Cerebral epithelioid hemangioendothelioma with thoracic simultaneous involvement: advanced MRI features

Oliveira, Paulo Cesar Rocha; Alcantara, Fabio Peixoto; Souza-Vianna, Pedro Erthal de; Brito, Andreia Portilho de

doi:10.1590/s0004-282x2012000800015

Cited by 7 publications

(3 citation statements)

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“…Two hypotheses have been proposed; metastasis[ 8 27 37 ] and multicentric tumors. [ 15 28 ] In our case, all the tumors found in multiple organs were approximately same in size, showing rather a latent progression. The patient's neurological symptoms were mild despite a large number of intracranial hemorrhagic nodules with significant edema on the initial MRIs, which appears to be incompatible with metastases of malignant tumors.…”

Section: Discussionmentioning

confidence: 47%

“…[ 9 29 ] There are no typical radiological findings on CT or MRI for EHE. [ 1 4 8 13 28 41 ] Our case demonstrated numerous small nodules in the brain showing low-intensity on MR T2-weighted and susceptibility-weighted images, for which the patient underwent extensive workups covering vascular, tumorous, infectious, and metabolic diseases. In the present case, the results of biopsy and autopsy suggested that brain lesions were composed of hemorrhage and hemosiderin of different ages.…”

Section: Discussionmentioning

confidence: 87%

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Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma

et al. 2015

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Background:Epithelioid hemangioendotheliomas (EHE) is an extremely rare tumor that can arise not only intracranially but also systemically. Its radiological characteristics and the mechanism underlying the multiple organ involvement in EHE are poorly understood.Case Description:A 24-year-old woman with a 7-month history of coughing and blood-stained sputum complained of visual disturbance in the right eye that had persisted for 1-month. Magnetic resonance (MR) imaging revealed multiple intraparenchymal masses with low-intensity on MR susceptibility-weighted images with minimal enhancement with gadolinium. Systemic computed tomography revealed multiple nodules in both lungs and the liver. Because her neurological status rapidly deteriorated, brain biopsy of the right frontal mass was performed. The pathological diagnosis was EHE. Over the following 3 months, the patient gradually developed disturbance of consciousness. She died at 4 months after admission because of significant intracranial hypertension.Conclusion:Although intracranial EHEs are extremely rare, they should be included in the differential diagnoses of multiple small-sized masses with low-intensity on MR susceptibility-weighted images. We also emphasize that the systemic involvement of this tumor was more compatible with multicentric development than metastasis.

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Section: Discussionmentioning

confidence: 47%

Section: Discussionmentioning

confidence: 87%

Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma

et al. 2015

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“…The current study presented HE lesions with low signals during DW imaging, which suggests the presence of loose connective tissue spaces and lower cell components compared with those of the other solid tumors (24). However, the use of DW MRI in differentiating between the histopathological subtypes of HEs has not been well described, and its value in forming a differential diagnosis should be evaluated in large studies.…”

Section: Discussionmentioning

confidence: 68%

Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of 7 cases

Tian

Wang

et al. 2016

Oncology Letters

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Abstract. The current study aimed to present the neuroradiological and histopathological features of intracranial hemangioendothelioma (HE). The computed tomography (CT; n=3) and magnetic resonance imaging (MRI; n=7) features, and the clinical presentations of 7 patients with pathologically documented HEs were retrospectively analyzed. Lesions were observed in the right side of the skull (the frontal bone in 1 patient and the parietal bone in 1 patient), the tentorium (2 patients), the cerebral falx (1 patient), the right cavernous sinus (1 patient) and the right temporal lobe (1 patient). The tumor was lobulated in 5 cases and round in 2 cases. The majority of tumors appeared isointense or hypointense with multiple scattered hyperintensities on T1-weighted MRI. Moreover, the lesions appeared as inhomogeneous hyperintense regions with multiple enlarged and tortuous blood flow voids on T2-weighted MRI. The lesions also showed marked gadolinium enhancement in a honeycomb pattern. CT scan results showed a isoattenuation region (32-47 HU), with numerous small, round, high-density foci. The 2 cases with skull lesions presented with local bone destruction and discontinuous bone lines of the tabula interna ossis cranii. In 1 case, MR angiography revealed abnormal vessels in the basilar region. A total of 4 cases were epithelial HE, 2 were retiform HE and 1 was kaposiform HE. Histological examination revealed endothelial cell proliferation with vascular lesions and a mucous matrix or dense fibrous mesenchyme. In conclusion, intracranial HE is rare, but should be considered in the differential diagnosis when evaluating intracranial neoplasms. A well-defined lobulated mass and imaging features that include internal heterogeneity, small scattered hemorrhages and thromboses, signal voids of vessels, and marked and delayed enhancement may confirm the diagnosis of HE.

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Surgical considerations in a paediatric case of a large skull-base epithelioid haemangioendothelioma

et al. 2018

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Cerebral epithelioid hemangioendothelioma with thoracic simultaneous involvement: advanced MRI features

Cited by 7 publications

References 4 publications

Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma

Multiple hemorrhagic intraparenchymal tumors presenting with fatal intracranial hypertension: A rare manifestation of systemic epithelioid hemangioendothelioma

Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of 7 cases

Surgical considerations in a paediatric case of a large skull-base epithelioid haemangioendothelioma

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