Appendiceal malakoplakia: a review of the literature and case presentation

Background∕Objectives: Malakoplakia is an uncommon clinicopathological entity defined by the accumulation of dysfunctional macrophages containing undigested bacterial remnants and pathognomonic Michaelis-Gutmann bodies. Although it most frequently involves the gastrointestinal tract, particularly the colon, rectum, and rectosigmoid junction, appendiceal involvement remains rare. This case represents only the fourth reported occurrence of appendiceal malakoplakia in a young, immunocompetent adult without notable comorbidities, underscoring its exceptional clinical presentation. Study selection for review: A PubMed search was performed to explore the current understanding of malakoplakia, with emphasis on its etiopathogenesis, diagnostic criteria, and therapeutic strategies. Case presentation: A 25-year-old immunocompetent male presented with a periappendicular abscess mimicking a tumor, ultimately requiring right hemicolectomy. Histopathological (HP) evaluation confirmed intestinal-appendicular malakoplakia and granulomatous lymphadenitis, with no evidence of tuberculosis, underscoring the diagnostic challenge and the need for further investigation into its etiopathogenesis in young, otherwise healthy individuals. Conclusions: This case illustrates a rare instance of appendicular malakoplakia in a young adult, emphasizing the importance of HP evaluation for accurate diagnosis and the need to consider this entity in the differential diagnosis of appendiceal masses to avoid mismanagement.