Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment

Rosenberg, Ari J.; Agulnik, Mark

doi:10.1007/s11864-018-0536-y

Cited by 128 publications

(146 citation statements)

References 48 publications

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“…Pseudomyogenic and composite subtypes are rare form of bones and soft tissue hemangioendotheliomas. All of these patterns of growth were not observed in our case [14] , [15] . Hemangiopericytoma characteristically grows in a sheet like, storiform pattern, around an intratumoral staghorn blood vessel.…”

Section: Discussioncontrasting

confidence: 44%

A unique case of benign intracranial hemangioma mimicking malignant transformation

Almaghrabi

Al-Maghrabi

2018

Radiology Case Reports

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Capillary hemangiomas are rare benign vascular lesions, commonly found on scalp, face, chest, or back of a neonate or infant. Hemangiomas of the central nervous system are very rare lesions. There are only a few cases of intracranial capillary hemangioma (ICH) arising in adults reported in the literature. We present a case of 59-year-old female with intermittent recurrent headache localized in the frontal area. Magnetic resonance imaging revealed left frontal extra-axial mass with peripheral enhancement. The patient underwent complete surgical resection of the tumor. Histopathology examination of the lesion revealed well defined vascular lesion composed of closely packed plump endothelial cells lining slit-like vascular channels containing scattered red blood cells. No evidence of infiltrative brain parenchyma was seen. Ki-67 proliferative index was low, less than 2%. The final diagnosis was confirmed to be ICH by histopathology and immunohistochemistry studies. The patient has remained healthy and free of disease 39 months since her initial surgery. ICH is a benign vascular lesion which rarely occurs in the central nervous system, particularly in the intracranial region. It can mimic malignant lesions on radiologic studies. Histopathology examination is the gold standard for diagnosis. If total resection is achieved, prognosis is generally good with no evidence of recurrence.

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Section: Discussioncontrasting

confidence: 44%

A unique case of benign intracranial hemangioma mimicking malignant transformation

Almaghrabi

Al-Maghrabi

2018

Radiology Case Reports

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“…It originates from endothelial-like cell with a clinical behavior intermediate between hemangioma and angiosarcoma [ 2 ]. Although World Health Organization has recommended that EHE be grouped with angiosarcomas [ 4 ], it has an unpredictable clinical behavior ranging from indolent to aggressively malignant [ 5 ]. Previous studies showed that the 5-year disease-specific survival for EHE was 81% in contrast to approximately 50% mortality rate at 1 year for soft-tissue angiosarcomas [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study

Shiba¹,

Imaoka

Shioji

et al. 2018

BMC Cancer

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BackgroundEpithelioid hemangioendothelioma is an exceedingly rare sarcoma often occurring as an indolent angiocentric vascular tumor at various anatomic sites. Few reports have evaluated large case series of epithelioid hemangioendothelioma.MethodsWe conducted a retrospective analysis of the clinical data of 42 consecutive patients with epithelioid hemangioendothelioma who were pathologically diagnosed between 1990 and 2014 at 13 Japanese tertiary hospitals. We analyzed their clinical characteristics, tumor features and prognostic factors.ResultsThe study included 22 men and 20 women, with a median age of 54 (range, 18–78) years. Pain was the most common symptom, occurring in 15 (68%) of the 22 symptomatic patients. The median maximum tumor diameter was 4.0 (range, 1.0–12.8) cm. The most commonly involved organs were the liver (81%), lungs (57%), and bones (12%). The overall survival rates were 79.5% at 1 year and 72.0% at 5 years. Substantially better survival was observed in asymptomatic patients than in symptomatic patients (P = 0.03), and better survival was also ovserved in patients with Ki-67 index ≤10% than in those with Ki-67 index > 10% (P = 0.04). By multivariate analysis, tumor size > 3.0 cm was associated with decreased survival (P = 0.049, hazard ratio 13.33).ConclusionsThis study showed the clinical characteristics of Japanese patients with epithelioid hemangioendothelioma. Tumor size > 3.0 cm is an independent indicator of a poor prognosis in epithelioid hemangioendothelioma. The presence of symptoms at the time of diagnosis and high Ki-67 index implied poor survival.

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“…The very rare EHE is derived from endothelial origins. These neoplasms are mainly localized to the superficial and deep soft tissues, the bone and the liver . The clinical manifestations and prognosis vary widely, but EHE is classified as an intermediate malignancy—although several experts describe it as a malignant tumour .…”

Section: Discussionmentioning

confidence: 99%

“…Epithelioid hemangioendothelioma (EHE) is a rare tumour of vascular origin with various clinical manifestations. The prognosis ranges from indolent to aggressively progressive disease . Because of its low incidence, only a few case reports describe the clinical manifestations.…”

Section: Introductionmentioning

confidence: 99%

A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

Zhou

et al. 2019

Clinical Respiratory J

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Cardiac epithelioid hemangioendothelioma (EHE) is a very rare tumour of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anaemia, and diffuse pulmonary nodules with halo signs that represented metastasis of cardiac EHE; these radiologic manifestations are relatively uncommon. During the initial workup for the patient’s pulmonary nodules, echocardiography missed the cardiac mass. However, positron emission tomography‐computed tomography revealed increased fluorodeoxyglucose intake in the right atrial wall, and cardiac magnetic resonance imaging (MRI) revealed an irregular nodule with normal T1‐weighted signal intensity and hyperintense T2‐weighted signal intensity. Enhanced abdominal computed tomography (CT) revealed micronodular liver metastases. Video‐assisted thoracic surgery was performed to make a definitive diagnosis. Immunohistochemistry staining proved the diagnosis of EHE with positive results for cluster of differentiation (CD) 34, CD31, erythroblast transformation‐specific‐related gene and Ki‐67. The patient started chemotherapy with docetaxel (75 mg/m2) and gemcitabine (900 mg/m2), but this failed to control his disease and he died from an opportunistic infection related to his immunocompromised status 5 months later. For the work out process of bilateral diffuse pulmonary nodules suspicious for cardiac origin, especially with atrial deviation, echocardiography alone is not sufficient to exclude atrial origin. Cardiac CT or MRI might be a better choice.

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Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment

Cited by 128 publications

References 48 publications

A unique case of benign intracranial hemangioma mimicking malignant transformation

A unique case of benign intracranial hemangioma mimicking malignant transformation

Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study

A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

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