Littoral cell angioma of the spleen: An additional report of four cases with emphasis on the association with visceral organ cancers

Aims and background: Littoral cell angioma (LCA) is an uncommon vascular tu mor of the spleen recently described and interpreted as the tumoral counter part of the normally present littoral cells lining the splenic sinus channe ls of red pulp. The diagnosis of LCA is suggested by a quite characteristic morphology and confirmed by the demonstration of a hybrid endothelial/hist iocytic phenotype. Methods: Four original and previously unreported cases o f LCA are presented. All four splenic vascular tumors were investigated by light microscopy and immunohistochemistry for endothelial and histiocytic m arkers. Results: All four cases were associated with visceral epithelial ma lignancies (colorectal adenocarcinoma in two cases, renal and pancreatic ad enocarcinoma in one case each). One case was also associated with an intrac ranial tentorial meningioma. Conclusions: We consider our findings as a nov elty and signal the possible existence of a clinical syndrome. Five of a to tal of 21 previously reported cases in the literature were also described a s being associated with other cancers (non-Hodgkin's lymphoma in two cases, two not further specified tumors of the liver and brain, an epithelial ova rian cancer, and a non-small cell lung cancer in one case each). Close foll ow-up and careful investigation in search of a second visceral neoplasm are strongly recommended in cases of LCA, but further clinical observations an d more in-depth genetic and molecular studies are needed before any valid c onclusions can be drawn.