Aims and background: Littoral cell angioma (LCA) is an uncommon vascular tumor of the spleen recently described and interpreted as the tumoral counterpart of the normally present littoral cells lining the splenic sinus channels of red pulp. The diagnosis of LCA is suggested by a quite characteristic morphology and confirmed by the demonstration of a hybrid endothelial/histiocytic phenotype.
Methods: Four original and previously unreported cases of LCA are presented. All four splenic vascular tumors were investigated by light microscopy and immunohistochemistry for endothelial and histiocytic markers.
Results: All four cases were associated with visceral epithelial malignancies (colorectal adenocarcinoma in two cases, renal and pancreatic adenocarcinoma in one case each). One case was also associated with an intracranial tentorial meningioma.
Conclusions: We consider our findings as a novelty and signal the possible existence of a clinical syndrome. Five of a total of 21 previously reported cases in the literature were also described as being associated with other cancers (non-Hodgkin's lymphoma in two cases, two not further specified tumors of the liver and brain, an epithelial ovarian cancer, and a non-small cell lung cancer in one case each). Close follow-up and careful investigation in search of a second visceral neoplasm are strongly recommended in cases of LCA, but further clinical observations and more in-depth genetic and molecular studies are needed before any valid conclusions can be drawn.