A case of type 1 infantile hemangioendothelioma of the liver in a 3 month old male infant who had an abdominal mass incidentally noted at 40 days of age, is described. Grossly, the tumor showed a well circumscribed, pinkish gray and gelatinous mass with areas of central necrosis, multifocal thrombi and hemorrhages, and small cysts. Microscopically, the lesion consisted of numerous vascular channels of various size lined by a single layer of flat or plump endothelial cells. Areas of infarction, thrombosis and calcification, and formation of cavernous hemangiomatous foci were also noted in the lesion. Ultrastructurally, the cells had the characteristics of endothelium, including basal lamina, pinocytic vesicles, and Weibel-Palade bodies. Factor VIII-associated antigen was demonstrated in the tumor cells.
Hemangioendothelioma/*pathology/surgery ; Humans ; Infant ; Liver Neoplasms/*pathology/surgery ; Male

Intravascular papillary endothelial hyperplasia (Masson's hemangioma) is a disease characterized by exuberant endothelial proliferation within the lumen of medium-sized veins. In 1923, Masson regarded this disease as a neoplasm inducing endothelial proliferation, however, now it is considered to be a reactive vascular proliferation following traumatic vascular stasis. The lesion has a propensity to occur in the head, neck, fingers, and trunk. Occurrence within the abdominal cavity is known to be very rare, and especially in the liver, there has been no reported case up to date. The authors have experienced intravascular papillary endothelial hyperplasia of the liver in a 69-yr-old woman, and report the case with a review of the literature.
Aged ; Angiography ; Endothelium, Vascular/pathology ; Female ; Hemangioendothelioma/*pathology/surgery ; Human ; Liver/*blood supply/*pathology ; Necrosis ; Vascular Neoplasms/*pathology/surgery

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.
Female ; Hemangioendothelioma/metabolism/*pathology/surgery ; Humans ; Infant ; Interferon-alpha/therapeutic use ; Liver Neoplasms/metabolism/*pathology/surgery ; Tomography, X-Ray Computed ; alpha-Fetoproteins/*analysis

Antigens, CD34 ; metabolism ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; surgery ; Hepatectomy ; methods ; Humans ; Liver Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Vascular Endothelial Growth Factor A ; metabolism ; von Willebrand Factor ; metabolism

Adenocarcinoma, Bronchiolo-Alveolar ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; surgery ; Hemangioma ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Lung Neoplasms ; metabolism ; pathology ; surgery ; Middle Aged ; Vimentin ; metabolism

Antibodies, Monoclonal, Murine-Derived ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioendothelioma ; metabolism ; pathology ; surgery ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; surgery ; Humans ; Lymphatic Metastasis ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma ; metabolism ; pathology ; surgery ; Thigh ; Vimentin ; metabolism ; von Willebrand Factor ; metabolism

OBJECTIVETo study the clinicopathologic features and differential diagnosis of epithelioid sarcoma-like hemangioendothelioma (ES-H).

METHODSThe clinical, radiologic and pathologic features of three cases of ES-H were analyzed.

RESULTSAll the 3 cases occurred in male adults. The age ranged from 44 to 53 years. The presentations included left neck mass, iliac pain and bilateral shoulder masses. Histologically, ES-H was composed of a mixture of spindle and epithelioid tumor cells. Transition between the two cell types was demonstrated. The tumor cells were arranged in compact sheets, vague nodules or intersecting fascicles, amongst a collagenous stroma. Central coagulative necrosis was identified in one case, reminiscent the morphology that seen in epithelioid sarcoma. There was no evidence of angiogenesis, though focal presence of intracytoplasmic vacuoles was seen in one case, as in classic examples of epithelioid hemangioendothelioma. Immunohistochemical study showed that the tumor cells expressed both epithelial (AE1/AE3, CAM5.2 and epithelial membrane antigen) and endothelial (CD31, Fli-1 and factor VIII-related antigen) markers. Two of the cases were also positive for CD34. All of the patients were treated by surgical resection. Two patients remain well at 14-month and 9-month follow up, respectively. The remaining patient had repeated local recurrences during a 6-year period.

CONCLUSIONSES-H represents a rare morphologic type of hemangioendothelioma. It has some overlapping histologic features with epithelioid sarcoma and epithelioid hemangioendothelioma. The endothelial nature of ES-H is difficult to be verified on the basis of morphologic examination alone. Confirmation of the diagnosis with immunohistochemistry is necessary. ES-H is likely related to epithelioid hemangioendothelioma and may represent a cellular spindle cell variant of epithelioid hemangioendothelioma.

Adult ; Antigens, CD34 ; metabolism ; Biomarkers ; metabolism ; Diagnosis, Differential ; Follow-Up Studies ; Hemangioendothelioma ; metabolism ; pathology ; surgery ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Humans ; Ilium ; Immunohistochemistry ; Keratins ; metabolism ; Lymphatic Metastasis ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Neck ; Neoplasm Recurrence, Local ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Proto-Oncogene Protein c-fli-1 ; metabolism ; Reoperation ; Retrospective Studies ; Sarcoma ; metabolism ; pathology ; surgery ; Shoulder ; von Willebrand Factor ; metabolism

OBJECTIVEto study the clinicopathological features, imaging characteristics, immunophenotypes and differential diagnosis of giant cell angioblastoma (GCAB).

METHODSa case of GCAB in the left middle-upper tibia and fibula was studied by light microscopy, X-ray and CT imaging, immunohistochemistry.

RESULTSX-ray and CT imaging showed a clearer lesion in the left middle-upper tibia than in the ipsilateral fibula with enlarged ostealleosis and increased inhomogeneously medullary cavity density, irregular thickening of cortical bone, local cortical default at the inner edge, soft tissue swelling around the abnormal bone. Histologically, tumor tissue was located between the bone trabeculae by nodular, linear and plexiform aggregates of oval-to-spindle cells, large mononucleate cells and multinucleate giant cells with prominent nucleoli and abundant granular eosinophilic cytoplasm. Some aggregates had uncentain amount of discernible lumens, either empty or containing few erythrocytes. A concentric arrangement of oval-to-spindle Cells around small-caliber vascular structures together with collagen fiber contributed to a so-called 'onion-skin' arrangement. The background showed a loose mesenchymal stroma formed of some inconspicuous spindle-fibroblast-like cells, stellate-shape mesenchymal cells, a moderate mononuclear inflammatory cell infiltrate and scattered mast cells. Immunophenotype showed the tumor cells and giant cells strongly positive for vimentin. A good many oval-to-spindle cells stained markedly for CD31 and CD34, but weakly for FVIII, while the giant cells are highlighted instead by CD68, occasionally, very few giant cells showed positive focally for FVIII, a-SMA decorated notedly the cells surrounding the endothelium-like cells but weakly positive in some other tumor cells.

CONCLUSIONGCAB is a rare, locally infiltrative but slow growing neoplastic angiogenesis with unique morphological characteristics during infancy, which may occur not only in the skin, mucosa, subcutis and deep soft tissue but also in the bone.

Actins ; metabolism ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Dermatofibrosarcoma ; metabolism ; pathology ; Diagnosis, Differential ; Fibula ; Giant Cell Tumor of Bone ; diagnostic imaging ; metabolism ; pathology ; surgery ; Hemangioblastoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Hemangioendothelioma ; metabolism ; pathology ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Hemangioma, Cavernous ; metabolism ; pathology ; Humans ; Infant ; Kasabach-Merritt Syndrome ; Male ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; Thrombocytopenia ; metabolism ; pathology ; Tibia ; Tomography, X-Ray Computed ; Vascular Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic features, diagnosis and differential diagnosis of epithelioid hemangioma.

METHODSThe morphologic features of 7 cases of epithelioid hemangioma of skin, bone and venous vessels were studied.

RESULTSThere were altogether 4 male and 3 female patients (median age = 34 years; age range from 14 to 54 years). The 3 skin cases presented as single or multiple erythematous to bluish nodules or papules, with or without itchiness. The 2 bone cases appeared as osteolytic expansile lesions on radiologic examination. The remaining 2 cases involved medium-sized venous structures and presented as small isolated nodules in soft tissue. Histologically, the lesions were characterized by the presence of exuberant endothelial proliferations with various degree of inflammatory reaction. The neoplastic endothelial cells were plump, eosinophilic and polygonal, forming vascular channels. Occasional solid sheet-like arrangement was demonstrated. Intracytoplasmic vacuoles were commonly identified, indicating formation of primary lumen. The surrounding stroma contained various number of eosinophils and lymphoplasmacytic cells. Immunohistochemical study showed that the tumor cells were positive for endothelial markers (CD31 and CD34) and negative for epithelial marker (cytokeratin). Follow-up information was available in 6 cases. The duration of follow-up ranged from 5 to 36 months (median = 14 months). There was no evidence of recurrence or distant metastasis.

CONCLUSIONSEpithelioid hemangioma is a rare benign curable lesion which can be multifocal, involving skin, soft tissue and bone. It needs to be distinguished from Kimura's disease and epithelioid hemangioendothelioma.

Adolescent ; Adult ; Angiolymphoid Hyperplasia with Eosinophilia ; pathology ; Antigens, CD34 ; metabolism ; Bone Neoplasms ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioendothelioma, Epithelioid ; pathology ; Hemangioma ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery