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Case report: MRI diagnosis of multifocal epithelioid hemangioendothelioma of the liver.

Verma SK, Mitchell DG, Bergin D - Indian J Radiol Imaging (2008)

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Thomas Jefferson University Hospital, Philadelphia 19107, USA.

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Epitheloid hemangioendothelioma (EHE) is a vascular tumor of intermediate malignancy that arises in soft tissues, liver, lung, bone, and spleen... Multiple, larger, lobulated lesions with similar appearances were seen in segments 4 and 8 [Figure 1B]... Mild right-sided intrahepatic duct dilatation was noted due to mass effect, but there was no obstruction... After a definitive diagnosis of multifocal hepatic EHE with no evidence of metastases, liver transplantation was planned for the patient... The tumor shows sclerosis, calcification, and hyalinization in up to 50% of patients... With progression, the multiple hepatic nodular lesions coalesce as they grow at the periphery, resulting in diffuse involvement of the liver... The MRI findings of coalescent peripheral hepatic masses with capsular retraction are highly suggestive of mutifocal hepatic EHE... Two histological types, nodular and diffuse, have been described... Infiltration into sinusoids and into intrahepatic veins, resulting in luminal narrowing and obliteration with polypoidal projections, are characteristic features of hepatic EHE... Demonstration of the vascular or endothelial origin of the tumor is critical for diagnosis and requires immunostaining for endothelial markers, including factor VIII-related antigen, CD31, and CD34... Surgical resection or orthotopic liver transplantation is considered the treatment of choice, though rarely radiotherapy and chemotherapy may be used in patients with associated metastases... Multifocal hepatic EHE has a better prognosis and better long-term survival than any other hepatic malignancy... The typical MRI features should raise a suspicion in the correct clinical setting and an appropriately guided biopsy will usually give the answer, thus avoiding more complicated diagnostic or therapeutic interventions.

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Photomicrograph shows cords of spindle-to-epithelioid cells in a myxoid stroma and prominent vacuoles, some of them containing red blood cells. (H&E, ×50)
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Figure 0003: Photomicrograph shows cords of spindle-to-epithelioid cells in a myxoid stroma and prominent vacuoles, some of them containing red blood cells. (H&E, ×50)

Mentions: A 39-year-old woman presented with right upper quadrant abdominal pain of six months duration. She denied any fever, chills, or malaise. On physical examination, there was painless hepatomegaly. Laboratory values, including serum glutamate oxaloacetate transaminase (SGOT), serum glutamate pyruvate transaminase (SGPT), bilirubin, and alpha-fetoprotein (AFP) levels, were all normal; there was increased alkaline phosphatase (620 U/l) and gamma-glutamyl-transpeptidase (300 U/l) and mild hypoalbuminemia (3 g/dl). She underwent abdominal CT, which revealed conglomerate mass lesions suggestive of metastases in the liver. MRI of the abdomen was then performed on a 1.5-T unit (GE Medical Systems, Milwaukee, USA) using T1W, T2W, and dynamic gadolinium-enhanced sequences. Innumerable small, rounded, predominantly peripheral hepatic lesions, measuring between 1 and 3 cm in size, were identified throughout segments 2 and 3 as well as in the subcapsular right lobe of the liver. These were centrally isointense on T2W and hypointense on T1W images, with an unusual peripheral rim of T2 hyperintensity [Figure 1]. There was moderate enhancement of these lesions except for the hyperintense T2 rim, which showed delayed enhancement [Figure 2]. Some of the lesions showed capsular retraction [Figure 2C]. Multiple, larger, lobulated lesions with similar appearances were seen in segments 4 and 8 [Figure 1B]. Mild right-sided intrahepatic duct dilatation was noted due to mass effect, but there was no obstruction. The patient was subjected to a real-time USG-guided percutaneous liver biopsy. On histological examination, poorly-defined infiltrative tumor was seen, characterized by nests and cords of spindle-to-epithelioid cells embedded in a hyaline, myxoid stroma. The cells showed prominent cytoplasmic vacuoles and intracellular lumina containing red blood cells [Figure 3]. There was positive immunohistochemical staining for the endothelial markers CD31 and CD34. After a definitive diagnosis of multifocal hepatic EHE with no evidence of metastases, liver transplantation was planned for the patient.


Case report: MRI diagnosis of multifocal epithelioid hemangioendothelioma of the liver.

Verma SK, Mitchell DG, Bergin D - Indian J Radiol Imaging (2008)

Photomicrograph shows cords of spindle-to-epithelioid cells in a myxoid stroma and prominent vacuoles, some of them containing red blood cells. (H&E, ×50)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2747429&req=5

Figure 0003: Photomicrograph shows cords of spindle-to-epithelioid cells in a myxoid stroma and prominent vacuoles, some of them containing red blood cells. (H&E, ×50)
Mentions: A 39-year-old woman presented with right upper quadrant abdominal pain of six months duration. She denied any fever, chills, or malaise. On physical examination, there was painless hepatomegaly. Laboratory values, including serum glutamate oxaloacetate transaminase (SGOT), serum glutamate pyruvate transaminase (SGPT), bilirubin, and alpha-fetoprotein (AFP) levels, were all normal; there was increased alkaline phosphatase (620 U/l) and gamma-glutamyl-transpeptidase (300 U/l) and mild hypoalbuminemia (3 g/dl). She underwent abdominal CT, which revealed conglomerate mass lesions suggestive of metastases in the liver. MRI of the abdomen was then performed on a 1.5-T unit (GE Medical Systems, Milwaukee, USA) using T1W, T2W, and dynamic gadolinium-enhanced sequences. Innumerable small, rounded, predominantly peripheral hepatic lesions, measuring between 1 and 3 cm in size, were identified throughout segments 2 and 3 as well as in the subcapsular right lobe of the liver. These were centrally isointense on T2W and hypointense on T1W images, with an unusual peripheral rim of T2 hyperintensity [Figure 1]. There was moderate enhancement of these lesions except for the hyperintense T2 rim, which showed delayed enhancement [Figure 2]. Some of the lesions showed capsular retraction [Figure 2C]. Multiple, larger, lobulated lesions with similar appearances were seen in segments 4 and 8 [Figure 1B]. Mild right-sided intrahepatic duct dilatation was noted due to mass effect, but there was no obstruction. The patient was subjected to a real-time USG-guided percutaneous liver biopsy. On histological examination, poorly-defined infiltrative tumor was seen, characterized by nests and cords of spindle-to-epithelioid cells embedded in a hyaline, myxoid stroma. The cells showed prominent cytoplasmic vacuoles and intracellular lumina containing red blood cells [Figure 3]. There was positive immunohistochemical staining for the endothelial markers CD31 and CD34. After a definitive diagnosis of multifocal hepatic EHE with no evidence of metastases, liver transplantation was planned for the patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Thomas Jefferson University Hospital, Philadelphia 19107, USA.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Epitheloid hemangioendothelioma (EHE) is a vascular tumor of intermediate malignancy that arises in soft tissues, liver, lung, bone, and spleen... Multiple, larger, lobulated lesions with similar appearances were seen in segments 4 and 8 [Figure 1B]... Mild right-sided intrahepatic duct dilatation was noted due to mass effect, but there was no obstruction... After a definitive diagnosis of multifocal hepatic EHE with no evidence of metastases, liver transplantation was planned for the patient... The tumor shows sclerosis, calcification, and hyalinization in up to 50% of patients... With progression, the multiple hepatic nodular lesions coalesce as they grow at the periphery, resulting in diffuse involvement of the liver... The MRI findings of coalescent peripheral hepatic masses with capsular retraction are highly suggestive of mutifocal hepatic EHE... Two histological types, nodular and diffuse, have been described... Infiltration into sinusoids and into intrahepatic veins, resulting in luminal narrowing and obliteration with polypoidal projections, are characteristic features of hepatic EHE... Demonstration of the vascular or endothelial origin of the tumor is critical for diagnosis and requires immunostaining for endothelial markers, including factor VIII-related antigen, CD31, and CD34... Surgical resection or orthotopic liver transplantation is considered the treatment of choice, though rarely radiotherapy and chemotherapy may be used in patients with associated metastases... Multifocal hepatic EHE has a better prognosis and better long-term survival than any other hepatic malignancy... The typical MRI features should raise a suspicion in the correct clinical setting and an appropriately guided biopsy will usually give the answer, thus avoiding more complicated diagnostic or therapeutic interventions.

No MeSH data available.


Related in: MedlinePlus