Epithelioid hemangioendothelioma is a neoplasm of vascular origin with a low-to-intermediate malignant potential and is one of the rare sarcomas arising from the liver. Its etiology is unknown and its clinical outcome is unpredictable. There is no generally accepted therapeutic strategy because of its rarity and the variable natural course between hemangioma and angiosarcoma. We report a case of a 64-year old woman who underwent hepatic resection due to epithelioid hemangioendothelioma in the right lobe that progressed to extrahepatic metastases of the bone, pleura, and peritoneum 22 months later. However, after resection there was no primary hepatic recurrence.
Bone Neoplasms/diagnosis/pathology/secondary ; Female ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/*secondary ; Hepatectomy ; Humans ; Liver Neoplasms/*diagnosis/pathology ; Lung Neoplasms/diagnosis/pathology/secondary ; Middle Aged ; Tomography, X-Ray Computed

Epithelioid hemangioendothelioma is a rare vascular origin tumor which usually occurs in soft tissues, liver, and lung. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It is difficult to diagnose and treat because of non-specific clinical manifestations and findings on the imaging study. Moreover, pathological misdiagnosis is common. We report a case of this rare tumor that was detected incidentally. Final diagnosis was based on histological evidence. A 52-years old man suffered from right upper quadrant abdominal pain for 3 months, and was initially misdiagnosed as a metastatic carcinoma. Physical examination revealed superior cervical lymphadenopathy with mild hepatomegaly. Finally, hepatic epithelioid hemangioendothelioma was diagnosed on the basis of positive immunohistochemical staining for factor VIII, CD34, and VEGF. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.
Antigens, CD34/analysis/immunology ; Carcinoma/secondary ; Diagnosis, Differential ; Factor VIII/analysis/immunology ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology ; Humans ; Immunohistochemistry ; Liver Neoplasms/*diagnosis/pathology ; Male ; Middle Aged ; Positron-Emission Tomography

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.
Antigens, CD34/metabolism ; Bone Neoplasms/diagnosis/secondary ; Diagnosis, Differential ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/ultrasonography ; Humans ; Liver Cirrhosis, Alcoholic/complications/diagnosis ; Liver Neoplasms/*diagnosis/pathology/ultrasonography ; Male ; Pancreatic Neoplasms/diagnosis/secondary ; Rupture, Spontaneous ; Tomography, X-Ray Computed

Epithelioid hemangioendothelioma is a rare vascular tumor, which occurs in the lung, liver, bone, and soft tissue. Hypertrophic osteoarthropathy is a syndrome characterized by subperiosteal new bone formation, joint effusion and clubbing, and may be associated with cyanotic heart disease, chronic pulmonary disease, liver disease, and other miscellaneous diseases. The activation of endothelium and platelets has been suggested to be involved in the development of hypertrophic osteoarthropathy. We report a rare case of hypertrophic osteoarthropathy, which developed in association with hepatic epithelioid hemangioendothelioma with pulmonary metastasis. We also discuss the role of vascular endothelial growth factor in its pathogenesis.
Adult ; Biopsy ; Blood Platelets/metabolism ; Endothelium/metabolism ; Femur/radiography ; Hemangioendothelioma, Epithelioid/*complications/*pathology ; Human ; Lung/pathology ; Lung Neoplasms/*complications/pathology ; Male ; Osteoarthropathy, Secondary Hypertrophic/*complications/*pathology ; Vascular Endothelial Growth Factor A/metabolism ; Vascular Neoplasms/*diagnosis/pathology

OBJECTIVETo evaluate the pathologic diagnosis of hepatic epithelioid hemangioendothelioma (EH) in needle biopsy specimens.

METHODSFive 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.

RESULTSAll 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.

CONCLUSIONSThe 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.

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Biopsy, Needle ; Carcinoma, Signet Ring Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Hemangioendothelioma, Epithelioid ; diagnostic imaging ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Liver Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Melanoma ; metabolism ; pathology ; secondary ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Radiography ; Retrospective Studies ; Young Adult ; von Willebrand Factor ; metabolism