Hemangioendothelioma is borderline or intermediate type of vascular neoplasm. Hemangioendothelioma is rare lesion that constitutes less than 0.5% of the malignant tumors of bone. We present a case of low-grade hemagioendothelioma of the skull in a 29-yr-old woman. She had pain, diplopia and exophthalmos of the left eye. Radiographic images showed a relatively well-demarcated, expansile osteolytic lesion with irregularly thickened trabeculae and calcifications in the left greater wing of sphenoid bone. Histologically, the tumor was an infiltrative vasoformative lesion. The vessels are generally well-formed with open or compressed lumina surrounded by endothelial cells showing mild atypia. It lacked frequent mitotic figures and severe atypia. Although excessive bleeding occurred during the operation, the mass was totally resected. Postoperative radiation was not necessary. She is free of disease and well 6 months postoperatively.
Adult ; Bone Neoplasms/*pathology/radiography ; Female ; Hemangioendothelioma/*pathology/radiography ; Human ; Pregnancy ; Sphenoid Bone/*pathology

An epithelioid hemangioma involving three contiguous bones in continuity has, to the best of our knowledge, not been reported in the literature. A case of a 48-year-old man presented with radiating pain to the lower thoracic region for two years. A radiograph and CT scan revealed both permeative osteolytic and multiple trabeculated lesions involving the left posterior part of the 10th rib as well as the 9th and 10th vertebral bodies in continuity and was misled as a malignant or infectious lesion. The histopathology and immuno-histochemistry of the lesion confirmed the diagnosis of an epithelioid hemangioma. The lesion was still stable as of three years after surgery.
Bone Neoplasms/pathology/*radiography/surgery ; Diagnosis, Differential ; Hemangioendothelioma, Epithelioid/pathology/*radiography/surgery ; Humans ; Male ; Middle Aged ; Ribs/pathology/*radiography/surgery ; Spinal Neoplasms/pathology/*radiography/surgery ; Thoracic Vertebrae/pathology/*radiography/surgery ; *Tomography, X-Ray Computed

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 study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.

METHODSFive cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study. The clinical, radiologic, pathologic and immunohistochemical features of the tumors were reviewed.

RESULTSThe patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma. Four of the cases affected long bones and the remaining case affected the clavicle. One case showed multifocal involvement of the humerus. Radiologically, the tumors showed borderline to low-grade bony destruction, with various degrees of cortical defect. Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case. The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation. Immunohistochemically, the tumor cells were positive for CD31 (5/5), CD34 (5/5), vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen.

CONCLUSIONSPrimary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts. Comparison of the biologic behavior requires long-term follow-up studies.

Actins ; metabolism ; Antigens, CD34 ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Child ; Clavicle ; pathology ; Diagnosis, Differential ; Female ; Femur ; pathology ; Hemangioendothelioma ; diagnostic imaging ; metabolism ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Humerus ; pathology ; Immunohistochemistry ; Infant ; Kasabach-Merritt Syndrome ; diagnostic imaging ; metabolism ; pathology ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Radiography ; Sarcoma, Kaposi ; diagnostic imaging ; metabolism ; pathology ; Vimentin ; metabolism

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