Localized amyloidosis of the stomach mimicking a superficial gastric cancer

Kagawa, Masaaki; Fujino, Yuichi; Muguruma, Naoki; Murayama, Noriaki; Okamoto, Koichi; Kitamura, Shinji; Kimura, Tooru; Kishi, Kazuhiro; Miyamoto, Hiroshi; Uehara, Hisanori; Takayama, Tetsuji

doi:10.1007/s12328-016-0651-x

Cited by 14 publications

(14 citation statements)

References 14 publications

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“…Localized AL amyloidosis accounts for approximately 12% of all AL amyloidosis cases, and the GI tract is involved in 7.7% of these cases [ 6 , 7 ]. Localized AL amyloidosis restricted to the stomach is extremely rare; clinical, endoscopic, and histopathologic findings from our review of cases in the literature are summarized in Table 1 [ 4 , 8 - 15 ]. Over 60% of patients had no symptoms and tended to remain asymptomatic throughout the observation period.…”

Section: Discussionmentioning

confidence: 99%

“…Although endoscopic ultrasound (EUS), endoscopic mucosal resection (EMR), and endoscopic submucosal dissection (ESD) were not performed in the patient discussed here, they can be used for definitive diagnosis and to rule out malignancy [ 11 - 14 ]. Because the gross appearance of this lesion usually resembles malignant lymphoma or gastric cancer, such as that observed in this case study, staining using Congo red should be performed to confirm the presence of amyloid proteins and ensure an accurate diagnosis [ 12 , 14 , 15 ]. If the lesion contains amyloid deposits, determining the extent of amyloidosis is critical [ 5 - 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, only 2% of patients with localized AL amyloidosis exhibited symptom progression, and overall survival was not different from that of the general population, except in cases involving the lungs [ 7 ]. Localized AL amyloidosis involving the stomach was also associated with a good prognosis, and long-term outcomes were excellent [ 4 , 7 - 15 ]. Therefore, if localized gastric AL amyloidosis is confirmed by histopathology, it is likely that invasive inspections, such as EUS, EMR, or ESD, are not necessary.…”

Section: Discussionmentioning

confidence: 99%

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Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Ahn¹,

Rhee²,

Choi

et al. 2018

Clin Endosc

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Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy. Immunohistochemical staining revealed the presence of kappa and lambda chain-positive plasma cells. There was no evidence of underlying plasma cell dyscrasia or amyloid deposition in other segments of the gastrointestinal tract. Echocardiography and computed tomography of the chest, abdomen, and pelvis did not show any significant findings. Thus, the patient was diagnosed with localized gastric amyloidosis with kappa and lambda light chain coexpression.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Ahn¹,

Rhee²,

Choi

et al. 2018

Clin Endosc

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“…If amyloid deposits are identified, it is important to determine whether the amyloidosis is localized or systemic. Typical sites for localized amyloidosis include the skin, 35 larynx, 36 bowel 37,38 or urinary tract, 39 which can include the renal pelvis, ureter, bladder, and urethra. Pulmonary nodules demonstrating amyloid are frequently localized deposits composed of light chains or transthyretin 40 .…”

Section: Disease Overviewmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Gertz

2020

American J Hematol

112

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Disease Overview Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or monoclonal gammopathy undetermined significance (MGUS).” Diagnosis Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy. Prognosis N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain (FLC) values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months. Therapy All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure >90 mmHg, troponin T < 0.06 ng/mL and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered cyclophosphamide‐bortezomib‐dexamethasone or daratumumab‐containing regimens as it appears to be highly active in AL amyloidosis. Future Challenges Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure.

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“…Until 1990s surgical resection was considered as proper treatment for localized gastric amyloidosis, 2,8 while after 2,000 most cases with localized gastric amyloidosis, especially symptom-free ones, maintained good condition without any treatment. 3,6,[9][10][11][12][13][14] So far, there have been no diagnostic criteria for localized gastric amyloidosis. It is only diagnosed by excluding other organ involvement.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic Localized Gastric Amyloidosis with Two Separate Lesions

Lim

Kim

Seo

et al. 2018

Korean J Helicobacter Up Gastrointest Res

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Amyloidosis is a disease in which amyloid is abnormally accumulated in the tissue. The kidney and heart are the most commonly involved organs. Gastric involvement is relatively common in systemic disease; however, localized gastric amyloidosis is rare. Here, we report a case of localized gastric amyloidosis with two separate lesions. A 56-year-old woman underwent a health surveillance checkup without any noticeable symptoms. She was under medication for diabetes and dyslipidemia, and was otherwise healthy. On surveillance upper endoscopy, an irregularly shaped hyperemic elevated erosion at the gastric fundus and a 1.5-cm, yellowish subepithelial tumor-like lesion with intact overlying mucosa at the lesser curvature of the lower body of the stomach were detected. Endoscopic biopsy revealed submucosal eosinophilic material deposition in both lesions. Congo-red staining showed amyloid deposit appearing as a yellow-green birefringence under polarizing microscopy. Echocardiography, abdominal sonography, and colonoscopy revealed no abnormality. The patient was diagnosed as having localized gastric amyloidosis and is now undergoing regular follow-up without any treatment. Localized gastric amyloidosis is a rare disease that may mimic nonspecific gastritis or subepithelial tumor. However, endoscopic biopsy with appropriate staining may be diagnostic and thorough evaluation for systemic involvement is important.

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Localized amyloidosis of the stomach mimicking a superficial gastric cancer

Cited by 14 publications

References 14 publications

Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Asymptomatic Localized Gastric Amyloidosis with Two Separate Lesions

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