Hepatic epithelioid hemangioendothelioma: a report of six patients (original) (raw)
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Epithelioid Hemangioendothelioma of the Liver—Case Report
Journal of Pharmacy and Pharmacology 6 (2018) 153-156
EH (Epithelioid hemangioendothelioma) is a rare vascular malignant neoplasm. Only 434 cases had been reported until 2006. Symptoms may be nonspecific, but it also can involve hepatic veins resulting in Budd-Chiari syndrome. The main differential diagnosis is cholangiocarcinoma, sometimes only distinguishable through immunohistochemistry study. We report a case of a young woman diagnosed with hepatic EH and listed for liver transplantation.
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.
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: 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.
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.
Hepatic Epithelioid Hemangioendothelioma and the Danger of Misdiagnosis: Report of a Case
Case Reports in Oncological Medicine, 2013
Malignant hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant tumor of vascular origin. Nonspecific symptoms and the absence of experience of surgeons, radiologists, and histopathologists due to the rarity of HEHE make the diagnosis of this entity very challenging. Misdiagnosis is not a rare event, and the consequences of such an event are catastrophic. We report a case of a patient suffering from HEHE in which the initial diagnosis was hepatocellular carcinoma (HCC). The presence of normal laboratory values, liver function tests, tumor markers along with the absence of a chronic liver disease, or any other predisposing factors for HCC, was in contrast with the diagnosis of HCC. Clinical suspicion drove us to the repetition of a liver biopsy and the reevaluation of the sample by a more experience histopathology department in liver tumors. The last biopsy confirmed the diagnosis of HEHE, and the patient escaped any unnecessary treatment for a nonexisting HCC.
Hepatic epithelioid hemangioendothelioma: A great mimicker
International Journal of Surgery Case Reports, 2018
INTRODUCTION: Epithelioid hemangioendothelioma is a malignant mesenchymal tumor of unknown etiology. They tend to be asymptomatic or with non-specific symptoms. The lesion is usually multiple and variable size. PRESENTATION OF CASE: We describe a clinical case of a 23-years-old patient diagnosed with a pelvic mass, a possible uterine fibroid or adnexal mass, and multiple liver lesions that seemed an advanced ovarian cancer presentation and after liver biopsy turned out to be a hepatic epithelioid hemangioendothelioma. DISCUSSION: It may be confused with a metastatic process in diagnostic imaging. There have been described some possible risk factors but the etiology remains unknown. The prognosis is usually lethal in 50% of cases. The surgical removal of the lesion and liver transplant appear to be the only hope for these patients. CONCLUSION: Epithelioid hemangioendothelioma must be part of our differential diagnosis when we find a liver tumour, especially in young women. Treatment is excision of the tumour in limited disease. In the case of unresectable disease are candidates for liver transplantation.