scholarly journals Hepatic Epithelioid Hemangioendothelioma Presenting as an Enlarging Vascular Lesion within the Spleen

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Juliet A. Emamaullee ◽  
Klaudia Nowak ◽  
Marla Beach ◽  
Julinor Bacani ◽  
A. M. James Shapiro
Keyword(s):  
Liver Transplantation ◽  
Vascular Tumor ◽  
Current Treatment ◽  
Vascular Lesion ◽  
Epithelioid Hemangioendothelioma ◽  
Long Term Results ◽  
Imaging Features ◽  
Treatment Paradigm ◽  
Hepatic Lesions ◽  
Left Hepatic Lobectomy

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm with variable malignant potential that most often presents within the liver. Many patients present with bilobar or extrahepatic disease, and the current treatment paradigm involves liver transplantation, with favorable long term results. Up to 25% of patients are diagnosed incidentally following imaging for other indications, and confirmation of diagnosis requires histologic analysis, as there are no classical imaging features to distinguish hepatic EHE (HEHE) from other solid hepatic lesions. Here we describe a case of microscopic HEHE that was diagnosed following splenectomy for an enlarging vascular tumor within the spleen. Due to the unexpected diagnosis of EHE within the spleen and coexisting but stable appearing liver hemangiomata, a left hepatic lobectomy was performed. Explant histology revealed benign hemangiomata and diffuse, microscopic HEHE. The patient ultimately underwent liver transplantation. HEHE can be a challenging diagnosis, and this case emphasizes that any enlarging vascular lesion, even within the spleen, should prompt a high index of suspicion for HEHE in the setting of known hemangiomata.

Malignant Vascular Bone Tumors

2019 ◽  
pp. 308-312
Author(s):  
G. Scott Stacy
Keyword(s):  
Bone Tumors ◽  
Malignant Tumors ◽  
Treatment Strategies ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Vascular Tumors ◽  
Bone Lesions ◽  
Imaging Features ◽  
Vascular Bone Tumors ◽  
Health Organization

The organization of vascular bone tumors can be thought of as a spectrum of disease including both benign and malignant entities. Chapter 61 focuses on the malignant tumors, which have elicited much controversy and debate. These include 2 vascular tumors of bone recognized by the World Health Organization: epithelioid hemangioendothelioma and angiosarcoma. Although there are no characteristic imaging features that would obviate the need for biopsy of these entities, they may present with multifocal bone lesions, which can suggest the diagnosis of a vascular tumor. This chapter will describe the clinical, pathophysiological, and imaging features of these malignant vascular bone tumors, with a brief mention of treatment strategies.

scholarly journals Multicentric epithelioid hemangioendothelioma of humerus and scapula

2021 ◽  
Vol 0 ◽  
pp. 1-5
Author(s):  
S B Dhanya ◽  
Ramakrishna Narayanan ◽  
Anu Kapoor ◽  
Shantveer G. Uppin
Keyword(s):  
Adult Population ◽  
Bone Lesion ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Adolescent Male ◽  
Lytic Lesion ◽  
Imaging Features ◽  
Post Contrast ◽  
Imaging Characteristics ◽  
The Right

Epithelioid hemangioendothelioma (EH) is a rare malignant vascular tumor occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. We present a rare case of multicentric EH in the right humerus in an adolescent male, who presented with complaints of pain and swelling in the right shoulder. Radiographs and Computed tomography showed a large expansile lytic lesion in the proximal end of the right humerus with areas of cortical destruction and matrix calcification. On Magnetic resonance imaging, the lesion was predominantly isointense on T1-weighted image, hyperintense on T2-weighted, and Short Tau Inversion Recovery images with prominent flow voids within. On post-contrast sequences, the lesion showed intense heterogenous enhancement with a non-enhancing central necrotic area. Multiple smaller lytic lesions with similar imaging characteristics were also found in the mid and distal shaft of the right humerus and the right coracoid process. Differentials of Telangiectatic osteosarcoma, Giant cell tumor, brown tumors of hyperparathyroidism, and metastasis were considered. Core biopsy revealed that the lesion was an EH. Though the imaging features of EH are non-specific, it may be considered in the differential diagnosis of an expansile lytic bone lesion with no periosteal reaction, showing cortical break and soft tissue component, especially if it is multifocal and multicentric.

scholarly journals Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma

1995 ◽  
Vol 2 (6) ◽  
pp. 483-487 ◽  
Author(s):  
J. R. Madariaga ◽  
I. R. Marino ◽  
D. D. Karavias ◽  
M. A. Nalesnik ◽  
H. R. Doyle ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Epithelioid Hemangioendothelioma ◽  
Long Term Results

Current concepts of the interventional treatment of proximal supraaortic vessel stenosis

VASA ◽  
2012 ◽  
Vol 41 (5) ◽  
pp. 313-318 ◽  
Author(s):  
Ernemann ◽  
Bender ◽  
Melms ◽  
Brechtel ◽  
Kobba ◽  
...  
Keyword(s):  
Treatment Strategies ◽  
Carotid Bifurcation ◽  
Current Treatment ◽  
Long Term Results ◽  
Clinical Indication ◽  
Interventional Treatment ◽  
Special Focus ◽  
Brachiocephalic Artery ◽  
Treatment Concepts ◽  
Angioplasty And Stenting

Interventional therapies using angioplasty and stenting of symptomatic stenosis of the proximal supraaortic vessels have evolved as safe and effective treatment strategies. The aim of this paper is to summarize the current treatment concepts for stenosis in the subclavian and brachiocephalic artery with regard to clinical indication, interventional technique including selection of the appropriate vascular approach and type of stent, angiographic and clinical short-term and long-term results and follow-up. The role of hybrid interventions for tandem stenoses of the carotid bifurcation and brachiocephalic artery is analysed. A systematic review of data for angioplasty and stenting of symptomatic extracranial vertebral artery stenosis is discussed with a special focus on restenosis rate.

NEUROENDOCRINE TUMORS OF THE PANCREAS: ETIOLOGY, PATHOGENESIS, DIAGNOSIS, AND CURRENT TREATMENT APPROACHES

2018 ◽  
Vol 64 (6) ◽  
pp. 830-839
Author(s):  
Temuri Morgoshiya
Keyword(s):  
Neuroendocrine Tumors ◽  
Clinical Manifestations ◽  
Gastrointestinal Hormones ◽  
Current Treatment ◽  
High Potential ◽  
Long Term Results ◽  
Considerable Part ◽  
Treatment Approaches ◽  
By Products

The overview of literature on modem classification issues, diagnostics and treatments of neuroendocrinal tumors of a pancreas is provided. According to modern views all neuroendocrinal tumors of a pancreas having clinical manifestations (in the form of the syndromes caused by products of specific hormones; increases in level of hormones in blood of patients without clinical manifestations; in the form of signs of existence of volume education in various departments of PZh) and/or the researches (more than 5 mm) revealed by means of beam methods are malignant in the biology as they have high potential to innidiation. In article it is shown that a considerable part of neuroendocrinal tumors of a pancreas are nonfunctioning, i.e. not cosecreting various gastrointestinal hormones and polypeptides in blood and thereof not followed characteristic clinical manifestations. It is noted that diagnostics of neuroendocrinal tumors of a pancreas is extremely difficult task on which solution the choice of a method of treatment and its long-term results depends...

Current State and Future Possibilities in Liver Transplantation

2021 ◽  
Author(s):  
Sebastian Rademacher ◽  
◽  
Niklas Aehling ◽  
Robert Sucher ◽  
Thomas Berg ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Survival Rates ◽  
Tolerance Induction ◽  
Meld Score ◽  
Long Term Results ◽  
Future Research ◽  
Industrialized Countries ◽  
Normothermic Machine Perfusion ◽  
Donor Characteristics

Due to medical and surgical progress, liver transplantation (LT) is is nowadays a routine treatment for terminal liver failure and hepatic malignancies. However, in recent years there has been a change in the indications for LT. Especially in western industrialized countries, the use of LT for chronic hepatis B and hepatitis C cirrhosis is continuously decreasing since the introduction of effective antiviral drugs. Liver cirrhosis due to non-alcoholic steatohepatitis (NASH), alcoholic liver disease and hepatocellular carcinoma (HCC) in cirrhosis are now among the leading indications for LT. Due to tremendous progress in oncology, immunology, and technical aspects, multidisciplinary cancer treatment increasingly includes LT for non-HCC hepatobiliary malignancies. Excellent 5-year survival rates of 75 to 80% can now be achieved after LT. However, in patients with liver cirrhosis, the implementation of a ‘sickest first’ principle for liver allocation has led to an increasing number of critically ill patients undergoing liver transplantation. This results in an increased morbidity and mortality after liver transplantation. Moreover, donor characteristics have markedly shifted to less ideal grafts due to an increasing shortage of donor organs in many countries. In this context, normothermic machine perfusion with oxygenated blood components using pulsatile flow has been shown to reduce liver damage despite a prolonged preservation time and might be able to provide viability testing for otherwise discarded organs. With favorable donor and recipient conditions, excellent long-term results can be obtained with a 10-year survival rate of close to 70%. However, in patients with a high MELD score (>30), survival rates markedly decrease by 12-18%. Future research should focus on optimization of organ allocation, optimization of immunosuppression including tolerance induction, and on increasing the donor organ pool to further improve and the numbers of successful LT.

The Quality of Life of Long Term Survivors of Patients with Biliary Atresia

2021 ◽  
Author(s):  
Melanie Le ◽  
Konrad Reinshagen ◽  
Christian Tomuschat
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
Long Term Results ◽  
Cochrane Library ◽  
Healthy Controls ◽  
Term Care ◽  
Associated Malformations

Abstract Purpose: Advances in surgical techniques and perioperative care have improved patients' short-and mid-term postoperative outcomes with Biliary Atresia (BA). However, the long-term results of these patients have not been thoroughly investigated. This systematic review aims to determine the long-term outcomes and the patients' health-related Quality of life (HrQoL) with their native livers or liver transplantation. Methods: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to August 2021. Original studies reporting on BA, Hepatoportoenterostomie, portoenterostomy, Kasai, Liver transplantation, Quality of life, or HrQoL were included. Pooled prevalence has been calculated for cholangitis, secondary liver transplantation, or associated malformations using MetaXL (version 5.3). Subgroup analysis on HrQoL followed surgical treatment after BA was calculated by using RevMan (version 5.4).Results: 12 articles were considered for data synthesis. Nine studies compared biliary atresia patients to an age-matched healthy reference group. 4/9 (n = 338) of these studies indicated lower scores for biliary atresia patients; 5/9 (n = 127) stated similar health status. A Forest plot analysis including all studies with total HrQoL showed a tendency of higher scores towards healthy controls (MD -0.79, 95% CI: -6.00-4.41). Comparing patients after Kasai Hepatoportoenterostomy with healthy controls demonstrated favorable outcomes for the control group (MD -3.22, 95% CI: -7.20-0.75) with no statistical significance (p = 0.11). The pooled estimation of the prevalence of cholangitis, secondary liver transplantation and associated malformations are 0.33 (95% CI: 0.06–0.66), 0.59 (95% CI: 0,42–0.75) and 0.13 (95% CI: 0,01–0.33).Conclusion: Biliary atresia patients have an overall high prevalence of progressive liver-related complications and risk of lower HrQoL compared to their healthy peers. Furthermore, those patients who received liver transplantation appear to have the same Quality of life as those living with their native livers. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research das focuses on the attributes and predictors of the long-term prognosis of patients with biliary atresia are necessary.

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