Hepatic Epithelioid Hemangioendothelioma: Sonographic, CT, and MR Imaging Appearances

Lyburn, Iain; Torreggiani, William C.; Harris, Alison; Zwirewich, Charles; Buckley, Anne; Davis, Jennifer; Chung, Steven W; Scudamore, Charles H.; Ho, Stephen

doi:10.2214/ajr.180.5.1801359

Cited by 130 publications

(102 citation statements)

References 6 publications

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“…The endothelial nature of HE is indicated by the histological features of the tumor and positive staining for endothelial markers, such as CD34, CD31 and factor VIII-related antigen. HE can affect adults and children, with no clear gender preponderance (13,14,18,19), However, the gender distribution in the present study was 6:1, females to males. Clinical symptoms were associated with the location of the lesion, whereas common symptoms included headaches and intracranial hypertension.…”

Section: Discussioncontrasting

confidence: 75%

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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Section: Discussioncontrasting

confidence: 75%

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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“…8 Common metastatic sites include abdominal lymph nodes, peritoneum, lungs, and bones. 7 On investigating, laboratory investigations (hemogram and biochemistry), tumor markers, and ultrasound are nonspecific for malignant HEH.…”

Section: Discussionmentioning

confidence: 99%

Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome

Hanchanale¹,

Varghese²,

Harika³

et al. 2017

Journal of Clinical and Experimental Hepatology

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Malignant hepatic epithelioid hemangioendothelioma is a rare, vascular tumor of the liver with peak incidence in the middle age with a female preponderance and of unknown etiology. Majority of the tumors are asymptomatic. The gold standard for diagnosis of the tumor is liver histology showing presence of epithelioid tumor cells on a background of a hyaline stroma. Immunohistochemical positivity for CD31, CD34, Factor VIII, and Podoplanin (D2-40) is confirmatory. The treatment of choice is liver transplantation. ( J CLIN EXP HEPATOL 2017;7:68-70) A 26-year-old woman was referred to our institution with a histopathological diagnosis of focal nodular hyperplasia for a space occupying lesion in the liver. The patient had a history of abdominal discomfort localized to the right upper quadrant with significant anorexia and weight loss of 8 kg over a period of 3 months. On examination of the abdomen, at admission, the liver was enlarged (span of 20 cm) and firm in consistency with a smooth surface and regular margin. Spleen was not palpable and there was no free fluid. Other system examinations were normal.The following laboratory investigations were investigated: Hemoglobin-11.9 g/dL, white blood cell count-8100 cells/mm 3 (neutrophils 72%, lymphocytes 17%, monocytes 07%, and eosinophil 04%), ESR-36 mm per h, and platelet count-259 Â 10 5 /mm 3 . Blood sugar, and serum creatinine were normal. The following liver function tests were conducted: serum total bilirubin 1.23 mg/dL, direct-0.35 mg/dL, serum asparate amino transferase-37 U/L, serum alanine amino transferase-37 U/L, serum alkaline phosphatase-430 U/L, and serum gamma glutamyl transpeptidase-123 U/L. International Normalized Ratio was 1.21.Ultrasound of the abdomen done elsewhere showed normal liver echotexture, with regular margins and multiple hypoechoic lesions in the right lobe, suggestive of hepatocellular carcinoma. Portal, hepatic veins, and suprahepatic inferior vena cava were normal. There was no free fluid. Ultrasound-guided fine needle aspiration of the lesion revealed focal nodular hyperplasia.Contrast-enhanced computed tomography (CECT) done at our institution showed multiple hypoattenutating lesions in both lobes of the liver with conglomeration of lesions in the right lobe. Differentials included multicentric hepatocellular carcinoma and malignant epithelioid hemangioendothelioma (Figure 1). A repeat ultrasound-guided biopsy was done, which showed spindle to epithelioid tumor cells embedded in myxoid background and cytoplasmic lumina (vacuole inside their cytoplasm corresponding to a vascular lumen) (Figure 2). Immunohistochemistry showed a diffuse positivity for CD 34 (Figure 3) and focal positivity for CD 31 (Figure 3) suggestive of malignant hepatic epithelioid hemangioendothelioma (HEH).Liver transplant was recommended, which she denied. She reported 7 months later with hoarseness of voice, dysphagia, and nasal regurgitation. Neurological examination revealed left IX, X, XI, XII cranial nerve palsy, and ipsilateral Horner's syndrome. Magne...

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“…No hay diferencia significativa en la frecuencia según sexo y edad, si bien en hígado y en pulmón son ligeramente más frecuentes en mujeres (1) , y en hueso son más frecuentes en personas jóvenes, mientras que el hígado y tejidos blandos son más frecuentes en la mediana edad (5,6) . Todos los pacientes que evaluamos eran relativamente jóvenes, con un promedio de 36 años de edad.…”

Section: Discussionunclassified

Características Radiológicas De Los Hemangioendoteliomas Epitelioides De Partes Blandas, Hígado Y Hueso

H¹,

et al. 2010

Rev. chil. radiol.

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IntroducciónEl hemangioendotelioma epitelioide (HEE) es un tumor vascular poco frecuente, de malignidad intermedia entre el hemangioma epitelioide y el angiosarcoma epitelioide, con una mortalidad estimada de 20% (1,2) . Aunque su frecuencia de presentación es baja, puede originar problemas en el diagnóstico diferencial con otros tumores primarios o metastásicos, por lo que consideramos interesante revisar nuestra experiencia y aportar elementos útiles en el diagnóstico de los HEE en las tres localizaciones más frecuentes, con el propósito de proporcionar información de utilidad para el diagnóstico de estos tumores mediante el uso de las técnicas de imagen cotidianas: radiografías simples, ultrasonografía (US), tomografía computarizada (TC) y resonancia magnética (RM). Rev Chil Radiol 2010; 16(3): 147-153. CAR ACTERÍSTICAS R ADIOLÓGICAS DE LOS HEMANGIOENDOTELIOMAS EPITELIOIDES DE PARTES BLANDAS, HÍGADO Y HUESO

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Hepatic Epithelioid Hemangioendothelioma: Sonographic, CT, and MR Imaging Appearances

Cited by 130 publications

References 6 publications

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

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

Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome

Características Radiológicas De Los Hemangioendoteliomas Epitelioides De Partes Blandas, Hígado Y Hueso

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