Hepatic epithelioid hemangioendothelioma
Updates to Article Attributes
A hepaticHepatic epithelioid haemangioendothelioma (HEHE) is a rare, low to intermediate grade malignant hepatic vascular tumour.
Epidemiology
There may be a greater female incidence (with reported male-to-female ratio, 3:2), with peak incidence is thought to be around 30
- 40-40 year-old.
Pathology
Histologically, the tumors are composed of dendritic and epithelioidcells. Tumour cells with intracytoplasmic lumina, occasionallycontaining red blood cells, appear as signet ring–likestructures 2. They can be difficult to diagnose on the basis of biopsy results .
Radiographic features
General
They tend to be multiple solid tumour nodules, located in a predominantlyperipheral distribution, with coalescence as individual nodules. Lesions adjacent to thecapsuleoften producehepatic capsular retraction.
Ultrasound
Usually seen as hepatic lesions that are predominantly hypoechoic; however, hepatic lesions can also have mixed echotexture or be predominantly hyperechoic.
CT
Typically seen as multiple hypo-attenuatinglesions in both hepatic lobes that coalesce to formlarger confluent hypo-attenuating regions in a peripheral or subcapsulardistribution and a halo or target pattern ofenhancement in larger lesions.
MRI liver
-
T1:
-hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images -
T2:
-heterogeneously increased signal intensity. -
C+ (Gd):
-some lesions demonstrate either a peripheral halo or a target-typeenhancement pattern after administration of a gadolinium-basedcontrast agent, with occasional observation of athin peripheral hypointense rim.
Ferumoxides-enhanced T2-weighted images may help physicians distinguish tumor margins
Treatment and prognosis
The clinical course of these lesions can be variable with histologic analysis being of little value in predicting the clinicaloutcome 1. The overall prognosis is much more favourable than other hepatic malignancies 4 .
Radical surgical resection or orthoptic liver transplantation are considered the treatments of choice 1,4. Due to the often multifocal nature of the tumour, transplantation may be the optimal treatment. Metastatic lesions have been reported in ~ 30~30% of patients at presentation and occur most commonly in the lungs 7. Other less common sites include the abdominal lymph nodes, omentum, mesentery, and peritoneum.
See also
-<p>A <strong>hepatic epithelioid haemangioendothelioma (HEHE)</strong> is a rare, low to intermediate grade malignant hepatic vascular <span>tumour</span>.</p><h4>Epidemiology</h4><p>There may be a greater female incidence (with reported male-to-female ratio, 3:2), with peak incidence is thought to be around 30-- 40 year-old.</p><h4>Pathology</h4><p>Histologically, the tumors are composed of dendritic and <span>epithelioid</span>-<span>cells</span>. <span>Tumour</span> cells with intracytoplasmic <span>lumina</span>, occasionally- <span>containing</span> red blood cells, appear as signet <span>ring–like</span>-<span>structures</span> <sup>2</sup>. They can be difficult to diagnose on the basis of biopsy results .</p><h4>-<span>Radiographic</span> features</h4><h5>General</h5><p>They tend to be multiple solid <span>tumour</span> nodules, located in a predominantly-<span>peripheral</span> distribution, with coalescence as individual <span>nodules</span>. Lesions adjacent to the-<span>capsule</span> <span>often produce</span> <a href="/articles/hepatic-capsular-retraction" title="hepatic capsular retraction">hepatic capsular retraction</a>.</p><h5>Ultrasound</h5><p>Usually seen as hepatic lesions that are predominantly <span>hypoechoic</span>; however, hepatic lesions can also have mixed <span>echotexture</span> or be predominantly <span>hyperechoic</span> </p><h5>CT</h5><p>Typically seen as multiple hypo-attenuating- <span>lesions</span> in both hepatic lobes that coalesce to form-<span>larger</span> confluent hypo-attenuating regions in a peripheral or <span>subcapsular</span>- <span>distribution</span> and a halo or <span>target pattern</span> of-<span>enhancement</span> in larger lesions</p><h5>MRI liver</h5><ul>-<li>-<strong>T1 </strong>- hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images</li>-<li>-<strong>T2</strong> - heterogeneously increased signal intensity. </li>-<li>-<strong>C+ (Gd)</strong> - some lesions demonstrate either a peripheral halo or a target-type-<span>enhancement</span> pattern after administration of a gadolinium-based- <span>contrast</span> agent, with occasional observation of a-<span>thin</span> peripheral <span>hypointense</span> rim. </li>-</ul><p>Ferumoxides-enhanced T2-weighted images may help physicians distinguish tumor margins </p><h4>Treatment and prognosis</h4><p>The clinical course of these lesions can be variable with <span>histologic</span> analysis being of little value in predicting the clinical- <span>outcome</span> <sup>1</sup>. The overall prognosis is much more <span>favourable</span> than other hepatic malignancies <sup>4</sup> .</p><p>Radical surgical resection or <span>orthoptic</span> liver transplantation <span>are considered</span> the treatments of choice <sup>1,4</sup>. Due to the often <span>multifocal</span> nature of the <span>tumour</span>, transplantation may be the optimal treatment. Metastatic lesions have been reported in ~ 30% of patients at presentation and occur most commonly in the lungs <sup>7</sup>. Other less common sites include the abdominal lymph nodes, omentum, mesentery, and peritoneum. </p><h4>See also</h4><ul>-<li>-<a title="epithelioid haemangioendothelioma" href="/articles/epithelioid-haemenagioendothelioma"><span>epithelioid</span> haemangioendothelioma</a> - general</li>-<li><a title="infantile haemangioendothelioma of liver" href="/articles/infantile-haemangioendothelioma-of-liver"><span>infantile</span> <span>haemangioendothelioma</span> of liver</a></li>- +<p><strong>Hepatic epithelioid haemangioendothelioma (HEHE)</strong> is a rare, low to intermediate grade malignant hepatic vascular tumour.</p><h4>Epidemiology</h4><p>There may be a greater female incidence (with reported male-to-female ratio, 3:2), with peak incidence is thought to be around 30-40 year-old.</p><h4>Pathology</h4><p>Histologically, the tumors are composed of dendritic and epithelioid cells. Tumour cells with intracytoplasmic lumina, occasionally containing red blood cells, appear as signet ring–like structures <sup>2</sup>. They can be difficult to diagnose on the basis of biopsy results .</p><h4>Radiographic features</h4><h5>General</h5><p>They tend to be multiple solid tumour nodules, located in a predominantly peripheral distribution, with coalescence as individual nodules. Lesions adjacent to the capsule often produce <a href="/articles/hepatic-capsular-retraction">hepatic capsular retraction</a>.</p><h5>Ultrasound</h5><p>Usually seen as hepatic lesions that are predominantly hypoechoic; however, hepatic lesions can also have mixed echotexture or be predominantly hyperechoic.</p><h5>CT</h5><p>Typically seen as multiple hypo-attenuating lesions in both hepatic lobes that coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution and a halo or target pattern of enhancement in larger lesions.</p><h5>MRI liver</h5><ul>
- +<li>
- +<strong>T1:</strong> hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images</li>
- +<li>
- +<strong>T2:</strong> heterogeneously increased signal intensity.</li>
- +<li>
- +<strong>C+ (Gd):</strong> some lesions demonstrate either a peripheral halo or a target-type enhancement pattern after administration of a gadolinium-based contrast agent, with occasional observation of a thin peripheral hypointense rim.</li>
- +</ul><p>Ferumoxides-enhanced T2-weighted images may help physicians distinguish tumor margins</p><h4>Treatment and prognosis</h4><p>The clinical course of these lesions can be variable with histologic analysis being of little value in predicting the clinical outcome <sup>1</sup>. The overall prognosis is much more favourable than other hepatic malignancies <sup>4</sup> .</p><p>Radical surgical resection or orthoptic liver transplantation are considered the treatments of choice <sup>1,4</sup>. Due to the often multifocal nature of the tumour, transplantation may be the optimal treatment. Metastatic lesions have been reported in ~30% of patients at presentation and occur most commonly in the lungs <sup>7</sup>. Other less common sites include the abdominal lymph nodes, omentum, mesentery, and peritoneum.</p><h4>See also</h4><ul>
- +<li>
- +<a href="/articles/epithelioid-haemenagioendothelioma">epithelioid haemangioendothelioma</a>: general</li>
- +<li><a href="/articles/infantile-haemangioendothelioma-of-liver">infantile haemangioendothelioma of liver</a></li>
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