Is littoral cell angioma of the spleen as rare as previously believed in the pediatric population?

Abstract

Littoral cell angioma (LCA) is a rare primary splenic vascular tumor, originating from the littoral cells lining the red pulp sinuses of the spleen. There are only a handful of case reports of LCA in children to be found in the literature. We performed a retrospective analysis of the medical charts of pediatric patients with splenic lesions who were treated between 2005 and 2010 in the Pediatric Surgery Department of the Medical University of Bialystok. Surprisingly, LCA accounted for 37.5% of the splenic lesions found in our series. The majority of LCA tumors are benign, but given their malignant potential, splenectomy and long-term follow-up should be the gold standard for their management. We strongly support the use of further cross-sectional studies to properly elucidate the prevalence of littoral cell angioma of the spleen in the pediatric population. (Folia Histochemica et Cytobiologica 2012, Vol. 50, No. 3, 480-485)

Littoral cell angioma: A rare cause of pediatric thrombocytopenia

“…Only a handful of pediatric cases have been reported thus far. [ 1 3 4 5 ] LCA is usually asymptomatic and is only discovered incidentally. Clinically, LCA may be characterized as a solid mass occurring most often along with splenomegaly, thrombocytopenia, or anemia, as well as a fever of unknown origin.…”

Littoral cell angioma of the spleen: a study of 25 cases with confirmation of frequent association with visceral malignancies

“…4 The Ki67 index tends to be very low (<1%), as repeatedly described in previous reports. 1,9,10,30 Littoral cells show dual endothelial and histiocytic differentiation, and are considered to be different from common endothelial cells. The immunoprofile of positive staining for factor VIII, CD31, ERG, CD68, CD163, CD21, cathepsin D, and vimentin, accompanied by an absence of staining for CD34, CD8, and…”

A Rare Case of Splenic Littoral Cell Angioma in a Child

“…Splenic hamartomas are rare benign vascular proliferations that are discovered incidentally or during autopsy. [ 3 ] Recently, based on specific immunohistochemical properties, a new variety of splenic vascular tumor has been defined. Because of the limited number of cases and case reports, the etiology and natural course of LCA are not completely understood.…”

“…The majority of the vascular tumors of the spleen display delayed intensity images similar to LCA. [ 3 10 11 ] MR scanning shows iso- and hyper-intense lesions on T1- and T2- weighted imaging's, respectively. In some cases, LCAs provide hypointense images even after gadolinium administration in both T1- and T2- weighted imagings.…”

“…Reports of LCAs, especially the solitary form in children are very rare. [ 3 ] Herein, we present the pathologic and radiologic findings of a solitary LCA in the spleen of an 11-year-old girl as a very rare case report in the literature.…”