Xanthogranulomatous appendicitis: A comprehensive literature review

Abstract

Background: Xanthogranulomatous inflammation is characterized histologically by a collection of lipid-laden macrophages admixed with lymphocytes, plasma cells, neutrophils, and often multinucleated giant cells with or without cholesterol clefts.

Aim: To review the medical literature on xanthogranulomatous appendicitis (XGA).

Methods: We present a patient with XGA and review published articles on XGA accessed via the PubMed, MEDLINE, Google Scholar, and Google databases. Keywords used were "appendix vermiformis," "appendectomy," "acute appendicitis," and "XGA." The search included articles published before May 2020, and the publication language was not restricted. The search included letters to the editor, case reports, review articles, original articles, and meeting presentations. Articles or abstracts containing adequate information about age, sex, clinical presentation, white blood cells, initial diagnosis, surgical approach, histopathological and immunohistochemical features of appendectomy specimens were included in the study.

Results: A total of 29 articles involving 38 patients with XGA, were retrospectively analyzed. Twenty (52.6%) of the 38 patients, aged 3 to 78 years (median: 34; IQR: 31) were female, and the remaining 18 (47.4%) were male. Twenty-five patients were diagnosed with acute appendicitis, ruptured appendicitis, or subacute appendicitis, and the remaining 13 patients underwent surgery for tumoral lesions of the ileocecal region. Twenty-two of the patients underwent urgent or semi-urgent surgery, and the remaining 16 patients underwent interval appendectomy.

Conclusion: Xanthogranulomatous inflammation rarely affects the appendix vermiformis. It is associated with significant diagnostic and therapeutic dilemmas due to its variable presentation. It is often associated with interval appendectomies, and a significant number of patients require bowel resection due to the common presentation of a tumoral lesion. XGA is usually identified retrospectively on surgical pathology and has no unique features in preoperative diagnostic studies.

Keywords: Acute appendicitis; Appendectomy; Appendix vermiformis; CD68 antibody staining; Interval appendectomy; Xanthogranulomatous inflammation.

Figure 1

Fibrous obliteration of appendix vermiformis (arrow head), acute and chronic inflammatory cell infiltration (arrow) within the appendix wall and subserosal fatty tissue (HE × 10).

Figure 2

Xanthogranulomatous inflammation (a mixture of macrophages, lymphocytes, plasma cells, and neutrophils) (HE × 50).

Figure 3

Macrophages showing positive staining for CD68 antibody.