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

Table 1 Collection of recent case reports of localized gastric amyloidosis

Ref.	Age/sex	Symptom	Gross	Size	Location	Endoscopic ultrasound	biopsy	Amyloid type	Exclusion test	Suspected diagnosis	Treatment
Ikeda et al[59], 1978	68/F	Epigastric pain, nausea	One grayish-white mural elastic soft tumor with an irregular shape and poor margins; thickened and uneven mucosa, partly nodular; swollen mucosal folds	6 cm × 5 cm	Antrum	/	Congo red (+); amyloid deposits in vessel walls; H&E staining: Amyloid deposits with foreign-body reactions; small nodules of amyloid proteins with a scattered distribution	/	Biopsy of the skin rectum, gingiva and liver; urine Bence-Jones protein levels	Gastric carcinoma	Surgery
Dastur et al[40], 1980	50/M	Abdominal distension, worse after meals	One mass with central small ulceration and defective mucosa	/	Antrum	/	The mass extended from the mucosa to superficial muscularis and consisted of lymphocytes and germinal centers; normal plasma cells and amyloid proteins; Congo red (+)	/	Urine Bence-Jones protein (-)	/	Surgery
Björnsson et al[45], 1987	60/F	Hematemesis	A considerable amount of blood, bleeding and irregular, thickened mucosa folds	/	/	/	H&E staining: Amyloid deposits in the lamina propria and muscularis mucosae, infiltration of plasma cells, mucosa atrophy; Congo red staining: Amyloid deposits in the submucosa, muscularis propria and subserosa, mainly around vessels	AL (κ&λ)	Biopsy of rectum, gingiva, cervix and bone marrow; analysis of renal function; urine analysis	/	Surgery
Yanai et al[31], 1991	52/F	None	One irregular erosion	2.5 cm	The lower body of the stomach	Thickened mucosa and submucosa	Amyloid deposits in vessels of the mucosa and submucosa	AL (λ)	Examinations of blood, urine and skin; ultrasound and CT of the abdomen; simple X-ray examination of the chest and abdomen; biopsy of the rectum; ECG; ultrasound of the heart; barium enema; tests of antigens, urine Bence-Jones protein, rheumatoid factors and C-reactive protein levels	Gastric cancer	/
Wu et al[38], 2003	50/F	Epigastric discomfort, dull pain in the upper abdomen	One ulcer with heaped-up rough borders and erosive fragile mucosa	3 cm × 1 cm	Lesser curvature of the gastric body	Uneven hypoechoic thickened gastric wall with infiltrated submucosa	Amyloid deposits in the mucosa, submucosa and walls visualized using H&E and Congo red staining (-); atrophy of gastric glands and intestinal metaplasia	AA	Biopsy of the bone marrow and other gastrointestinal tissues (esophagus, duodenum, colon and stomach) (-)	Gastric cancer	Subtotal gastrectomy, clearance of perigastric lymph nodes
Shibukawa et al[44], 2004	51/F	Tarry stool	One irregular ulcer with swollen edges and dirty slough-like advanced cancer; bleeding	/	/	Structural loss of the first three layers of the gastric wall, a small amount of ascites	H&E staining: Amyloid deposits infiltrated the submucosal connective tissues, lamina propria, and muscularis mucosae and were mainly observed around vascular walls in the submucosa; Congo red (+)	AL or primary type	/	Carcinoma	Partial gastric resection
Deniz et al[41], 2006	67/M	Fatigue, weight loss, poor appetite	One mass	5 mm × 5 mm × 5 mm	Paracardiac region	/	H&E staining: Amyloid deposits in the mucosa; Congo red (+)	/	Biopsy of other gastrointestinal tissues (-); urine Bence-Jones protein (-)	/	/
Rotondano et al[60], 2007	55/M	Epigastric pain, heartburn, weight loss	Two white-yellow granular-like circular areas	3 cm	Distal portion of the gastric body and angle of the stomach	Mucosal and submucosal layers exhibited slight thickening	H&E staining: Lymphocytes and polyclonal plasma cells infiltrated the lamina propria; Congo red (+)	/	Biopsy and endoscopy of the rectum, duodenum and esophagus (-)	/	None
Ebato et al[62], 2012	77/F	Anemia	One flat, depressed area	46 mm × 28 mm	Lower gastric body	/	H&E staining: Amyloid deposits in the mucosa and submucosa; DFS staining (+)	AL	/	/	Endoscopic removal
Sawada et al[46], 2012	72/F	/	Flat elevations, tumors; ulcers resemble advanced cancer, intramural hematomas	/	Scattered distribution in the antrum, proximal and middle stomach	Structural loss, thickened hypoechoic mucosa and submucosa	Congo red (+)	AL (κ&λ)	Biopsy and endoscopy of other gastrointestinal tissues (-)	/	/
Rivera et al[42], 2012	67/M	Melena, anemia	One round and erosive mass with errhysis	2.5-3 cm	Cardia	/	Confirmed amyloidosis	/	Biopsy of bone marrow (-)	Gastric adenocarcinoma	Surgery, hematology consultations
Kamata et al[36], 2012	76/F	Epigastric discomfort	Multiple swollen and reddish folds with a hemorrhagic and erosive mucosa	/	Greater curvature of the gastric body	Thickened submucosal layer	Amyloid deposits in the submucosa and mucosa, Congo red (+)	AL	Biopsy of the rectum and ileum (-); Bence-Jones protein (-); echocardiography (-)	Gastric carcinoma	None
Jin et al[61], 2014	33/F	Epigastric pain, dyspepsia, heartburn, acid reflux	One area with irregular borders and a hemorrhagic mucosa; another area with normal borders and smooth surfaces	1.2 cm × 1.2 cm; 10 mm × 20 mm	Lesser curvature of the gastric body; gastric fundus	Hypoechoic thickened stratum mucosum and lamina muscularis protruded in the lesser curvature; nonechoic lesions in the fundus	Amyloid deposits detected from the submucosa to muscularis propria and around small blood vessels using H&E staining; Congo red (+); van Gieson staining (-)	/	/	/	ESD; DMSO
Yamaguchi et al[47], 2015	49/M	/	One elevated lesion similar to a submucosal tumor	15 mm	Greater curvature of the lower body	A hypoechoic mass with hyperechoic spots in the submucosa and the muscular layer	Amyloid deposits in the submucosa; Congo red (+)	AL	Biopsy of other tissues in the gastrointestinal tract (-)	/	/
Kobara et al[37], 2015	80/M	Epigastric discomfort	One granular, elevated lesion	20 mm	Posterior wall of the prepyloric ring	A hypoechoic mass in the submucosa	Congo red (+)	AL	/	/	/
Kagawa et al[32], 2016	73/M	None	One pale, depressed area with clear borders	15 mm	The anterior wall of the lower gastric body	/	H&E staining: Amyloid deposits in the lamina propria and submucosa; Congo red (+)	AL	CT of the chest, abdomen and pelvis (-); urine Bence-Jones protein (-); electrocardiogram, echocardiography	/	None
Ahn et al[33], 2018	55/F	None	One pale, round, central-depressed area with irregular and heaped-up edges	20 mm	Lesser curvature of the mid-gastric body	/	Lymphoplasmacytes and Congo red (+) staining in the lamina propria	AL (κ&λ)	Biopsy of colon and duodenum (-); echocardiography (-); CT of chest abdomen and pelvis (-); antineutrophilic antibodies, rheumatoid factors, serum immunoglobulin and components, antinuclear antibodies and urine Bence-Jones protein	cancer	None
Ding et al[53], 2018	54/M	None	One well-defined lesion with irregularly distorted vessels	/	/	Thinned superficial mucosa, thickened deep mucosa	Congo red (+); H&E: Amyloid deposits in the mucosa	/	/	Early gastric cancer	Surgery
Kinugasa et al[34], 2018	64/M	None	One submucosal tumor with a hard elastic character	40 mm	Middle body of the greater curvature	In the second and third layers of the mucosa	Congo red (+) staining in the mucosal propria	AL (λ)	Bone marrow biopsy; biopsy of other gastrointestinal tissues; ultrasound and CT of the liver, kidney and heart	Myoma, malignant lymphoma, gastrointestinal submucosal tumor	/
Savant et al[43], 2018	64/M	/	One mass	3.6 cm	/	One hypoechoic mass in the muscularis propria	Congo red (+); H&E staining: Amyloid deposits with a foreign-body giant cell reaction	AL (λ)	CT, urine analysis and serology (-)	Gastrointestinal stromal tumor	/
Matsueda et al[35], 2019	59/M	None	Multiple pale and depressed lesions	/	Through the stomach	/	Congo red (+)	AL	Biopsy of other gastrointestinal tissues; Bence-Jones protein (-); ultrasound and CT	Healing gastric ulcer	/
Present case	70/F	Hematemesis	Multiple congestive erosions; one area of the mucosa with edema and ulcers exhibited unclear boundaries	4.0 cm × 4.0 cm	The anterior wall of the gastric body and fundus	/	Congo red (+); H&E staining: Massive amyloid fibrous connective tissues deposited in the interstitium with inflammatory cell infiltration	/	CT of the liver, colon, kidney; HRCT of the lung; ultrasound of the liver, heart, and kidney	/	None

ESD: Endoscopic submucosal dissection; DMSO: Dimethyl sulfoxide; CT: Computed tomography; ECG: Electrocardiograph; AL: Light chain; AA: Amyloid A; PFS: Progression-free survival.