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

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