Littoral cell angioma as a rare cause of splenomegaly

Abstract

A 58-year-old, otherwise healthy man presented with a sudden onset of watery diarrhea. A pseudomembranous colitis due to antibiotics was identified as the cause of the diarrhea. Enlargement of the spleen was detected during the evaluation. The enlarged, plump spleen (20 cm long, 7.1 cm wide) had multiple nodules that differed in size from 1 to 8 cm. Neither clinical nor other symptoms of an underlying malignant disease could be detected. Because the signs were of little diagnostic value we arranged a splenectomy, which showed a littoral cell angioma (LCA) to be the cause of splenomegaly. In addition to the case report, we have reviewed the literature, clinical manifestations, differential diagnosis, special gross and microscopic pathological findings, and the location of this benign vessel tumor in the pathology of the spleen.

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

“…At present, the final diagnosis is only possible via histopathological examination [5,6,[12][13][14]. The lesions may be solitary or multiple, and are composed of vascular channels lined by cells that share the morphological and immunohistochemical findings characteristic for both the endothelial and histiocytic cells…”

Vasoformative Disorder, Resembling Littoral Cell Angioma, of the Spleen in a Geriatric Japanese Macaque(Macaca fuscata)

“…6 The neoplasm generates from littoral cells of the red pulp sinuses; the cells have features that lie intermediately between vascular endothelial cells and macrophages/histiocytes, and the cells display higher lysosomal activity than vascular endothelial cells. 4,21 The activity may be confirmed by hemosiderin deposition or hematophagocytosis. 21 Pathologic diagnosis is based on neoplastic proliferation of anastomosing vascular channels rimmed by cells reacting immunohistochemically to both endothelial cell and macrophage markers.…”

“…4,16 Here, we describe a macaque case of a vasoformative lesion of the spleen, the pathologic characteristics of which bore a resemblance to those of littoral cell angiomas of the spleen in humans. 3,5,6,21 In veterinary literature, there have been no reports of littoral cell angiomas in domestic and experimental animals. 7,19 Splenic vasoformative disorders in humans are often accompanied by a disturbance of blood coagulation.…”

“…6 The littoral cells are considered to be unique lining cells possessing both vascular endothelial and macrophage-like properties. 3,5,6,11,21 Splenic hemangiomatosis consists of vascular endothelial cells forming cavernous and capillary vessels but lacks a macrophagelike nature of the neoplastic cells. 4,16 Here, we describe a macaque case of a vasoformative lesion of the spleen, the pathologic characteristics of which bore a resemblance to those of littoral cell angiomas of the spleen in humans.…”

“…1,4,19 In addition, in humans, littoral cell angioma and hemangiomatosis (angiomatosis) have been recorded as vasoformative disorders of the spleen. 4,6,12,13,16,21 The littoral cell angioma, a benign vasoformative tumor, originates from the sinus endothelial cells, called littoral cells, of the splenic red pulp. 6 The littoral cells are considered to be unique lining cells possessing both vascular endothelial and macrophage-like properties.…”