muramidase and Hemangioma

Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure.. We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor VIII related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis.. If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.

Topics: Aged; Antigens, CD; Antigens, CD34; Antigens, Differentiation, Myelomonocytic; Diagnosis, Differential; Factor VIII; Hemangioma; Humans; Male; Muramidase; Peroxidase; Splenic Neoplasms; Syndrome; Vimentin

We describe histological, immunohistochemical and ultrastructural findings in a case of littoral cell angioma of the spleen in a 44 year old man. Beside phagocytosis and heavy haemosiderin deposits in the cytoplasm, a very characteristic and hitherto undescribed feature of the littoral cells was focal accumulations of eosinophilic globules 0.5-2 microns in size, which often entirely filled the cytoplasm of the tumour cells. Ultrastructurally the globules were composed of abundant cytoplasmic deposits of lysosomes and residual bodies. The globules most probably originate from the phagocytized red blood cells, lymphocytes and plasma cells. Immunohistochemically the tumour cells reacted positively with antibodies against factor VIII-related antigen, KiM1P, KP1 and lysozyme and negatively with antibodies against cytokeratins AE1-AE3, EMA and S-100 protein. Ultrastructurally the tumour cells often formed long cytoplasmic processes without external lamina and pinocytic vesicles. Scarce and poorly formed junctions between the tumour cells were seen. Very rarely cytoplasmic rod-shaped microtubulated bodies, often difficult to distinguish from heavy accumulations of lysosomes were observed.

Topics: Adult; Antigens, Neoplasm; Hemangioma; Hemosiderin; Humans; Immunohistochemistry; Keratins; Male; Membrane Glycoproteins; Microscopy, Electron; Mucin-1; Muramidase; Phagocytosis; S100 Proteins; Splenic Neoplasms; von Willebrand Factor

This report presents the interesting case of a 50-year-old white man with an unusual benign tumor composed predominantly of a proliferation of atypical endothelial cells combined with a variable inflammatory response. This case represents an instance of the recently renamed entity "histiocytoid hemangioma" in which two organ systems are involved. Both skin and bone showed typical lesions. No physical connection jointed the separate lesions. The results of examination by light microscopy, electron microscopy, and immunoperoxidase examination for lysozyme and Factor VIII are reported. The significance of this case is that it supports the concept of classifying similar vascular lesions, despite varied organ system origin, into a single entity, the histiocytoid hemangioma.

Topics: Bone Neoplasms; Factor VIII; Hemangioma; Histiocytes; Humans; Immunoenzyme Techniques; Male; Microscopy, Electron; Middle Aged; Muramidase; Neoplasm Recurrence, Local; Radiography; Scapula; Skin Neoplasms