scholarly journals HepaticYAP1-TFE3Rearranged Epithelioid Hemangioendothelioma

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Mira M. Lotfalla ◽  
Andrew L. Folpe ◽  
Karen J. Fritchie ◽  
Patricia T. Greipp ◽  
Gretchen G. Galliano ◽  
...  
Keyword(s):  
Fusion Gene ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Diagnostic Pitfall ◽  
Vascular Neoplasms ◽  
Nodular Hyperplasia ◽  
Genetic Features ◽  
Diagnostic Confusion

Epithelioid hemangioendothelioma (EHE) is an uncommon low-grade malignant vascular tumor that may arise in soft tissue/bone or visceral sites such as the liver and lung. As this tumor exhibits epithelioid morphology, it frequently causes diagnostic confusion with other epithelioid vascular neoplasms as well as carcinoma. While 90% of classic EHE are driven by aWWTR1-CAMTA1fusion gene, a histologically distinctive subset of EHE has been recently shown to harbor a different fusion gene,YAP1-TFE3. This variant is likely underrecognized given its rarity and only recent description. Notably, EHE frequently involves the liver but only one case of hepaticYAP1-TFE3rearranged EHE has been reported to date. We present the second case ofYAP1-TFE3rearranged EHE affecting a 65-year-old woman and presenting as multiple liver masses, with characterization of the fusion gene at the transcriptomic and genomic levels. There are several educational points noted from this case.YAP1-TFE3rearranged EHE shows distinctly vasoformative foci, unlike classic EHE and mimicking angiosarcoma or epithelioid hemangioma. The tumors cells show a histiocytoid appearance with voluminous cytoplasm, similar to otherTFE3-rearranged tumors. Finally, in the liver, this tumor may in part mimic focal nodular hyperplasia of the liver which is an underrecognized diagnostic pitfall. This report highlights the key diagnostic and genetic features of this newly recognized variant of hepatic EHE to aid pathologists in appropriately classifying these tumors.

scholarly journals Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Gintaras Januzis ◽  
Dovydas Sakalys ◽  
Martynas Mantas Krukis ◽  
Dmitrij Seinin
Keyword(s):  
Correct Diagnosis ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Vascular Tumors ◽  
Low Grade ◽  
X Rays ◽  
Adequate Treatment ◽  
Clinical Appearance ◽  
Allogenic Bone ◽  
Bony Destruction

Abstract Background Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. Case presentation We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. Conclusions With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

scholarly journals Pazopanib in Pulmonary Epithelioid Hemangioendothelioma: Report of a Case

2020 ◽  
Vol 5 (02) ◽  
Author(s):  
Ibtihal Ahalli ◽  
Karima Oualla ◽  
Loubna Hejjane ◽  
Raihana Boujarnija ◽  
Kaouthar Messoudi ◽  
...  
Keyword(s):  
Lung Tumor ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Vegf Receptor ◽  
Vascular Endothelial ◽  
Pulmonary Epithelioid Hemangioendothelioma ◽  
Grade Malignancy ◽  
Rare Malignancy ◽  
Endothelial Growth Factor

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, Vascular endothelial growth factor (VEGF) and the VEGF receptor were found on PEH tumor cells, suggesting that target therapies that block VEGFR have a logical base in this rare malignancy. We report a 25 years old woman with a pulmonary hemangioendothelioma, treated with pazopanib, the only anti angiogenic registered agent for sarcoma, with clinical improvement of symptoms and durable stabilization for more than two years of lung tumor.

Epithelioid Hemangioendothelioma, Multiple Focal Nodular Hyperplasias, and Cavernous Hemangiomas of the Liver

1999 ◽  
Vol 123 (9) ◽  
pp. 846-849 ◽  
Author(s):  
Marie-Pierre Bralet ◽  
Benoit Terris ◽  
Valérie Vilgrain ◽  
Laurence Brégeaud ◽  
Georges Molas ◽  
...  
Keyword(s):  
Young Woman ◽  
Focal Nodular Hyperplasia ◽  
Vascular Supply ◽  
Epithelioid Hemangioendothelioma ◽  
Unusual Association ◽  
Vascular Neoplasms ◽  
Nodular Hyperplasia ◽  
Cavernous Hemangiomas ◽  
True Neoplasm

Abstract Malignant vascular neoplasms of the liver are uncommon. We report the case of a young woman who developed an epithelioid hemangioendothelioma of the liver associated with multiple focal nodular hyperplasias and hepatic cavernous hemangiomas. Such an unusual association is probably not fortuitous and could support the theory that focal nodular hyperplasia is a reaction to an abnormal vascular supply rather than a true neoplasm.

scholarly journals Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene

2005 ◽  
Vol 85 (3) ◽  
pp. 408-415 ◽  
Author(s):  
Fredrik Mertens ◽  
Christopher D M Fletcher ◽  
Cristina R Antonescu ◽  
Jean-Michel Coindre ◽  
Maurizio Colecchia ◽  
...  
Keyword(s):  
Genetic Characterization ◽  
Fusion Gene ◽  
Molecular Genetic ◽  
Low Grade ◽  
Fibromyxoid Sarcoma ◽  
Molecular Genetic Characterization

scholarly journals Low-Grade Uterine Epithelioid Hemangioendothelioma Presented as a Submucosal Leiomyoma during Labor

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Anastasios V. Koutsopoulos ◽  
Efthimios Sivridis ◽  
Panagiotis Tsikouras ◽  
Vasileios Liberis ◽  
Georgia Karpathiou ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Pregnant Woman ◽  
Soft Tissue ◽  
Soft Tissue Tumors ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
High Grade ◽  
Unique Case ◽  
Uterine Corpus ◽  
Vascular Neoplasms

With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas). We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed.

scholarly journals Hepatic Epithelioid Hemangioendothelioma: A Review of Three Liver Transplant Recipients

2021 ◽  
Vol 21 (4) ◽  
Author(s):  
Ghazaleh Shaker ◽  
Farid Azmoudeh-Ardalan ◽  
Ali Jafarian ◽  
Masoomeh Safaei ◽  
Niloofar Ayoobi Yazdi
Keyword(s):  
Clinical Course ◽  
Treatment Strategies ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Transplant Recipients ◽  
Fatal Disease ◽  
Case Presentation ◽  
Disease Case ◽  
Liver Transplant Recipients

Introduction: Hepatic epithelioid hemangioendothelioma (HEHE) is a discrete vascular tumor with an unpredictable natural course. This rare tumor is commonly found incidentally and not too often is mistaken radiologically and histologically for another tumor. No single treatment strategy has yet been established for it, partly due to its variable clinical course, ranging from an indolent tumor with prolonged survival to an aggressive, fatal disease. Case Presentation: Among 1,029 liver transplantation cases performed at our hospital between January 2001 and November 2019, three were done for HEHE. In this study, we present these three cases and review their clinical and histopathologic characteristics. Conclusions: Epithelioid hemangioendothelioma (EHE) of the liver is a low-grade malignant tumor with different presentations, treatment strategies, and outcomes. The histopathologic characteristics of HEHE can hide its vascular nature, thus leading to its confusion with other lesions. This tumor is unique in that its clinical and histopathologic features do not always correlate with its biologic behavior. There are not reliable criteria in predicting the clinical outcome of HEHE, which needs further research.

Comparison of Immunological and Functional Assays for Measurement of Soluble Fibrin

1995 ◽  
Vol 74 (02) ◽  
pp. 673-679 ◽  
Author(s):  
C E Dempfle ◽  
S A Pfitzner ◽  
M Dollman ◽  
K Huck ◽  
G Stehle ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Low Grade ◽  
Plasma Samples ◽  
Normal Range ◽  
Soluble Fibrin ◽  
Discriminating Power ◽  
Fibrin Monomer ◽  
Cerebral Ischemic

SummaryVarious assays have been developed for quantitation of soluble fibrin or fibrin monomer in clinical plasma samples, since this parameter directly reflects in vivo thrombin action on fibrinogen. Using plasma samples from healthy blood donors, patients with cerebral ischemic insult, patients with septicemia, and patients with venous thrombosis, we compared two immunologic tests using monoclonal antibodies against fibrin-specific neo-epitopes, and two functional tests based on the cofactor activity of soluble fibrin complexes in tPA-induced plasminogen activation. Test A (Enzymun®-Test FM) showed the best discriminating power among normal range and pathological samples. Test B (Fibrinostika® soluble fibrin) clearly separated normal range from pathological samples, but failed to discriminate among samples from patients with low grade coagulation activation in septicemia, and massive activation in venous thrombosis. Functional test C (Fibrin monomer test Behring) displayed good discriminating power between normal and pathological range samples, and correlated with test A (r = 0.61), whereas assay D (Coa-Set® Fibrin monomer) showed little discriminating power at values below 10 μg/ml and little correlation with other assays. Standardization of assays will require further characterization of analytes detected.

scholarly journals Synthesis of a New Phosphonate-Based Sorbent and Characterization of Its Interactions with Lanthanum (III) and Terbium (III)

Polymers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 1513
Author(s):  
Yuezhou Wei ◽  
Khalid A. M. Salih ◽  
Mohammed F. Hamza ◽  
Toyohisa Fujita ◽  
Enrique Rodríguez-Castellón ◽  
...  
Keyword(s):  
High Tech ◽  
Low Grade ◽  
Acid Solutions ◽  
Hydrogel Beads ◽  
Secondary Sources ◽  
Composite Sorbents ◽  
Synthesis Procedure ◽  
New Generation ◽  
Mild Acid

High-tech applications require increasing amounts of rare earth elements (REE). Their recovery from low-grade minerals and their recycling from secondary sources (as waste materials) are of critical importance. There is increasing attention paid to the development of new sorbents for REE recovery from dilute solutions. A new generation of composite sorbents based on brown algal biomass (alginate) and polyethylenimine (PEI) was recently developed (ALPEI hydrogel beads). The phosphorylation of the beads strongly improves the affinity of the sorbents for REEs (such as La and Tb): by 4.5 to 6.9 times compared with raw beads. The synthesis procedure (epicholorhydrin-activation, phosphorylation and de-esterification) is investigated by XPS and FTIR for characterizing the grafting route but also for interpreting the binding mechanism (contribution of N-bearing from PEI, O-bearing from alginate and P-bearing groups). Metal ions can be readily eluted using an acidic calcium chloride solution, which regenerates the sorbent: the FTIR spectra are hardly changed after five successive cycles of sorption and desorption. The materials are also characterized by elemental, textural and thermogravimetric analyses. The phosphorylation of ALPEI beads by this new method opens promising perspectives for the recovery of these strategic metals from mild acid solutions (i.e., pH ~ 4).

scholarly journals HGG-02. ADOLESCENT AND YOUNG ADULT (AYA) GLIOMA WITH BRAF V600E-MUTANTATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii344-iii344
Author(s):  
Yui Kimura ◽  
Yukitomo Ishi ◽  
Yuko Watanabe ◽  
Yoshiko Nakano ◽  
Shigeru Yamaguchi ◽  
...  
Keyword(s):  
Braf Inhibitor ◽  
Clinical Information ◽  
Early Recurrence ◽  
Pleomorphic Xanthoastrocytoma ◽  
Adolescent And Young Adult ◽  
Braf V600e ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Genetic Features ◽  
Epithelioid Glioblastoma

Abstract BACKGROUND Biological features of pediatric glioma differ significantly from those of adult glioma, and limited data are available on those of AYA patients. Here, we focused on AYA patients with glioma, especially those harboring BRAF V600E mutation, and investigated their clinical and genetic features. METHOD: We retrospectively analyzed AYA patients with brain tumors harboring BRAF V600E, who were treated in two hospitals in Japan. RESULTS Clinical information was available for 14 patients. The median age at diagnosis was 25 years (range: 15–38). Five patients were diagnosed with glioblastoma (GBM), including one epithelioid type. These patients were over 25. Although one patient with GBM died of the disease 6.9 years after initial diagnosis, the remaining patients were alive. Two patients were alive without recurrence at 38 and 51 months after the treatment. The patient with epithelioid glioblastoma experienced early recurrence. The remaining nine patients (64%) were diagnosed with low-grade glioma, including ganglioglioma, pilocytic astrocytoma, diffuse astrocytoma, oligodendroglioma, pleomorphic xanthoastrocytoma, and polymorphous low-grade neuroepithelial tumor of the young. No patients died of the disease, and four patients are alive without recurrence after initial operation without adjuvant treatment. Two patients are (epithelioid glioblastoma and ganglioglioma) currently undergoing treatment with a BRAF inhibitor for recurrent tumors. DISCUSSION Although the number of this study is limited, our study suggested that the prognosis of AYA patients with BRAF-V600E positive GBM may not be as dismal as that of children or adults.

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