No abstract available.
Adult ; Antigens, CD31/metabolism ; Antigens, CD34/metabolism ; Female ; Hemangioendothelioma, Epithelioid/metabolism/*pathology/radiography ; Humans ; Immunohistochemistry ; Liver Neoplasms/metabolism/*pathology/radiography ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; Tomography, X-Ray Computed ; von Willebrand Factor/metabolism

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