Primary localized gastric amyloidosis: A scoping review of the literature from clinical presentations to prognosis

Abstract

Localized gastric amyloidosis (LGA) is a rare disease characterized by abnormal extracellular deposition of amyloid protein restricted to the stomach and it is confirmed by positive results of Congo red staining. Over decades, only a few cases have been reported and studies or research focusing on it are few. Although LGA has a low incidence, patients may suffer a lot from it and require proper diagnosis and management. However, the pathology of LGA remains unknown and no overall review of LGA from its presentations to its prognosis has been published. Patients with LGA are often asymptomatic or manifest atypical symptoms, making it difficult to differentiate from other gastrointestinal diseases. Here, we report the case of a 70-year-old woman with LGA and provide an overview of case reports of LGA available to us. Based on that, we conclude current concepts of clinical manifestations, diagnosis, treatment, and prognosis of LGA, aiming at providing a detailed diagnostic procedure for clinicians and promoting the guidelines of LGA. In addition, a few advanced technologies applied in amyloidosis are also discussed in this review, aiming at providing clinicians with a reference of diagnostic process. With this review, we hope to raise awareness of LGA among the public and clinicians.

A 65-Year-Old Woman with an Enlarged Tongue Due to Amyloidosis

“…Amyloidosis is a general diagnosis with various forms, including localized (eg, brain in Alzheimer disease, genito-urinary tract, or skin) and systemic involvement (eg, light chain and amyloid A protein deposits in multiple organs) [ 2 ]. In general, localized amyloidosis tends to be non-life-threatening in nature, and more likely to be driven by immunoglobulin light chain amyloidosis (AL) deposition [ 7 – 9 ]. Based on the affected organs and severity, amyloidosis signs and symptoms vary and can include edema, fatigue, weight loss, nephropathy, cardiomegaly or interstitial pulmonary edema [ 2 ].…”

Localized gastric amyloidosis successfully treated with endoscopic submucosal dissection

“…Twenty two cases of localized gastric amyloidosis have been reported to date. [ 10 ] The endoscopic findings of gastric amyloidosis are very different depending on the type of protein and the depth of deposition. It can show thickening of the mucosal folds, increased mucosal fragility, erosion, ulcers, and submucosal tumors.…”

“…Single digestive organ deposition in amyloidosis is very rare. Twenty two cases of localized gastric amyloidosis have been reported to date [10] . The endoscopic findings of gastric amyloidosis are very different depending on the type of protein and the depth of deposition.…”

Systemic AL amyloidosis: current approach and future direction

“…Localized AL Amyloidosis is generally present in a single organ, such as the lungs, part of the bowel, upper airways, or skin along with the absence of the monoclonal protein in the plasma and absence of clonal plasma cells in the bone marrow [ 15 – 17 ]. Localized AL amyloidosis is not treated by systemic therapy since the long-term prognosis is favorable, and not complicated with systemic AL amyloidosis and is generally managed by local resection [ 18 ]. While a monoclonal gammopathy of unknown significance (MGUS) may develop to systemic amyloidosis or multiple myeloma with an average rate of transformation of 1% per year [ 19 , 20 ], finding of AL amyloidosis in bone marrow is not an indication for systemic treatment in the absence of the organ involvement.…”