Diagnosis Of Hepatic Epithelioid Hemangioendothelioma Research Articles

Hepatic Epithelioid Hemangioendothelioma: A Case Report of an Extremely Rare Tumor

Epithelioid hemangioendothelioma is a very rare vascular tumour, with fewer than 500 cases described worldwide. Diagnosis is often multifocal or at an advanced stage with non-specific symptoms. Pathological examination by biopsy is crucial to confirm the diagnosis. The liver is the most frequent site of predilection, followed by the lungs and bones. The etiology of hepatic epithelioid hemangioendothelioma is poorly understood and is not linked to chronic liver disease, with a heterogeneous and unpredictable course depending on the individual. Given the rarity of this cancer, there are no established treatment standards for this type of tumor of intermediate malignancy. Surgical resection is performed in the case of unifocal disease, and orthotopic liver transplantation remains an option in the case of multifocal and/or unresectable disease, with high survival rates and disease free survival (DFS). Transcatheter arterial chemoembolization (TACE) and chemotherapy/radiotherapy regimens are other treatment possibilities. However, surveillance may be proposed for asymptomatic patients with a slow progression. We report the case of a patient followed for a multifocal EHE with pulmonary metastases, discovered following pain in the right hypochondrium. Morphological and immunohistochemical examination confirmed the diagnosis of hepatic epithelioid hemangioendothelioma. Given the moderate symptoms, surveillance was recommended for our patient with a good clinical course and radiological stability for 3 years.

A Young Female with a Hepatic Epithelioid Hemangioendothelioma.

Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide. A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team's findings, liver transplantation was indicated as the only curative treatment. Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis. Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis.

Diagnostic value of contrast-enhanced ultrasound in hepatic epithelioid hemangioendothelioma

Objective: To investigate the features of contrast-enhanced ultrasound (CEUS) in hepatic epithelioid hemangioendothelioma (HEHE) in order to improve the preoperative diagnosis rate. Methods: CEUS images of 32 pathologically-proven cases of hepatic epithelioid hemangioendothelioma from January 2004 to August 2021 were collected. Lesions were analyzed to observe the features of enhancement mode, enhancement intensity, and distinct enhancement phases. Results: Among the 32 cases, one had a solitary lesion, 29 had multiple lesions, and two had diffuse-type lesions. Contrast-enhanced ultrasound revealed a total of 42 lesions in 32 cases. In terms of arterial phase enhancement, 18 lesions had overall enhancement, six lesions had uneven dendritic enhancement, 16 lesions had rim-like enhancement, and two lesions had just slight peripheral spot enhancement around the lesions. Among the three cases, there were multiple lesions that had overall enhancement and ring enhancement. In terms of the enhancement phase, 20 lesions showed "fast progression", 20 lesions showed "same progression", and two lesions showed "slow progression". During the late arterial or early portal venous phases with rapid washout, all lesions manifested as hypoechoic. With peaked enhanced intensity, 11 lesions had a lower enhancement intensity than the surrounding normal liver parenchyma; 11 lesions had the same enhancement degree as the surrounding normal liver parenchyma; and 20 lesions had a higher enhancement degree than the surrounding normal liver parenchyma. All 16 ring-enhancing lesions had marked hyperenhancement. In the typical enhancing lesions, four showed hyperenhancement, five showed low enhancement, and nine showed isoenhancement. In the dendrite-enhancing lesions, there were two isoenhancing and four hypoenhancing. Contrast-enhanced ultrasound delineated the boundaries of all lesions more clearly than two-dimensional ultrasound. Conclusion: Contrast-enhanced ultrasound has certain value in the diagnosis of hepatic epithelioid hemangioendothelioma.

Conventional Ultrasound and Contrast-Enhanced Ultrasound in Hepatic Epithelioid Hemangioendothelioma: Retrospective Evaluation in 20 Cases.

ObjectivesThis study aimed to analyze the patterns of conventional ultrasound (CUS) and contrast-enhanced ultrasound (CEUS) in 20 patients with diagnosis of hepatic epithelioid hemangioendothelioma (HEHE).MethodsTwenty patients (12 females and 8 males) with mean age of 43.6 ± 13.6 years were included in this study from January 2012 to May 2020. CUS, CEUS, computed tomography (CT) and magnetic resonance imaging (MRI) features of the twenty patients with histologically proven HEHE were retrospectively reviewed by two radiologists. The clinical manifestations and the pathological findings of all patients with HEHE are described.ResultsThere were 3 types of HEHE in imaging, including single nodular (8/20, 40%), multifocal nodular (10/20, 50%), and diffuse type (2/20, 10%). The mean size of lesions was 4.2 ± 2.6 cm. B-mode ultrasound of HEHE showed hypoechoic (15/20, 75%), heterogeneous echogenicity (4/20, 20%), or hyperechoic (1/20, 5%) lesions with regular shape (18/20, 90%) near the liver capsule (17/20, 85%), and occasionally with a halo (4/20, 20%) and calcifications (3/20, 15%). Eight out of the 20 patients also had CEUS. On CEUS, HEHE demonstrated peripheral rim-like (5/8, 62.5%), heterogeneous (2/8, 25%), or homogeneous (1/8, 12.5%) hyperenhancement in the arterial phase. All patients (8/8, 100%) showed hypoenhancement in the portal and late phase. CEUS detected more lesions than CUS in 3 patients (3/8, 37.5%). In addition, central irregular unenhanced zones were observed in 6 patients (6/8, 75%). On contrast-enhanced CT or MRI, most cases presented with capsule retraction sign and lollipop sign.ConclusionsHEHE demonstrated specific findings on ultrasound, which includes multifocal hypoechoic lesions in a subcapsular distribution with typical enhancement characteristics of malignant hepatic tumors.

Post-resection prognosis of patients with hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare borderline vascular tumor. This retrospective, single-center study evaluated the outcomes of hepatic resection (HR) in patients with hepatic EHE. Over the 10-year period from 2009 to 2018, 11 patients with hepatic EHE underwent HR, accounting for 0.1% of the 11,979 adults who underwent HR at our center. Diagnosis of hepatic EHE was confirmed by immunohistochemical staining for CD34, CD31, and factor VIII-related antigen. The 11 patients included nine (82%) women and two (18%) men, of mean age 43.5 ± 13.6 years. Preoperative imaging resulted in a preliminary diagnosis of suspected liver metastasis or EHE, with nine patients (82%) undergoing liver biopsy. No patient presented with abnormally elevated concentrations of liver tumor markers. The extents of HR were determined by tumor size and location from trisectionectomy to partial hepatectomy. All patients recovered uneventfully from HR. Five patients showed tumor recurrence, with four receiving locoregional treatments for recurrent lesions. The 1-, 3-, and 5-year disease-free survival rates were 90.9%, 54.5%, and 54.5%, respectively. Currently, all patients remain alive and are doing well. Univariate analysis on tumor recurrence showed that tumor size ≥4 cm was significantly associated with tumor recurrence (p = 0.032), but tumor number ≥ 4 was not related to tumor recurrence (p = 0.24). Hepatic EHE is a rare form of primary liver tumor often misdiagnosed as a metastatic tumor. Because of its malignant potential, HR is indicated if possible. HR plus, when necessary, treatment of recurrence yields favorable overall survival rates in patients with hepatic EHE.

Post-resection prognosis of patients with hepatic epithelioid hemangioendothelioma.

PurposeEpithelioid hemangioendothelioma (EHE) is a rare borderline vascular tumor. This retrospective, single-center study evaluated the outcomes of hepatic resection (HR) in patients with hepatic EHE.MethodsOver the 10-year period from 2009 to 2018, 11 patients with hepatic EHE underwent HR, accounting for 0.1% of the 11,979 adults who underwent HR at our center. Diagnosis of hepatic EHE was confirmed by immunohistochemical staining for CD34, CD31, and factor VIII-related antigen.ResultsThe 11 patients included 9 females (81.8%) and 2 males (18.2%) with mean age of 43.5 ± 13.6 years. Preoperative imaging resulted in a preliminary diagnosis of suspected liver metastasis or EHE, with 9 patients (81.8%) undergoing liver biopsy. No patient presented with abnormally elevated concentrations of liver tumor markers. The extents of HR were determined by tumor size and location from trisectionectomy to partial hepatectomy. All patients recovered uneventfully from HR. Five patients showed tumor recurrence, with 4 receiving locoregional treatments for recurrent lesions. The 1-, 3- and 5-year disease-free survival rates were 90.9%, 54.5%, and 54.5%, respectively. Currently, all patients remain alive and are doing well. Univariate analysis on tumor recurrence showed that tumor size ≥ 4 cm was significantly associated with tumor recurrence (P = 0.032), but tumor number ≥ 4 was not related to (P = 0.24).ConclusionHepatic EHE is a rare form of primary liver tumor often misdiagnosed as a metastatic tumor. Because of its malignant potential, HR is indicated if possible. HR plus, when necessary, treatment of recurrence yields favorable overall survival rates in patients with hepatic EHE.

Laparoscopic repeat liver resection for hepatic epithelioid hemangioendothelioma

BackgroundOptimal treatment for patients with hepatic epithelioid hemangioendothelioma (HEHE) remains unclear. Laparoscopic repeat liver resection (LR) is a minimally invasive and potentially effective surgical option for multiple HEHEs.Case presentationA 42-year-old woman with no relevant history was admitted for multiple liver tumors. Six tumors were observed on T2-weighted magnetic resonance imaging (MRI) including one in S2, two in S3, two in S7, and one in S8. Pathological evaluation of percutaneous tumor biopsy tissue suggested a diagnosis of HEHE and laparoscopic LR was planned. The procedure began with partial resection of S7 and partial resection of S8 and left lateral sectionectomy were performed. Another tumor was found intraoperatively on the surface of S6, necessitating removal by partial resection. Pathological evaluation of the resected tumor tissue from all seven tumors concurred with that of the preoperative biopsy. The patient was discharged on postoperative day 6 without any complications. A follow-up MRI 15 months after the primary surgery revealed one tumor each in S4, S6, and S8. Laparoscopic repeat LR was performed. The patient was discharged on postoperative day 5 without any complications. All three recurrent tumors were pathologically confirmed as HEHEs.ConclusionsWe successfully treated primary and recurrent HEHEs with laparoscopic LR, which is a reasonable minimally invasive procedure considering the possibility of multiple courses of liver surgery in patients with HEHE.

Imaging findings of hepatic epithelioid hemangioendothelioma and fibrolamellar hepatocellular carcinoma: a critical appraisal of current literature about imaging features of two rare liver cancers.

Hepatic epithelioid hemangioendothelioma (HEHE) and fibrolamellar hepatocellular carcinoma (HCC) are rare malignant neoplasms of the liver. Contrarily to HCC, no distinctive imaging criteria based on contrast enhancement pattern are known for these neoplasms, thus they are frequently misdiagnosed as other liver tumors. Due to their infrequency, very few data principally based on reports of few patients are currently available in literature. This results in several issues pending on the application of imaging in the diagnosis of these cancers, which absolutely needs pathological examination. Nevertheless, imaging techniques are fundamental in the diagnostic work-up and in the management of affected patient. This review summarizes the current evidence about imaging systems in the diagnosis of HEHE and fibrolamellar carcinoma (FLC), discussing the significance of the principal iconographic hallmarks and the prospective potentials of the most employed techniques.

MicroRNA profile of hepatic epithelioid hemangioendothelioma: A case report.

A 72-year-old female was referred for further evaluation of epigastralgia. Abdominal contrast computed tomography revealed numerous tumors in the two lobes of the liver. Liver biopsy and immunohistochemical staining revealed that the tumor cells were positive for factor VIII-associated antigen, platelet endothelial cell adhesion molecule 1 and human hematopoietic progenitor cell antigen, concordant with a diagnosis of hepatic epithelioid hemangioendothelioma (HEH). To elucidate the etiology of HEH, particularly the microRNA (miRNA) profiles, tissue samples obtained from normal and tumor tissues were analyzed using a miRNA array system. A total of 14 miRNAs were significantly upregulated and 93 miRNAs were downregulated in the tumor tissues (P<0.01). Additionally, unsupervised hierarchical clustering analysis using Pearson's correlation revealed that the tumor tissues clustered separately from the normal tissues. The miRNA expression profile was analyzed in HEH and compared with angiosarcoma, which exhibits histology similar to HEH. Out of a total of 107 miRNAs, only miR-122-5p and miR-1290 demonstrated a differential expression pattern in angiosarcoma. Therefore, these miRNAs may be novel biological markers for the determination of a diagnosis of HEH in primary mesenchymal tumors of the liver. To the best of our knowledge, this study is the first report of a miRNA microarray analysis in HEH.

The study on hepatic epithelioid hemangioendothelioma by contrast-enhanced ultrasound

Objective To investigate the value of contrast enhanced ultrasonography(CEUS) in the diagnosis of hepatic epithelioid hemangioendothelioma(HEHE). Methods The images of CEUS had been retrospectively evaluated in 21 lesions of HEHE which were confirmed by pathology. The perfusion patterns and time of enhancement were observed,including time to begin enhancement,time to peak enhancement,the washout time and the appearance of internal structures. Results The mean time of begin enhancement,time to peak,time to isoechogenity and hypoechogenity were (17.4±5.2)s,(22.0±7.2) s,(23.9±4.6)s and (42.2±13.7)s,respectively. During the arterial phase,8 lesions showed diffuse enhancement,7 lesions showed rim-like enhancement and 6 lesions showed branch enhancement. The inner margin in 6 lesions showed speculate enhancement. The central portion of the tumors was not enhanced in part lesions of 8 cases. As to time of peak enhancement,compared to the normal hepatic parenchyma,hypoenhancement,isoenhancement and hyperenhancement were observed in 10 lesions,6 lesions and 5 lesions respectively. Synchronous enhancement was observed in 11 lesions on CEUS,rapid enhancement was observed in 8 lesions and slow enhancement was observed in 2 lesions. Twenty-one lesions manifested hypoechoic mass in portal phase and delayed phase. The boundary of lesions was unclear on conventional ultrasound and became distinct after contrast injection in 21 lesions. Conclusions CEUS is useful for the diagnosis of HEHE. Key words: Contrast-enhanced ultrasound; Liver neoplasms; Hemangioendothelioma,epithelioid

Hepatic epithelioid hemangioendothelioma: a report from three university centers.

ObjectiveTo determine common imaging findings of hepatic epithelioid hemangioendothelioma on magnetic resonance images.Materials and MethodsA search was made of three institutional databases between January 2000 and August 2012. Seven patients (mean age, 47 years; range, 21-66 years; 6 women) with pathology-confirmed diagnosis of hepatic epithelioid hemangioendothelioma who had undergone magnetic resonance imaging were identified. None of the patients had received any treatment for hepatic epithelioid hemangioendothelioma at the time of the initial magnetic resonance imaging examination.ResultsHepatic epithelioid hemangioendothelioma tumors appeared as focal masses in 7/7 patients, greater than 5 in number, with a coalescing lesion in 1/5, and peripheral localization in 6/7. Capsular retraction was present in 4/7, and was associated with peripherally located lesions. Early ring enhancement was appreciated in the majority of lesions in 7/7 patients. Centripetal progressive enhancement was shown in 5/7 patients on venous phase that exhibited a distinctive thick inner border of low signal on venous phase images, and a central core of delayed enhancement. Small lesions did not show this.ConclusionThe combination of multifocal round-configuration lesions that are predominantly peripheral and exhibit early peripheral ring enhancement and late appearance of an inner thick border of low signal and central core of high signal may represent an important feature for hepatic epithelioid hemangioendothelioma.

Hepatic Epithelioid Hemangioendothelioma Complicated by Esophageal Variceal Bleeding

Case Report: A 39-year-old male presented with four episodes of melena. History was significant for metastatic hepatic epithelioid hemangioendothelioma (HEH) status-post three cycles of trans-arterial chemoembolization (TACE) three years prior. Patient denied significant NSAID use. He denied abdominal pain, dysphagia, unintentional weight loss, or change in appetite. He had no previous GI bleeding and no history of esophageal or gastric varices. Vital signs were stable. Physical exam demonstrated a well-nourished male with no icterus. Abdomen was soft with mild tenderness to palpation over the right upper quadrant. Liver span was 14 cm. There was no ascites. Rectal exam demonstrated melenic, hemepositive stool. The CBC demonstrated a microcytic anemia with hemoglobin of 9.8. Platelets were within normal limits, and INR was 1.4. Basic metabolic panel was within normal limits except for a BUN of 21. AST, ALT and bilirubin were normal, but alkaline phosphatase was 372. Viral hepatitis panel was unremarkable. A recent CT scan of the abdomen and pelvis showed multiple, stable hepatic lesions, consistent with the known diagnosis of HEH. A subsequent esophagogastroduodenoscopy revealed moderate to large distal esophageal varices with red whale signs. Band ligation was performed, and an octreotide drip and non-selective beta-blocker were started. Abdominal ultrasound showed a patent portal vein. He was discharged on day #7 of his hospitalization with no further episodes of bleeding. Discussion: Epithelioid hemangioendothelioma is a rare malignant neoplasm of vascular origin that may affect the soft tissue, lung, bone, brain, and liver. First described in 1982, its estimated incidence is less than 1 in 1,000,000. The etiology of primary HEH is unclear. Clinical manifestations are variable. Liver transplant can be curative in the absence of metastatic disease. Treatment for extra-hepatic disease is not clear due to limited experience with HEH and its unpredictable course. Both TACE and surgical intervention have been described. Portal hypertension may arise in HEH patients as tumor cells infiltrate the vasculature of the portal system. Post-TACE portal hypertension is transient and likely due to increased portal blood flow and endothelial dysfunction. While TACE may influence portal hemodynamics, the incidence of significant varices is variable. Bleeding is seen in approximately 1.5-8% of patients at 17-month follow up with most occurring 3 to 6 months post-TACE. Our patient, with a remote history of TACE (3yrs) presented with acute blood loss anemia due to variceal bleeding, likely as a result of significant tumor burden. This case demonstrates a unique presentation of an uncommon entity.Figure 1Figure 2

Hepatic epithelioid hemangioendothelioma: Pitfalls in the diagnosis on fine needle cytology and “small biopsy” and review of the literature

Hepatic epithelioid hemangioendothelioma is a rare vascular neoplasm with an unpredictable malignant potential. Different therapeutic options are available, depending on the basis of disease extension and the patient's overall condition. A correct pathological diagnosis is necessary and is often based on scant material. Here, we report a case diagnosed on fine needle aspiration and on a small surgical biopsy. In addition, we will review the literature. The patient is a 54-year-old woman who presented with persistent pain in the right hypochondrium and suffered from weight loss. Ultrasound examination and CT scan showed several focal and confluent hepatic lesions. Thus, an ultrasound-guided fine-needle aspiration (US-FNA) was performed. A cytological diagnosis of vascular proliferation with epithelioid component was performed. Afterwards, a hepatic “small biopsy” (SB) was made. Histological and immunohistochemical data were consistent with a hepatic epithelioid hemangioendothelioma diagnosis. The patient, however, is in good general condition and is waiting for a hepatic transplantation; repeated total CT scan showed no signs of metastasis.The literature was reviewed in order to define the pathological features that were helpful in the cytological and histological diagnosis of hepatic epithelioid hemangioendothelioma, and to better understand if pathological data is prognostically useful.

Hepatic Epithelioid Hemangioendothelioma: Vascular Penetration in the Tumor as a Characteristic Imaging Finding

Primary Hepatic Epithelioid Hemangioendothelioma (HEH) is a rare, low-grade, malignant hepatic neoplasm. Here we present the typical CT and MRI features of HEH in a 35 year old young woman, which were confirmed by needle biopsy. The most significant CT and MRI imaging findings were capsular retraction and peripheral location with slow progression. In addition, there were multiple hypermetabolic liver tumors seen on FDG-PET/CT and hepatic arterial penetration of the tumor on Dynamic CT (DCT), which may be useful in the diagnosis of HEH.

Hemangioendotelioma epitelioide hepático: un desafío diagnóstico para el médico radiólogo

We report a case of a patient referred to our institution presenting with 1 year of abdominal pain, associated to the finding of multiple solid liver lesions suggestive of metastases. Diagnosis of hepatic epithelioid hemangioendothelioma was performed after percutaneous liver biopsy.We describe clinical and imaging features of this tumor. Imaging findings are analized, providing a new case of hepatic epithelioid hemangioendothelioma. Since this is a rare entity that must be taken into account when multiple liver lesions are detected, it is important to recognize it and include it in the differential diagnosis of liver metastases.

Pitfalls in the diagnosis of hepatic epithelioid hemangioendothelioma by FNA and needle core biopsy

Epithelioid hemangioendothelioma (EHE) of the liver is an extremely rare tumor that masquerades as an epithelial neoplasm and poses significant diagnostic pitfalls for the cytopathologist. This report describes a 64-year-old woman whom on computerized tomography was found to have multiple peripherally calcified hypodense lesions throughout the liver and an ipsilateral adrenal mass. Fine needle aspiration and needle cores biopsies of the largest liver lesion showed epithelioid cells and spindle cells in fibrous stroma. The epithelioid cells had round or oval, hyperchromatic nuclei with smooth nuclear contours, delicate cytoplasm and indistinct cytoplasmic borders. Rare cells had intracellular vascular lumen containing fragmented or intact red blood cells. The cells were individually dispersed or arranged in nests and vague tubular arrays. No mitotic figures or necrosis were seen. An immunohistochemical profile demonstrated diffuse CD31 and focal CD 34 and nuclear Fli-1 immunoexpression and low Ki-67 proliferative activity (1%) within lesional cells confirming the diagnosis of EHE. The differential diagnosis of EHE which includes cholangiocarcinoma, hepatocellular carcinoma, metastatic carcinoma and melanoma is discussed.

Hemangioendotelioma epitelióide hepático: um caso de rápida progressão

Hepatic Epithelioid Hemangioendothelioma (HEH) is a malignant vascular tumor, however its aggressive behavior is variable. Its signs and symptoms are not specific and its identification is incidental. The heterogeneity in the imagiologic exams difficult its diagnosis and this is only established in the histological examination. Its treatment is not standardized and is sometimes controversial. The authors present the clinical case of a male patient in the fifth decade of life, with the diagnosis of HEH with pulmonary and bone metastasis, with a rapid clinical deterioration that determined his death in a period of time inferior to 2 months.

Diagnostic and Therapeutic Challenges in Hepatic Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is a very rare, low-grade vascular tumor known to arise in soft tissues and visceral organs. Clinical diagnosis of hepatic epithelioid hemangioendothelioma remains a challenge, and although it is frequently managed with a liver transplant due to its multifocal nature, recurrence is a common complication. We review recent advances in the diagnosis of hepatic epithelioid hemangioendothelioma, including major genetic breakthroughs, and discuss efforts to reduce post-liver transplant recurrence of hepatic epithelioid hemangioendothelioma.

Hepatic epithelioid hemangioendothelioma in needle biopsy specimens: report of 5 cases with review of literature

To evaluate the pathologic diagnosis of hepatic epithelioid hemangioendothelioma (EH) in needle biopsy specimens. Five cases of hepatic EH diagnosed in needle biopsies encountered during the period from 1999 to 2010 in Beijing Cancer Hospital were retrospectively reviewed. The specimens were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study was also carried out. All the 5 patients were females. The age ranged from 23 to 47 years (mean = 39 years). The tumors in 4 patients were multiple and diagnosed as "metastasis" on ultrasound examination. The blood test results in all of the 5 patients were normal. Histologically, the tumor cells had an epithelioid appearance and were arranged in cords, solid nests or isolation, amongst a myxoid or hyaline matrix. The tumor cells contained scattered intracytoplasmic vacuoles which sometimes harbored red blood cells. There was no evidence of significant cellular pleomorphism, high mitotic activity and necrosis. Immunohistochemically, all of the 5 cases were positive for at least two endothelial markers (CD31, CD34 and factor VIII-related antigen). Smooth muscle actin was expressed in 1 case. The diagnosis of hepatic EH can be established in needle biopsy specimens. The histologic pattern, when coupled with immunohistochemical findings, is useful in arriving at the correct diagnosis.

Bright‐dark ring sign in MR imaging of hepatic epithelioid hemangioendothelioma

We describe the imaging findings in a rare case of multifocal nodular form of hepatic epithelioid hemangioendothelioma on ultrasound (US) and dynamic contrast-enhanced spiral computed tomography (CT) and MRI. The nodules showed multilayered target appearance on MR images with prominent peripheral rim with high signal intensity (SI) on T1-weighted and very low SI on T2-weighted images, corresponding to thrombosed vascular channels. The above combination of imaging findings is to our knowledge distinct and should suggest the diagnosis of hepatic epithelioid hemangioendothelioma.