Primary hemangioendothelioma of liver; report of a case and review of literature (original) (raw)
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Hepatic epithelioid hemangioendothelioma: review of three cases
Clinical Imaging, 2010
Objective: To determine common imaging findings of hepatic epithelioid hemangioendothelioma on magnetic resonance images. Materials and Methods: A 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. Results: Hepatic 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. Conclusion: The 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: a report from three university centers
Radiologia Brasileira, 2016
Objective: To determine common imaging findings of hepatic epithelioid hemangioendothelioma on magnetic resonance images. Materials and Methods: A 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. Results: Hepatic 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. Conclusion: The 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: a report of six patients
2010
Epithelioid hemangioendothelioma (EH) was first reported by Weiss and Enzinger in 1982 1 and was defined as a unique borderline tumor characterized by the composition of an epithelioid, endothelial or dendritic cell. 2 EH is not specific to soft tissue and has been reported in other organs such as the lung, 3,4 liver, 2 bone, 5,6 brain, 7,8 heart, 9 salivary gland, 10 vein 11,12 and pleura. 13 Hepatic EH (HEH) is a rare tumor that was reported first by Ishak et al. in 1984. 2 Here, we report six clinical cases of epithelioid hemangioendothelioma of the liver. CASE PRESENTATIONc dd_447 254..258 General conditions and clinical manifestations From 2003 to 2008, six HEH patients confirmed by pathology were treated in our hospital. Their general information, including the site and size, symptoms and signs of HEH are listed in Table 1.
Annals of International Medical and Dental Research, 2020
Hepatic Epithelioid Hemangioendothelioma (HEHE) is a very rare vascular tumor of epithelioid origin. This tumor is composed of epithelioid cells and dendritic. HEHE tumor growth leads to hepatic failure and later on death but this tumor is less aggressive as compared to the other tumors of the liver. We present a case in which a 32 years old female patient admitted with complaints of left side abdominal pain with dyspepsia over 5-6 days. She was then referred to the medical Radio-diagnosis Department for Ultrasonography (USG) and Contrast-enhanced computed tomography (CECT) scan. The diagnosis of USG revealed that a complex heterogeneous lesion with an internal cystic component was seen in the left lobe of the liver and heterogeneously enhancing lesion with the non-enhancing necrotic area, non-enhancing cystic lesion, and minimally enhancing lesion was seen in contrast-enhanced computed tomography (CECT). The clinical diagnosis was confirmed as HEHE and histopathologically correlation was done to confirm the diagnosis.
Pathology - Research and Practice, 2015
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.
Oncology letters, 2014
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Whether HEHE in Chinese patients exhibits similar characteristics compared with Western patients is not well known. The aim of the present study was to summarize the characteristics of HEHE in Chinese patients and identify its prognostic factors. In total, six patients diagnosed with HEHE at the Beijing Friendship Hospital between 2000 and 2012 were combined with 44 previously reported cases in China, retrieved from the literature between 1989 and mid-2012. These 50 cases from China were compared with 402 patients from Western populations. Prognostic factors were identified by the χ(2) test and Cox regression analysis. The male to female ratio of the Chinese patients was 1:2.1 with the mean age of 44.2 years (range, 22-86 years). The percentage of asymptomatic Chinese patients was significantly higher than in the Western patients (40.0 vs. 24.8%; P=0.026), and that of extrahepatic metastasis (16.0 vs...
Epithelioid hemangioendothelioma of the liver
Journal of Hepato-biliary-pancreatic Surgery, 2006
Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. We present two cases of this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Both our cases are highly illustrative of the two ways in which hepatic EH can present (nodular or diffuse) and of its diagnostic and therapeutic management. Neoplastic cells expressed the factor VIII-related antigen, CD31 or CD34. Treatment was surgical resection in one patient and liver transplant in the other. Although EH of the liver has a better prognosis than other hepatic neoplasms, conservative treatment is not recommended. Our cases highlight the importance of a histological diagnosis to avoid it being mistaken for another entity.
Translational gastroenterology and hepatology, 2023
Background: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplastic disease of varied presentation and unspecific radiological signs in the early stages. The diagnostic delay can lead to metastatic disease, thus increasing the tumor burden and reducing the treatment options. HEHE is usually deemed a slow-growing tumor, but its speed of growth is poorly reported and still unknown. Case Description: In this case report, we documented a HEHE diagnosed in a young woman who had complaints of abdominal pain, weight loss and bloating for a long time. The typical findings observed in histological studies were not promptly recognized in the histological analyzes, even after two laparoscopicguided liver biopsies, delaying the diagnosis until extrahepatic tumor spreading. Findings observed in computed tomography, magnetic resonance imaging and histological studies are presented. The coalescence of nodules and the rising of giant masses, occupying large parts of the liver in a specific time span, were registered and quantified. As opposed to prior reports, the results show that hepatic HEHE can grow rapidly, reinforcing the need of early diagnosis, thus avoiding the complications presented herein. Conclusions: The findings observed via radiological and histological imaging that could have avoided the diagnosis delay are depicted and discussed, showing that HEHE can rise faster than previously documented.
Malignant Hepatic Epithelioid Hemangioendothelioma with Rapid Progression and Fatal Outcome
Internal Medicine, 2010
A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.