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Hepatic tumors
Published in Prem Puri, Newborn Surgery, 2017
Benjamin A. Farber, William J. Hammond, Michael P. La Quaglia
Antiangiogenic drugs show potential in the treatment of liver hemangiomas. Bevacizumab, a recombinant monoclonal antibody against VEGF, was incidentally found to reduce the size of liver lesions significantly in a patient with colorectal adenocarcinoma initially suspected to be metastasis, but later found on biopsy to be hemangiomas.35 A similarly dramatic response was seen in a 41-year-old patient with pulmonary epithelioid hemangioendothelioma.36
Angiosarcoma
Published in Dongyou Liu, Tumors and Cancers, 2017
In addition, epithelioid angiosarcoma should be distinguished from epithelioid hemangioendothelioma. The former is noted for its greater cellularity, larger cells, more prominent mitotic activity, greater nuclear and nucleolar pleomorphism, and more frequent tightly cohesive cell clusters without a myxohyaline matrix (immunoreactivity for vimentin and cytokeratin). The latter tends to affect younger patients (20–40 years), has an indolent course, and contains specific gene fusion at WWTR1 (WW domain–containing transcription regulator protein 1) and CAMTA1 (calmodulin-binding transcription activator 1) due to the nonrandom reciprocal t(1;3)(p36;q25) translocation, turning the affected endothelial cells to tumors [5]
Hepatic Tumors
Published in John F. Pohl, Christopher Jolley, Daniel Gelfond, Pediatric Gastroenterology, 2014
Rohit Gupta, Laura S. Finn, Karen F. Murray
Epithelioid hemangioendothelioma (EHE) is a rare slow-growing vascular tumor of the liver that primarily affects adult patients, with only rare cases reported in late childhood (12–14 years of age). EHE is considered a low-grade malignant neoplasm in contrast to the more aggressive angiosarcoma.
Multifocal and hormone-dependent epithelioid hemangioendothelioma with osteolysis of the second cervical vertebral body: report of an unprecedented surgical approach by using autologous bone graft
Published in British Journal of Neurosurgery, 2023
Hanno M. Witte, Armin Riecke, Thomas Mayer, Carsten Hackenbroch, Konrad Steinestel, Niklas Gebauer, Dirk Puckhaber, René Mathieu, Uwe Max Mauer, Chris Schulz
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with endothelial differentiation1 and is described as of intermediate malignancy between that of hemangiomas and angiosarcomas.2 The tumor may arise in a wide variety of locations. Pulmonary, hepatic, osseous, splenic, cardiac, intracranial and other manifestations1,3,4 have been reported in the literature. EHE accounts for less than 1% of primary malignant bone tumors.1 Primary vertebral manifestations are considerably less common. Most of the vertebral manifestations involve the thoracic and lumbar regions; the cervical spine is less commonly affected. Moreover, EHE accounts for less than 1% of all vascular tumors. There is no sex predilection (45.2% of the tumors occur in women and 54.8% in men). The mean age at the onset of EHE is 39.5 years. EHE can present as multifocal lesions. Nevertheless the five-year survival rate is over 60%.5 The tumor was described for the first time by Dail and Liebow6 in 1975. The term epithelioid hemangioendothelioma was first coined by Weiss and Enzinger7 in 1982.
The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib
Published in Expert Review of Anticancer Therapy, 2019
Christopher P Wilding, Mark L Elms, Ian Judson, Aik-Choon Tan, Robin L Jones, Paul H Huang
A further cohort of 15 patients with metastatic or locally advanced epithelioid hemangioendothelioma (EHE) not amenable to curative resection were enrolled onto this trial [92]. PFS at 9 months was chosen as the primary end-point given the indolent nature of EHE [93]. Seven of the 15 patients (46%) had undergone previous surgery and 5 patients (33%) had received prior systemic anticancer therapy. mPFS was 6 months, with a non-progression rate at 9 months of 30.7% (4 of 13 assessable patients). Best response rate on cross-sectional imaging per RECIST following sorafenib was a partial response in 2 of 13 assessable patients (13.3%) and stable disease in 9 of 13 (69.2%). In the French Sarcoma Group study, a sorafenib dose reduction was required in 3 of 15 patients (20%), whilst 5 patients (33.3%) required a transient drug discontinuation due to toxicity.
What’s the latest with investigational drugs for soft tissue sarcoma?
Published in Expert Opinion on Investigational Drugs, 2022
Elena Cojocaru, Andrea Napolitano, Cyril Fisher, Paul Huang, Robin L Jones, Khin Thway
Angiosarcoma and epithelioid hemangioendothelioma (EHE) are both highly vascular tumors but have different underlying mechanisms of development and biology. EHE has been histologically compared to angiosarcoma but is characterized by WWTR1(TAZ)-CAMTA, or YAP1-TFE3 fusions [72]. EHE are tumors with a spectrum of clinical behaviors, ranging from slow-growing and indolent disease to very aggressive and rapidly progressing disease which sometimes necessitates treatment with chemotherapy agents used in other types of STS [73,74]. In EHE, the use of mechanistic target of rapamycin (mTOR) inhibitors has shown the most meaningful clinical benefit and should be offered to patients with advanced disease [73,75].