Systematic Review: Littoral Cell Angioma of the Spleen

Background: Littoral Cell Angioma is a rare vascular tumor of spleen mostly diagnosed incidentally and though have malignant potential; may often run a benign course. Given its rarity, it is of utmost significance to throw more light onto the clinical features, diagnostic approaches, therapeutic interventions and outcomes associated with LCA which has been served through this systematic review of existing literatures. Method: A cumulative total of 215 records was identified via database searches. Data extraction was carried out and the quality of case reports and case series was evaluated using the Joanna Briggs Institute (JBI) critical appraisal tools. Following the elimination of duplicates and the screening of titles and abstracts, 34 studies qualified for comprehensive full-text evaluation. Finally, 21 studies, encompassing a total of 85 cases of Littoral Cell Angioma, satisfied the inclusion criteria and were incorporated into the systematic review and reported in accordance with the PRISMA guidelines. Results: Out of the 85 cases of LCA, it was found that there was a slight female preponderance with incidental detection predominating in majority of the cases. Total splenectomy was preferred against other surgical options. HPE and IHC forms the mainstay of the diagnosis. Overall prognosis is good except for rare malignant transformation. Discussion: This review highlights the need for heightened awareness of LCA among clinicians, especially when evaluating splenic masses. Further research is necessary to identify the molecular pathways underlying malignant transformation and to develop potential biomarkers for early detection.