hepatic epithelioid hemangioendothelioma
Three cases
of histologically proven hepatic epithelioid hemangioendothelioma evaluated using a second-generation microbubble contrast medium in ultrasonography: case reports. a Conventional abdominal ultrasonography (US) in case 1 showed multiple low echoic liver nodules (arrow) with a vague margin, and nodules located in the peripheral sites tended to coalesce with each other. The vascular pattern inside the nodules was not precisely visible in the arterial dominant phase of contrast-enhanced computed tomography (CECT) (b). c The apparent diffusion coefficient (ADC) mapping in case1 is shown. d In the diffusion-weighted imaging (DWI) at a b score of 1000 (sec/mm2), the peripheral area of the nodules in case1 showed a much higher intensity than its central lesion. e SonazoidⓇ contrast enhanced US (CEUS) in case1 showed the vascularity (arrow) in the early vascular phase, but the vascular pattern is not specified. f Later in the portal venous and late phases (PVLP) and post vascular phase, they were defective. g Defect re-perfusion imaging clearly showed that the nodules were gradually enhanced from the peripheral sites as ringed enhancement. h Photomicrograph of a histological section of a hepatic specimen obtained via percutaneous liver needle biopsy in case 1 (200× with hematoxylin and eosin stain) is shown. i A high number of epithelioid tumor cells with spindle-shaped nuclei, form intracellular vascular lumina (arrow) (800× with hematoxylin and eosin stain). In immunostaining (200×), the sample was positive for cluster of differentiation (CD) 31 (j) and CD34 (k)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. A 35-year-old male HEH patient. a Target sign on portal phase image, consisted of concentric layers including markedly hypointense in central tumor regions and peripheral zones which were moderately hypointense relative to liver parenchyma (arrow). b Capsular retraction caused by a subcapsular lesion (white arrow) and lollipop sign, consisted of a well-defined tumor (as the “candy”) and an obstructed portal vein (as the “stick”) (black arrow) on portal phase image
Clinical
characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. A 34-year-old female with diffuse nodular type HEH. Axial and multiple phase contrast-enhanced CT images (a–c) show multiple, different-sized, coalescing, homogeneous, hypoattenuating nodules diffusing throughout the liver parenchyma. Masses show ring-like enhancement and the “target” sign. At the same time, there was arc-shaped effusion around the liver and peritoneal thickening. Peripheral distribution of masses and capsular retraction are also observed. The axial lung window image (d) shows lung involvement consisting of multiple round-like nodules
Clinical
characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. A 40-year-old female with diffuse nodular type HEH. Axial arterial and portal phase CT images (a, b) show multiple, diffuse nodules that lack obvious enhancement and confluent masses with strip-like enhancement located at the subcapsular region of the liver, accompanied by liver morphological changes and proportion imbalance. On follow-up CT performed 18 months later without any treatment, axial arterial and portal phase images (c, d) show no obvious changes in the lesions
Clinical
characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. A 48-year-old female with multifocal nodular type HEH. An axial unenhanced CT image (a) shows multiple round-like low-density lesions in the right lobe of the liver. The lesions show slight enhancement in the arterial phase (b). Axial and coronal reconstruction of portal phase CT images (c, d) show a larger nodule in the right lobe with portal veins entering and terminating in the periphery of the lesion (red arrow). This configuration resembles a typical “lollipop” sign
Clinical
characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. A 41-year-old female with solitary nodular type HEH. An axial unenhanced CT image a shows a round nodule with ill-defined, heterogeneous hypodensity. An axial arterial phase image (b) shows rink-like enhancement. An axial portal phase image (c) shows “target” sign enhancement. Coronal reconstruction of the portal venous phase (d) shows a narrowing portal vein branch adjacent to the lesion. This patient was initially misdiagnosed with hepatic abscesses. On follow-up CT performed 10 months after resection (e, f), a new lesion was detected in the right lobe of the liver (red arrowheads)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. a, b A 40-year-old male HEH patient with coexistence of target appearance on both T2 weighted and portal phase images. a Target sign on fat-saturated T2-weighted image, consisted of concentric layers including markedly hyperintense in central tumor regions and moderately hyperintense peripheral zone (arrow). b Target sign on portal phase image, consisted of concentric layers including markedly hypointense in central tumor regions and peripheral zones which were moderately hypointense relative to liver parenchyma (arrow). c, d A 51-year-old male rectal carcinoma patients with multiple liver metastasis. c A classic target sign on fat-saturated T2-weighted image (arrow). d Heterogeneous enhancement on portal phase image (arrow), instead of target sign
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. The appearance of lollipop sign on MRI images of 4 HEH patients (arrows)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. The appearance of capsular retraction on MRI images of 4 HEH patients (arrows)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. A 22-year-old male HEH patient. On portal phase image, target sign could be observed on a medium lesion (black arrow), while both small and large lesions failed to show target appearance (white arrow)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. A 38-year-old male HEH patient. a On fat-saturated T2 weighted image, target sign could be observed on a medium lesion (black arrow), while both small and large lesions failed to show target appearance (white arrow). b Target sign on DWI image (800 b-value), consisted of concentric layers including relatively hyperintense in central tumor regions and peripheral zones which were markedly hyperintense relative to liver parenchyma (arrows)
MRI
appearances of hepatic epithelioid hemangioendothelioma: a retrospective study of 57 patients. A 42-year-old male HEH patient. a Coalescent lesion with target sign on T1-weighted 3D GRE image, consisted of concentric layers including markedly hypointense in central tumor regions and peripheral zones which were moderately hypointense relative to liver parenchyma (arrows). b Coalescent lesion with target sign on fat-saturated T2-weighted TSE image, consisted of concentric layers including markedly hyperintense in central tumor regions and moderately hyperintense peripheral zone (arrows). c Lollipop sign on portal phase image, consisted of a well-defined tumor (as the “candy”) and an obstructed hepatic vein (as the “stick”) (arrow)
Three cases
of histologically proven hepatic epithelioid hemangioendothelioma evaluated using a second-generation microbubble contrast medium in ultrasonography: case reports. a The CT scan in case 2 showed multiple hypodense liver nodules, including cystic lesions, and enhancement inside the tumors started in peripheral lesions in the arterial dominant phase. b In the DWI, at a b score of 1000 (sec/mm2) the peripheral area of the nodules showed a much higher intensity than its central lesion. c ADC mapping is shown. d In the conventional abdominal US of case2, there are multiple low echoic nodules, some of which contained cystic lesions. Color flow signals were found in color Doppler imaging. In CEUS, the enhancement inside the nodules was found in both (e) the PVLP and (f) post vascular phase, whereas anechoic areas in the inner portion were not enhanced. g Photomicrograph of a histological section of a hepatic specimen obtained via percutaneous liver needle biopsy in case 2 (200× with hematoxylin and eosin stain) is shown. h A high number of epithelioid tumor cells with spindle-shaped nuclei, form intracellular vascular lumina (arrow) (800× with hematoxylin and eosin stain). In immunostaining (200×), the sample was positive for cluster of differentiation (CD) 31 (i) and CD34 (j)
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic and pulmonary epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic and pulmonary epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Hepatic
epithelioid hemangioendothelioma • Hepatic epithelioid hemangioendothelioma - Ganzer Fall bei Radiopaedia
Three cases
of histologically proven hepatic epithelioid hemangioendothelioma evaluated using a second-generation microbubble contrast medium in ultrasonography: case reports. a In the conventional abdominal US in case 3, there were multiple low echoic liver nodules, predominantly seen in the right lobe (arrow). b In the DWI at a b score of 1000 (sec/mm2), the peripheral site of the nodules showed a much higher intensity than its central lesion. c ADC mapping is shown. d The early vascular phase of CEUS in case3 showed that the tumors were gradually enhanced from the peripheral sites. e In the PVLP and post vascular phase, they were defective. f Photomicrograph of a histological section of a hepatic specimen obtained via percutaneous liver needle biopsy in case 3 (200× with hematoxylin and eosin stain) is shown. g A high number of epithelioid tumor cells with spindle-shaped nuclei, form intracellular vascular lumina (arrow) (800× with hematoxylin and eosin stain). In immunostaining (200×), the sample was positive for cluster of differentiation (CD) 31 (h) and CD34 (i)
Clinical
characteristics and CT features of hepatic epithelioid haemangioendothelioma and comparison with those of liver metastases. A 52-year-old female with diffuse nodular type HEH. Axial unenhanced CT images (a) show diffuse coalescing hypoattenuating lesions. Arterial phase CT images (b) show slight enhancement, and some nodules confluent to strip‑like or snowmelt are located subcapsularly in the liver. In the portal phase (c), the lesions are not progressively enhanced, a small branch of the portal vein is seen passing through the middle of the lesions (red arrow), and capsular retraction (yellow arrow). On follow-up CT performed 10 months after radiofrequency ablation, axial portal vein phase CT image (d) shows lesion reduction
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, low to intermediate grade malignant hepatic vascular tumor.
Epidemiology
There may be a greater female incidence (with reported male-to-female ratio, 3:2), with peak incidence thought to be around 30-40 years old.
Pathology
Histologically, the tumors are composed of dendritic and epithelioid cells. Tumor cells with intracytoplasmic lumina, occasionally containing red blood cells, appear as signet ring-like structures . They can be difficult to diagnose on the basis of biopsy results.
Radiographic features
General
They tend to be multiple solid tumor nodules, located in a predominantly peripheral distribution, which coalescence to individual nodules. Lesions adjacent to the capsule often produce hepatic 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 hypoattenuating lesions in both hepatic lobes that coalesce to form larger confluent hypoattenuating regions in a peripheral or subcapsular distribution, with a halo or target pattern of enhancement in larger lesions. Subcapsular lesions often present with capsular retraction. Hepatic or portal veins or their branches may taper and terminate at or just within the edge of these lesions (lollipop sign).
Calcifications are uncommon, but do occur on occasion.
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-type enhancement pattern after administration of a gadolinium-based contrast agent, with an occasional thin peripheral hypointense rim.
Ferumoxide-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 clinical outcome . The overall prognosis is much more favorable than other hepatic malignancies .
Radical surgical resection or orthoptic liver transplantation are considered the treatments of choice . Due to the often multifocal nature of the tumor, transplantation may be the optimal treatment. Metastatic lesions have been reported in ~30% of patients at presentation and occur most commonly in the lungs . Other less common sites include the abdominal lymph nodes, omentum, mesentery, and peritoneum.
See also
Siehe auch:
- epitheloides Hämangioendotheliom
- hepatic capsular retraction
- Hämangioendotheliom
- seltene Lebertumoren
- Leberhämangiom bei Kindern
und weiter:
Assoziationen und Differentialdiagnosen zu epitheloides Hämangioendotheliom der Leber:
