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Littoral cell angiomas (LCA) of the spleen are vascular tumors of unknown etiology arising from the littoral cells of the splenic red pulp sinuses. Usually a benign and incidental finding, LCA have been repeatedly reported in association with a variety of visceral malignancies and hold the potential for dissemination per se. We encountered a case of a 30 year old female who was diagnosed with solid pseudopapillary tumor of the head and distal pancreas by fine needle aspiration cytology. A distal pancreatectomy with splenectomy was performed in addition to a pylorus-preserving Whipple's procedure and cholecystectomy. Histopathological examination confirmed solid pseudopapillary tumor of the pancreas and showed multiple well-circumscribed anastomosing vascular channels in the spleen. The diagnosis of LCA of the spleen was confirmed by immunohistochemistry that revealed co-expression of endothelial cell marker, CD31 and CD34, along with histiocytic marker, CD68 by the vascular lining cells. LCA has been previously reported in association with colorectal and pancreatic adenocarcinoma, malignant lymphoma, myelodysplasia and autoimmune disorders. We report the first case of LCA associated with solid pseudopapillary tumor of the pancreas.

Littoral cell angiomas of the spleen associated with solid pseudopapillary tumor of the pancreas