Definition
Littoral Cell Angioma (LCA) is a benign vascular neoplasm that is unique to the spleen. Its tumor cells exhibit a distinct immunophenotype that combines endothelial and histiocytic features. Etiology and pathogenesis of LCA are unknown, but the enigmatic association of LCA with various malignancies is so strong that all LCA patients should be thoroughly screened and followed for concomitant cancers.
Malignant counterparts of LCA, littoral cell hemangioendothelioma and littoral cell angiosarcoma have been described, as well as combined tumors featuring LCA and malignant littoral cell tumor components.
Clinical Features
Incidence and clinical symptoms
The true incidence of LCA is not known. First described in 1991 (Falk et al. 1991) about 200 LCA cases have been published to date. Tumors combining LCA and malignant littoral cell tumor features are exceedingly rare (Ben-Izhak et al. 2001).
Most LCA are found incidentally by imaging studies as single or multiple, hyperdense...
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References
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Marx, A., Rüdiger, T. (2019). Littoral Cell Angioma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3830-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3830-1
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