malign. Intravascular leiomyomatosis is a benign neoplasm that extends through the veins and caries significant morbidity. Angioleiomyoma is a benign neoplasm of the extremities that caries minimal morbidity. Vascular leiomyosarcomas are malign neoplasms derived from vascular smooth cells. They are usually localized to the inferior vena cava, but can also arise from the pulmonary arteries or veins or other peripheral vessels. This study reviews literature for epidemiology, clinical presentation, diagnosis and management of patients with vascular smooth muscle tumors.

We report a case of jugular foramen schwannoma associated with tuberous sclerosis. A 28-year-old female with tuberous sclerosis presented to Hikone Municipal Hospital with subacute onset of somnolence, ataxic gait and frequent vomiting. Her tuberous sclerosis was diagnosed at the age of 9, when she developed hydrocephalus caused by subependymal giant cell astrocytoma near the foramen of Monro, which was treated by surgical resection and VP shunt placement followed by radiation and chemotherapy. Brain MR images revealed a 3 cm enhancing mass extending from the left jugular foramen to the cerebellopontine cistern, accompanied by perifocal edema of the brain stem and cerebellar hemisphere. The tumor was partially removed via suboccipital craniotomy and histologically diagnosed as a schwannoma. After surgery her symptoms promptly improved and 4 months later she underwent additional stereotactic radiosurgery. No tumor regrowth has been observed at 94 months. Intracranial schwannoma complicating tuberous sclerosis is very rare. Only one other case has been reported in the literature.

Portal cavernoma is a rare complication of portal vein thrombosis and may be a component of the myeloproliferative hematologic disorders. In the present case report, although the symptoms induced by portal hypertension and portal biliopathy had a concordant evolution with the portal cavernoma, the peripheral thrombocytosis and the medullar expression of essential thrombocythemia became relevant at 18 months.