Synchronous occurrence of littoral cell angioma and multiple primary malignancies – A rare case report

Littoral cell angioma (LCA) is a rare primary vascular neoplasm of the spleen that originates from the endothelial littoral cell lining of the red pulp sinuses. LCA has mostly benign behavior with a few reported cases of malignancy. LCA is strongly associated with diverse immunologic and neoplastic disorders. We report a case of LCA with synchronous renal cell carcinoma, adrenal cortical adenoma and JAK2 EXON 14 mutation positive Polycythemia vera. Histologically, entire spleen showed a neoplasm with anastomosing vascular channels lined by plump round cells. Diagnosis was confirmed by the endothelial/histiocytic phenotype of tumor. To the best of our knowledge, this case is the first description of LCA associated with renal cell carcinoma, adrenal cortical adenoma and MPN.