Splenosis of the liver: a unique clinicopathologic challenge

We report two rare cases of intrahepatic splenosis in patients with distant histories of abdominal trauma and highlight the diagnostic challenges splenosis can pose for clinicians, radiologists, and pathologists.

Pathologic and clinical data for the patients were obtained from our institutional and referral records.

Patient 1 is a 44-year-old male with a history of heavy alcohol use who presented with chest pain. Imaging study revealed alcoholic steatosis and multiple liver lesions. Patient 2 is a 53-year-old female with a history of primary biliary cholangitis and chronic liver disease who was found to have multiple hepatic lesions during routine follow-up. Imaging studies in both cases raised concern for both benign and malignant processes. Biopsies demonstrated prominent congested thin-walled vascular structures, scant fibrous stroma, and background lymphocytic infiltrates. Immunohistochemistry (IHC) for ERG, CD31, and CD8 highlighted the endothelial cells of the vascular structures, phenotypically consistent with littoral cells of splenic sinuses. Further review of the clinical history revealed remote traumatic motor vehicle accidents resulting in splenectomy in both patients. After clinical, radiologic, and pathologic correlation, the diagnosis of splenosis was made in both cases.

Our cases highlight the distinctive clinical history and characteristic histologic and immunophenotypic features essential for diagnosing splenosis.