Primary Localized Amyloidosis of the Intestine: A Pathologist Viewpoint

BACKGROUND: Localized amyloidosis of the intestine is a rare entity, which can clinically masquerade several conditions such as colitis, polyps, and malignant tumors. This study aims to evaluate the clinicopathological features of this entity. METHODS: To evaluate the clinicopathological features of this entity, a comprehensive search of the literature (1960 to 2019) was done using the following keywords: “amyloidosis” and “small intestine” or “duodenum” or “ileum” or “jejunum” or “colon”. We identified 756 studies about gastrointestinal amyloidosis. Data were examined for 27 studies about localized intestinal amyloidosis. The clinicopathological features were described. RESULTS: The age at presentation ranged from 29 to 88 years. The male to female ratio was 3:1. The jejunum and sigmoid colon were the most commonly involved sites. Abdominal pain and intestinal obstruction (small intestine), or rectal bleeding (sigmoid region) were the most common clinical presentations. Colonoscopic findings included wall thickening, mucosal ulcerations (small intestine), and tumor-like masses (colon). CONCLUSIONS: The clinical presentations of localized intestinal amyloidosis depend on the site of the deposition of the amyloid. In most cases, amyloid deposits consisted of light chain protein.