Lymphocyte-rich capillary-cavernous hemangioma of the mitral valve: a case report and review of the literature.

Valvular hemangioma incidence is extremely low. In this report, we describe a 62-year-old man who presented with mild edema of the lower limbs. An echocardiogram revealed an incidental 1.3-cm diameter mass on the anterior mitral valve leaflet for which he underwent surgical resection and mitral valve replacement. Histopathological examination showed a lymphocyte-rich capillary-cavernous hemangioma. The exuberant lymphoid stroma is unusual for hemangioma and represents an undescribed pattern of cardiac hemangioma. Including the present report, only 13 cases of mitral valve hemangioma have been reported to date. Most patients are adult. Mitral hemangioma originates in the atrial aspect of the valve and involves more commonly the anterior leaflet. The average maximum diameter of the lesion is 1.7 (S.D.=0.75) cm. Pure cavernous hemangioma is the predominant type of mitral hemangioma. Most of them are described as pedunculated or polypoid. Surgical excision appears to be curative. Recurrences have not been reported. Lymphocyte-rich cardiac hemangioma represents a peculiar type of hemangioma which should be included in the differential diagnosis of other vascular lesions.

[Littoral cell angioma of spleen: a clinicopathologic study of 17 cases].

OBJECTIVE: To study the clinicopathologic features and immunophenotype of splenic littoral cell angioma. METHODS: The clinical features, radiologic findings and histopathology of 17 cases of splenic littoral cell angioma were retrospectively reviewed. Immunohistochemical study was carried out on paraffin-embedded tissues, using normal spleen, cases of congestive splenomegaly and cavernous hemangioma as controls. RESULTS: All the 17 cases had similar clinical manifestations and radiologic findings. There was mild to moderate splenomegaly, with solitary or multifocal space-occupying lesions. Hepatic cysts were observed in 5 of the 17 cases. One case was also accompanied by serous cystadenoma of ovary. Gross examination revealed enlarged spleen containing single or multiple tan-colored nodules which ranged from 0.2 cm to 6.0 cm in diameter. Histologically, the lesions consisted of anastomosing vascular channels, sometimes with papillary or cavernous appearance. Two types of component cells were identified. A population of smaller cells lined the vascular channels, while another population of larger cells often floated in the vascular lumen. Both cell populations showed little cytologic atypia. Immunohistochemical study demonstrated that the smaller cells of all cases were positive for CD31 and polyclonal factor VIII-related antigen. They were negative for CD34, histiocytic markers and S-100 protein. CD8 and CD21 were expressed in 1 and 1 of the 17 cases respectively. On the other hand, the larger cells expressed histiocytic markers, including CD68 (KP1 and PG-M1), CD163 and lysozyme. There was also focal positivity for CD31. The staining for CD34, monocolonal factor VIII-related antigen and S-100 protein was negative. The immunophenotype of splenic littoral cell angioma was different from that of the controls. CONCLUSIONS: Littoral cell angioma is a benign condition, likely secondary to hemodynamic disturbance in spleen. The littoral cells become hyperplastic and anastomose, resulting in a hemangioma-like growth associated with histiocytic reaction. Attention to the characteristic histopathologic findings and immunophenotype are crucial for diagnosis.

Proliferating cell nuclear antigen (PCNA) in cerebral cavernomas: an immunocytochemical study of 42 cases.

BACKGROUND: The natural history and growth mechanisms of cerebral cavernous angiomas are unclear, which makes them difficult to manage. We attempted to evaluate the evolutive potential of cavernomas by studying the proliferative capacity of cells. METHODS: We studied 42 histologically verified cavernomas with monoclonal antibody to proliferating cell nuclear antigen (PCNA), an accessory protein of the cell cycle, the rate of which is increased in proliferative cells. The PCNA Labeling Index (PCNA LI) was calculated in each case, and the results were compared with histologic findings (lacy areas, thick walls, thrombi, hemosiderin) and clinical features (epilepsy, hematomas, pseudotumorous signs). RESULTS: Thirty-six of 42 cases (85.7%) revealed stained cells. PCNA LI ranged from 1 to 48% (mean: 23.39%). Statistical analyses showed a positive correlation between PCNA LI and the extent of lacy areas (p < 0.05). On the contrary, collagenous-walled and thrombotic areas rarely showed positively stained cells. We found no relationship between PCNA LI and clinical features. CONCLUSIONS: A proliferative capacity of endothelial cells does exist in some areas of cavernomas and may explain, besides thromboses and hemhorrages, the growth and even de novo appearance of these lesions. Occurrence of fragile blood cavities, thickening of others, and changes in blood flow may influence the evolution of lesions. Our results suggest that in cavernomas, some areas may undergo specific changes, which makes them more dynamic lesions than previously thought.

Monoclonal antibody H3/5-47 recognizes an inducible cell surface antigen expressed differently in endothelium of normal and diseased tissues and in vitro.

Monoclonal antibody H3/5-47 was raised against a human melanoma metastasis and recognizes an antigen expressed in the endothelial cells of all normal human organs as assessed by immunohistochemistry. Antigen expression is higher in venous than in capillary or arterial endothelia; capillary endothelia of different microvascular beds, such as skin, lung, gut or liver, may express varying amounts of this antigen. H3/5-47 antigen expression in the endothelia of diseased tissues (inflammatory diseases, neoplasias) largely reflects its expression pattern in normal tissues. As might be anticipated, the highest expression of H3/5-47 antigen is found in resting adult cutaneous and hepatic cavernous venous hemangiomas. In contrast, psoriatic vessels, characterized by hypertrophy and fenestrations, tend to express H3/5-47 antigen at a much lower density. In human umbilical vein endothelial cells, half the single donor cases show no expression of H3/5-47 antigen, while the rest express the antigen at relatively low densities in about half the cells. Treatment with interferon-gamma or thrombin, but not interleukin-1, lipopolysaccharide, endothelial cell growth factor or phorbolester, either enhances or induces de novo expression in cultured human umbilical vein endothelial cells within 24h; maximum expression of H3/5-47 antigen is induced by interferon-gamma within 72 h. H3/5-47 antigen is not similar to other antigens inducible in human umbilical vein endothelial cells such as HLA-DR, ICAM-1, HECA-452, Leu13, MCP-1 or gamma-IP-10. It is not specifically expressed in the endothelium as it may also recognize certain epithelia, peripheral nervous tissue and bone marrow-derived cell populations.(ABSTRACT TRUNCATED AT 250 WORDS)

[Histochemical study using lectin and anti-human von Willebrand factor antibody of oral hemangioma].

Twenty nine cases of oral hemangiomas composed of capillary hemangioma, cavernous hemangioma and pyogenic granuloma were examined with lectin histochemistry using ulex europaeus agglutinin I, ricinus communis agglutinin I, wheat germ agglutinin, concanavalin A, dolichos biflorus agglutinin, soybean agglutinin and peanut agglutinin, and immunohistochemistry using anti von Willebrand factor antibody in order to understand the nature of each endothelial cell. The following results were obtained; 1. UEA-I that specifically bound alpha-L-fucose was observed mainly in the endothelial cells of capillary hemangioma and hemangioma of granulation tissue type. 2. RCA-I which had specific affinity to beta-D-galactose was so identified in all endothelial cells of all type hemangiomas and normal blood vessels that it was supposed to be a useful marker of the cells. 3. WGA with specific affinity to N-acetyl-glucosamine was also found in various endothelial cells. 4. Con A which had specific affinity to alpha-D-mannose was not observed in various endothelial cells. 5. DBA, SBA and PNA that were specific bound up with N-acetyl-galactosamine were seen in the normal micro-vessels, especially in subepithelial regions. 6. Immunohistochemically, von Willbrand factor was identified chiefly in the endothelial cells of normal blood vessels and cavernous hemangioma, but it was not found in the cells of capillary hemangioma and pyogenic granuloma which had no differentiation in the functional point of view.

Giant cavernous hemangioma of the liver and multiple primary malignant tumors in a patient with suspected familial inhibition of natural killer cell activity--a case report.

A woman was operated on for a nonepithelial malignant tumor of the left leg and subsequently, for an epithelial carcinoma of the right breast and a borderline malignant tumor of the right ovary. She also developed a giant cavernous hemangioma that caused disseminated intravascular coagulation syndrome, which necessitated a left trisegmentectomy of the liver. Her family history suggested a hereditary predisposition to diverse malignant neoplasms, and also to giant cavernous hemangioma of the liver. Immunological evaluation disclosed selective inhibition of natural killer cell activity. Hormonal and hereditary factors are discussed in relation to the development of multiple primary tumors and giant cavernous hemangioma of the liver.

Blood group antigens in vascular tumours. Evaluation of the immunoperoxidase technique.

Determination of blood group isoantigens A, B and H, was performed in benign and malignant vascular tumours of the skin and subcutaneous tissue, using the immunoperoxidase technique. No differences were noted between benign haemagioendotheliomas from children or adults: neither tumour showed the presence of antigens in intercapillary cells. Reactive conditions such as angiolymphoid hyperplasia with eosinophilia showed an intense positive reaction to blood group substances of the endothelial proliferative cells. In malignant tumours no relationship between tumour differentiation and loss of blood group isoantigens was seen. Cases of Kaposi's sarcoma did not show antigens in spindle cells or capillaries but in medium sized vessels variable preservation or loss of blood group isoantigens was found.