Juxtaoral organs known as organs of Chievitz are intramuscular embryonic structures found close to the angle of the mandible near the insertion of the pterygomandibular raphae. They are considered of neuroepithelial origin with no known function. We describe the first tumor of the organ of Chievitz which presented intraorally in a child. Immunohistochemically, the Chievitz nests showed positive reaction for vimentin, cytokeratins, and epithelial membrane antigen and ultrastructurally demonstrated cytoplasmic processes and intermediate filament bundles. These observations, together with light microscopic features, suggest that the epithelial nests of the organ of Chievitz are meningothelial rather than neuroepithelial.
Gaucher or Gaucher-like cells are described in the spleen and bone marrow of patient with thalassemia major, by light and electron microscopy. The ultrastructure shows intracytoplasmic tubules and phagocytosis of mature and immature erythrocytes. The spleen has an increase in monohexosyl ceramide. These findings support the concept that the intracytoplasmic tubular material of the Gaucher’s cells is of extracellular origin. Erythrophagocytosis common in Gaucher’s disease, chronic myelogenous leukemia, and thalassemia suggest that impaired catabolism of erythrocytes may give rise to the increased glucocerebroside.
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