Vascular Malignancy Research Articles

Primary and secondary angiosarcomas: a comparative single-center analysis.

Background Angiosarcomas (AS) are rare vascular malignancies. They are subdivided into primary (PAS) and secondary angiosarcomas (SAS). The objective was to compare the characteristics of AS subtypes.MethodsEighteen PAS and ten SAS patients treated at our institution between 2004 and 2012 were included in this study.ResultsMedian age of PAS and SAS patients was 52.9 and 64.2 years, respectively (p = 0.1448). The percentage of women was 27.8% for PAS, but 80.0% for SAS (p = 0.0163). While PAS occurred throughout the body, the majority of SAS arose from the breast (p = 0.0012). All SAS were radiation-induced with a median latency of 7.7 years. The majority of patients with PAS and SAS underwent surgery as primary or recurrence treatment (p > 0.95). Local recurrence was developed by 27.8% of PAS and 50.0% of SAS (p = 0.4119). 61.1% of PAS metastasized, but only 40.0% of SAS (p = 0.4328). Median overall survival for PAS and SAS was 19 and 57 months, respectively (p = 0.2306).ConclusionRadical surgery remains the mainstay of both primary and recurrence treatment. SAS show a high local recurrence rate, while PAS tend towards developing early metastases. Overall, prognosis is poor for both groups.

Causes of death and mortality and evaluation of prognostic factors in patients with severe aortic stenosis in an aging society

BackgroundSevere aortic stenosis (AS) is now predominantly a disease of the elderly, with significant mortality and morbidity. In order to investigate the burden of severe AS in the current population, we assessed mortality, causes of death, clinical event rates, and prognostic factors of patients diagnosed with severe AS. MethodsA total of 519 consecutive patients (mean age, 78±9 years) with severe AS (aortic valve area <1.0cm2) were retrospectively analyzed. All-cause mortality and clinical events including aortic valve replacement, heart failure requiring admission, acute coronary syndrome, and syncope were measured as main outcome. ResultsDuring a median follow-up of 3.5 years, 167 patients (32%) died. Overall survival rates at 1 and 3 years were 86% and 70%, respectively. Of all deaths, 101 (61%) were cardiovascular-related and 56 (33%) were non-cardiovascular. Syncope occurred in only 18 (4%) patients, while heart failure requiring admission occurred in 188 (43%) patients as the most frequent event. Male, severe symptoms (New York Heart Association functional class, III/IV), inactive state, previous history of heart failure, renal insufficiency, hemodialysis treatment, peripheral vascular disease, malignancy, and statin use at enrollment were significantly and independently associated with death among the patients. ConclusionsAmong the one-third of severe AS patients who died during follow-up, 61% of deaths were cardiovascular-related. Cardiovascular death may be the leading, but not the only, cause of death for contemporary severe AS patients. Factors such as severe symptomatic status, lower daily activity level, and chronic kidney diseases were strong predictive factors of worse survival in this population.

Can c-myc amplification reliably discriminate postradiation from primary angiosarcoma of the breast?

PurposeBreast angiosarcomas are rare vascular malignancies that arise secondary to irradiation or de novo as primary tumours. The aim of this study is to know whether c-myc amplification can reliably discriminate these two entities. Materiel and methodsForty-seven patients treated for breast angiosarcomas were studied. Thirty-two patients were diagnosed with postradiation angiosarcomas after breast cancer treatment and 15 patients with primary angiosarcomas. Interphase fluorescence in situ hybridization (FISH) was performed by hybridization of probes covering C-MYC (chromosome 8q24.21) and CEP8 on tissue sections. ResultsAmplification (5- to 20-fold) of the c-myc oncogene was found in all breast radiation-induced angiosarcomas (32 tumours) but in none of the 15 primary angiosarcomas except one (7%). ConclusionThis study reinforces that there are true pathogenetic differences between the two types of breast angiosarcomas which are morphologically indistinguishable. These data point the pathways preferentially involved in the pathogenesis of post radiation angiosarcomas of the breast and may provide the basis for an additional targeted therapy.

Influencia del perfil clínico del donante, receptor y tiempo de isquemia en la supervivencia del trasplante cardíaco. Subanálisis del Registro Español de Trasplante Cardíaco

Background and aimHeart organs should be prioritized to patients who will obtain the maximum survival with the least morbidity. The objective of the study was to analyze the impact of recipient, donor, and organ ischemia time on mortality. MethodsAll heart transplants (HTx) performed in Spain until December 2011 (6,528 HT) were included. The following were excluded: 1) Transplants prior to 2000, 2) Age <16 years, 3) Combined with other organs, and 4) Re-transplants. A total of 3,006 patients were analyzed. The categories of optimal/suboptimal were defined for the variables of recipient, donor, and ischemia time. Survival curves were constructed and compared for donor, recipient, ischemia time, and combinations of these 3 variables. ResultsThe percentage of patients who died was compared. The differences (P<.05) showed: 1) Optimal recipient: Age<60 years, BMI<30, no diabetes, peripheral vascular diseases, malignancy or prior surgery. No renal or hepatic dysfunction. Non-emergency transplant. 2) Optimal donor: Age<40 years, no dobutamine use, dopamine <5μ/kg/min. 3) Optimal ischemia time: <240min. Survival curves showed differences between optimal vs non-optimal (P<.05) and between the different combinations (P<.05). Thus, implantation of an optimal heart in an optimal recipient with an ischemia time <240min showed a probability of survival at 30 days of 94%, and at 1, 5 and 10 years of 87%, 81% and 71%, respectively. ConclusionsHTx survival is influenced by many characteristics of the donors and recipients, as well as ischemia time. The number of variables considered non-optimal will negatively and significantly affect the HTx survival.

Epithelioid hemangioma of the spine: an uncommon cause of spinal cord compression

A 65-year-old female presents to the emergency room with worsening mid-back pain, rapidly progressive numbness starting from below the breasts (T5 sensory level), and bilateral lower extremity weakness. She reported increasing difficulty ambulating and was unsteady on her feet. She had radiating pain into her anterior thighs, occasionally extending into her calves, right greater than left and had new-onset loss of sphincter, bowel and bladder control. Her focused neurologic exam showed quadriceps strength of 4?/5 on the left and 4-/5 on the right. Ankle dorsiflexion was 4-/5 bilaterally. She had a known history of midthoracic back pain for the preceding 4 months. She denied any history of remote or recent spinal trauma. Spinal cord compression was suspected and the initial CT imaging revealed a large lytic lesion at the T7–8 level with involvement of the corresponding ribs. Coronal imaging revealed destruction, expansion and trabeculation of the vertebral bodies of T7–9 and loss of vertebral body height. On axial imaging, there was paravertebral soft tissue extension with central canal narrowing (Figs. 1, 2, 3). An initial differential diagnosis of vascular tumor versus malignancy was considered and a CT-guided biopsy with angiographic embolization of feeding vessels was planned urgently, followed by definitive surgical intervention. She underwent an uneventful biopsy and successful Onyx-18 (eV3, Irvine, California, USA) embolization of feeding vessels (Fig. 3). The post-embolization surgical procedure involved laminectomies from T6 to T9, T7–8 vertebroplasty and partial transpedicular vertebrectomies followed by tumor resection with T4–T11 arthrodesis and posterior spinal fusion (Figs. 4, 5). She regained motor strength within 48 h and near complete resolution of numbness postoperatively. Final pathology was consistent with epithelioid hemangioma (EH).

Dabska tumor of the tongue: A clinicopathological study with confocal laser scanning microscopy

Introduction: Dabska tumor, or papillary intra- lymphatic angioendothelioma (PILA), first described in 1969 by Dabska, is a rare vascular low grade malignancy that usually affects the skin and subcutaneous tissues of infants. case report: We report a case of PILA of the right tongue in a 65 years old male patient presenting as a white- bluish multinodular lesion, measuring 4x3 cm. After wide excision, histological examination was carried out with confocal laser scanning microscopy (cLsM). the histological features seen during optical examination were: thin- walled vascular spaces resembling a cavernous lymphangioma, and the formation of prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. the cLsM analysis highlighted muscle infiltration, and high fluorescence of intraluminal papillary core due to the presence of young thin collagen fibers devoid of cross-links. thus, a final diagnosis of PILA was made. conclusion: confocal laser scanning microscopy analysis could surely facilitate the histological

Solid form of epithelioid hemangioma: a case report.

Epithelioid hemangioma (EH) is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing nodule on the face or digit of a middle-aged woman. 1 Most lesions have a nonspecific nodular appearance with frequent secondary changes such as excoriation and bleeding. Multiplicity is also a common finding. Histopathologically, EH is characterized by a mixture of vascular proliferation and marked mixed inflammatory cell infiltration, and was first de scribed by Wells and Whimster 2 as angiolymphoid hyperplasia with eosinophilia. The proliferation of vascular structures lined with prominent endothelial cells is a distinguishing feature of EH. The epithelioid endothelial cells that protrude into the vascular lumen create a characteristic “cobble-stone” or “tombstone” appearance. A chronic inflammatory cell infiltration in cluding lymphoplasma cells and eosinophils is a consistent find ing. EH has a diverse range of microscopic features, depending on the composition and distribution of the vascular and inflam matory cellular components. EH has been described in the literature as an inflammatory angiomatous nodule or, an atypical or pseudopyogenic granuloma, 3,4 when infiltration of various inflammatory cells is predominant, and a histiocytoid hemangi oma 5 when cobble stone-like endothelial cells are conspicuous. Some cases of EH consist entirely of solid sheets of epithelioid to spindled cells without fully canalized vascular structures. The solid form of EH can be difficult to diagnose and is occa sionally misdiagnosed as a malignant vascular tumor. EH is a benign neoplasm and surgical excision is sufficient for its treat ment. Recently, we experienced a solid form of EH with no inflammatory component, which showed a dramatic change into the typical morphology on the consecutive biopsy. This is the first reported Korean case of a solid form of EH, which patholo gists should include in the differential diagnosis of epithelioid vascular lesions to avoid overdiagnosis of epithelioid vascular malignancies.

Amputation stump: Privileged harbor for infections, tumors, and immune disorders

The stump site of amputees presents a fragile cutaneous landscape that is prone to skin disease due to many factors. Amputations damage cutaneous, nervous, musculoskeletal, and vascular structures, and alter lymphatic drainage. This altered skin, when placed in the socket of a prosthesis, must adapt to a humid environment and withstand the compressive and frictional forces for which it is poorly adapted. These factors and any history of vascular disease, diabetes, or malignancy make stump skin more vulnerable to immunologic and tissue breakdown, leading to an area of local immune dysregulation called an immunocompromised district. This term encompasses the idea that stump skin is more prone to dermatologic conditions, such as inflammation, infection, and malignancies. Unlike the volar skin found on the palms and soles, the stump skin is not adapted to withstand the compressive forces generated from the prosthesis during movement, particularly during ambulation. In some cases, skin disease and pain at the stump lead to prosthesis abandonment, which has a negative impact on amputees’ quality of life. Researchers are looking into ways to increase the adaptation and durability of stump skin, thereby decreasing skin breakdown, infections, tumors, and malignancies commonly seen on this vulnerable site. Skin disease continues to be a vexing problem for amputees and those who care for them. This contribution reviews skin disease at the stump site and explores the broader context of the amputee stump as an immunocompromised district.

Ultrasound Molecular Imaging of Secreted Frizzled Related Protein-2 Expression in Murine Angiosarcoma

Angiosarcoma is a biologically aggressive vascular malignancy with a high metastatic potential. In the era of targeted medicine, knowledge of specific molecular tumor characteristics has become more important. Molecular imaging using targeted ultrasound contrast agents can monitor tumor progression non-invasively. Secreted frizzled related protein 2 (SFRP2) is a tumor endothelial marker expressed in angiosarcoma. We hypothesize that SFRP2-directed imaging could be a novel approach to imaging the tumor vasculature. To develop an SFRP2 contrast agent, SFRP2 polyclonal antibody was biotinylated and incubated with streptavidin-coated microbubbles. SVR angiosarcoma cells were injected into nude mice, and when tumors were established the mice were injected intravenously with the SFRP2 -targeted contrast agent, or a control streptavidin-coated contrast agent. SFRP2 -targeted contrast agent detected tumor vasculature with significantly more signal intensity than control contrast agent: the normalized fold-change was 1.6±0.27 (n = 13, p = 0.0032). The kidney was largely devoid of echogenicity with no significant difference between the control contrast agent and the SFRP2-targeted contrast agent demonstrating that the SFRP2-targeted contrast agent was specific to tumor vessels. Plotting average pixel intensity obtained from SFRP2-targeted contrast agent against tumor volume showed that the average pixel intensity increased as tumor volume increased. In conclusion, molecularly-targeted imaging of SFRP2 visualizes angiosarcoma vessels, but not normal vessels, and intensity increases with tumor size. Molecular imaging of SFRP2 expression may provide a rapid, non-invasive method to monitor tumor regression during therapy for angiosarcoma and other SFRP2 expressing cancers, and contribute to our understanding of the biology of SFRP2 during tumor development and progression.

Fever of Unknown Origin in Children: A 6 year- Experience in a Tertiary Pediatric Egyptian Hospital.

Fever of unknown origin (FUO) is among the most conditions which poses challenge in diagnosis. The presence of information on regional patterns of FUO will shorten the time for diagnosis and reduces health services costs. There are almost no previous studies describing the etiology of FUO in children of Egypt or nearby countries. To determine different causes of FUO and the possible diagnostic procedures. Data of patients with FUO, presented to the Infectious Diseases Unit of Mansoura University Children Hospital, were retrospectively collected in a 6 year-period from May 2006 to May 2011. The study included children with a fever of 38.3° C or more documented by a health care provider and for which the cause could not be identified after 3 weeks of evaluation as an outpatient or after a week of evaluation in hospital. Patients were then categorized into 5 groups. 127 patients met the diagnostic criteria. Infectious diseases were the commonest causes of FUO in 46 cases (36.22%) followed by the miscellaneous causes in 38 cases (29.9%). Meanwhile, collagen vascular diseases and malignancy were diagnosed in 13 cases (10.2%) and 10 cases (7.87%) respectively. While, 20 cases (15.75%) remained undiagnosed. Infectious diseases are the commonest cause of FUO. The delay in diagnosis was due to atypical presentations or inappropriate use of antibiotic prior to the referral. Non infectious causes, malignancy and collagen or vascular disorders were diagnosed in rest of the patients. However, about 15% of our patients remained undiagnosed. The diagnosis was established by non-invasive means in more than two-third of the cases.

Active TGF-β signaling and decreased expression of PTEN separates angiosarcoma of bone from its soft tissue counterpart

Angiosarcomas constitute a heterogeneous group of highly malignant vascular tumors. Angiosarcoma of bone is rare and poorly characterized. For angiosarcoma of soft tissue, some pathways seem to be involved in tumor development. Our aim was to evaluate the role of these pathways in angiosarcoma of bone. We collected 37 primary angiosarcomas of bone and used 20 angiosarcomas of soft tissue for comparison. Immunohistochemistry was performed on constructed tissue microarrays to evaluate expression of CDKN2A, TP53, PTEN, BCL2, CDK4, MDM2, cyclin D1, β-catenin, transforming growth factor-β (TGF-β), CD105, phospho-Smad1, phospho-Smad2, hypoxia-inducible factor-1α, plasminogen activator inhibitor type 1 (PAI-1), VEGF, CD117 and glucose transporter–1. PIK3CA was screened for hotspot mutations in 19 angiosarcomas. In nearly 55% of the angiosarcoma of bone, the retinoblastoma (Rb) pathway was affected. Loss of CDKN2A expression was associated with a significantly worse prognosis. No overexpression of TP53 or MDM2 was found, suggesting that the TP53 pathway is not important in angiosarcoma of bone. Angiosarcoma of bone showed highly active TGF-β signaling with immunoreactivity for phospho-Smad2 and PAI-1. Although the phosphatidylinositol 3-kinase (PI3K)/Akt pathway seems to be active in both tumor groups, different mechanisms were involved: 41% of angiosarcoma of bone showed a decrease in expression of PTEN, whereas in angiosarcoma of soft tissue overexpression of KIT was found (90%). PIK3CA hotspot mutations were absent. In conclusion, the Rb pathway is involved in tumorigenesis of angiosarcoma of bone. The PI3K/Akt pathway is activated in both angiosarcoma of bone and soft tissue, however, with a different cause; PTEN expression is decreased in angiosarcoma of bone, whereas angiosarcomas of soft tissue show overexpression of KIT. Our findings support that angiosarcomas are a heterogeneous group of vascular malignancies. Both angiosarcoma of bone and soft tissue may benefit from therapeutic strategies targeting the PI3K/Akt pathway. However, interference with TGF-β signaling may be specifically relevant in angiosarcoma of bone.

Angiosarcoma Associated with a Kasabach–Merritt Syndrome: Report of Two Cases Treated with Paclitaxel

Angiosarcomas are rare, aggressive vascular malignancies of endothelial cell differentiation. Kasabach-Merritt syndrome is a rare condition defined by the association of thrombocytopenia and consumption coagulopathy with specific vascular tumors, such as tufted angioma or kaposiform hemangioendothelioma. We report here two cases of angiosarcomas complicated by a Kasabach-Merritt syndrome and their outcome after treatment with paclitaxel.

Tumor Angiogenesis: A New Source of Pericytes

Glioblastoma stem cells have been reported to directly contribute to the tumor vasculature by endothelial cell differentiation. Interestingly, a recent study demonstrates that glioblastoma stem cells preferentially differentiate into vascular pericytes to support vasculature function and tumor growth.

AB0661 Mortality in patients with systemic lupus erythematosus in a single centre in singapore

BackgroundSLE is associated with an increased risk of death compared to the general population. Survival in SLE patients has improved significantly over the last 20 years with a decrease in...

AB0483 Causes of hospitalisation in behcet’s syndrome over a ten-year period

ObjectivesWe are unaware of previous studies on hospitalisation in Behçet’s syndrome (BS). We surveyed the causes and outcome of hospitalisations among BS patients in a dedicated center.MethodsWe surveyed the records...

Thoracic manifestations of vasculitis

•. The vasculitides are rare and their clinical and imaging findings may be confused with infection, collagen vascular disease or malignancy. •. There is the potential for a delay in diagnosis, and therefore radiologists and physicians must remain alert to the possibility of these diseases, despite their rarity. •. Pulmonary involvement with vasculitis should be considered in the presence of nodules or masses, with or without cavitation, or with diffuse air-space opacity that may represent diffuse alveolar haemorrhage. •. Large vessel vasculitis is commonly picked up as an incidental finding on imaging in a patient being investigated for non-specific signs and symptoms, and therefore aortic or pulmonary arterial wall thickening should be routinely looked for when interpreting a thoracic CT study. The vasculitides encompass a disparate group of symptomatically and radiologically diverse conditions associated with an inflammatory and destructive process affecting the blood vessels. The vasculitis may be primary or idiopathic, or may be secondary to another disease process such as infection, connective tissue disease (such as systemic lupus erythematosus), malignancy and hypersensitivity disorders. Primary vasculitides, which may lead to pulmonary involvement, include Wegener's granulomatosis, Churg Strauss syndrome and Goodpasture's syndrome. Systemic lupus erythematosus may lead to pulmonary and cardiovascular abnormalities within the chest. Abnormalities of the pulmonary arteries and aorta may be seen in conditions such as Takayasu's arteritis and Behçet's disease. The vasculitides are all relatively uncommon and their clinical and radiological signs and symptoms may be confused with infection, collagen vascular disease and malignancy. There is consequently a risk of delay in diagnosis. The radiologist should be alert to the possibility of vasculitis in undiagnosed chest problems. Radiological findings include nodules with cavitation (which suggest Wegener's granulomatosis) and air-space opacity—particularly ground-glass opacity, which may represent diffuse alveolar haemorrhage, a feature of several of the vasculitides. The patient is best served by a multidisciplinary approach to diagnosis and management.

Metastatic epithelioid angiosarcoma to the mandible: report of a case and review of the literature

Angiosarcoma and its epithelioid variant are vascular malignancies that rarely affect the facial skeleton. Epithelioid angiosarcoma resembles carcinoma and can be difficult to diagnose. A case is presented of metastatic epithelioid angiosarcoma to the mandible from an angiosarcomatoid portion of renal carcinoma. The diagnostic challenge is outlined and the literature is reviewed.

Vulvar Vascular Tumors

The subepidermal hormonally sensitive tissue of the vulva is anatomically unique and may give rise to a wide variety of vascular tumors. As a consequence, classifying vulvar vascular lesions has been challenging due both to the wide variety of lesions that may be encountered and the heterogeneity in reporting across several disciplines. The purpose of this study is to present an institutional experience of vulvar vascular lesions. Overall, 85 patients were identified over a 26-year period. Vascular lesions belonging to the following classes included (n, %total) benign vascular tumors (32, 38%), dilatations of preexisting vessels (31, 36%), hyperplasia/reactive (7, 8%), tumors with significant vascular component (11, 13%), malformations (3, 4%), and malignant vascular tumors (1, 1%). Two reaction patterns based on vulvar lymphatic pathology were identified: one is a stromal dominant pattern and the other is a vascular dominant pattern. Vulvar vascular malformations and true vascular malignancies, although rare, may have associated high morbidity. To accurately classify vulvar lymphatic lesions, the pathologist must carefully consider the patient's clinical history taking into account features such as preexisting lymphedema.

Haemophagocytic Lymphohistiocytosis

Haemophagocytic lymphohistiocytosis is a rare macrophage related hyperinflammatory disorder characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections collagen vascular disease and malignancies. The pathological hallmark of this disorder is aggressive proliferation of macrophages and histiocytes. The symptoms are due to high levels of cytokines secreted by immune effector cells which display functional defects. Recognizing the morphologic changes in the liver and the immunophenotypic feature of the infiltrate are critical for a rapid diagnosis and prompt initiation of treatment.We report a case of haemophagocytic lymphohistiocytosis as it is one of the emerging infectious diseases and review current literature.

In-hospital mortality for pulmonary embolism: relationship with chronic kidney disease and end-stage renal disease. The hospital admission and discharge database of the Emilia Romagna region of Italy

The impact of chronic kidney disease (CKD) on the outcome of acute pulmonary embolism (PE) is uncertain. We aimed to evaluate the effect of renal dysfunction (defined by ICD-9-CM codification) on in-hospital mortality for PE. We considered all cases of PE (first event) recorded in the database of hospital admissions for the Emilia-Romagna region, Italy, from 1999 to 2009. The inclusion criterion was the presence, as a main discharge diagnosis, of acute PE codes according to ICD-9-CM. Diagnoses of immobilization, dementia, sepsis, skeletal fractures, hypertension, heart failure, myocardial infarction, diabetes mellitus, peripheral vascular disease, cerebrovascular disease, chronic pulmonary disease, pneumonia, malignancy, CKD and end-stage renal disease (ESRD) were also considered to evaluate comorbidity. The outcome was in-hospital mortality for PE, and multivariate logistic regression analyses was performed. We considered 24,690 cases of first episode of PE. In-hospital mortality for PE was not different in patients without renal dysfunction, with CKD, or ESRD (23.6 vs. 24 vs. 18 % p = ns). In-hospital mortality for PE was independently associated with age (OR 1.045, 95 % CI 1.042-1.048, p < 0.001), female sex (OR 1.322, 95 % CI 1.242-1.406, p < 0.001), hypertension (OR 1.096, 95 % CI 1.019-1.178, p = 0.013), diabetes mellitus (OR 1.120, 95 % CI 1.001-1.253, p = 0.049), dementia (OR 1.171, 95 % CI 1.020-1.346, p = 0.025), peripheral vascular disease (OR 1.349, 95 % CI 1.057-1.720, p = 0.016) and malignancy (OR 1.065, 95 % CI 1.016-1.116, p = 0.008). Age and comorbidity are associated with in-hospital mortality for PE, whereas CKD does not appear to be an independent predictor of adverse outcomes in patients hospitalized for PE.