Localized gastric amyloidosis differentiated histologically from scirrhous gastric cancer using endoscopic mucosal resection: a case report
© Kamata et al.; licensee BioMed Central Ltd. 2012
Received: 19 January 2012
Accepted: 18 April 2012
Published: 3 August 2012
Amyloidosis most often manifests as a systemic involvement of multiple tissues and organs, and an amyloidal deposit confined to the stomach is extremely rare. It is sometimes difficult to provide a definitive diagnosis of localized gastric amyloidosis by biopsy specimen and diagnosis of amyloidosis in some cases has been finalized only after surgical resection of the stomach.
A 76-year-old Japanese woman with epigastric discomfort underwent an esophagogastroduodenoscopy procedure. The esophagogastroduodenoscopy revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, at the greater curvature from the upper to the lower part of the gastric corpus. A biopsy specimen revealed amyloid deposits in the submucosal layer with no malignant findings. We resected a representative portion of the lesion by endoscopic mucosal resection using the strip biopsy method to obtain sufficient tissue specimens, and then conducted a detailed histological evaluation of the samples. The resected specimens revealed deposition of amyloidal materials in the gastric mucosa and submucosa without any malignant findings. Congo red staining results were positive for amyloidal protein and exhibited green birefringence under polarized light. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL) amyloid protein type. Based on these results, gastric malignancy, systemic amyloidosis and amyloid deposits induced by inflammatory disease were excluded and this lesion was consequently diagnosed as localized gastric amyloidosis. Our patient was an older woman and there were no findings relative to an increase in gastrointestinal symptoms or anemia, so no further treatment was performed. She continued to be in good condition without any finding of disease progression six years after verification of our diagnosis.
We report an unusual case of primary amyloidosis of the stomach resembling scirrhous gastric carcinoma. This case of localized gastric amyloidosis was differentiated from scirrhous gastric cancer after performing endoscopic mucosal resection without an invasive surgical resection, as endoscopic mucosal resection provided sufficient tissue specimens from the lesion to make an accurate histological evaluation.
KeywordsEndoscopic mucosal resection (EMR) Localized gastric amyloidosis Scirrhous gastric cancer
The term amyloidosis refers to a group of disorders characterized by extracellular accumulation of insoluble, fibrillar proteins in various organs and tissues. Amyloidosis most often manifests as systemic involvement of multiple tissues and organs, and an amyloidal deposit confined to the stomach has been extremely rare in previously published reports. It is sometimes difficult to provide a definitive diagnosis of localized gastric amyloidosis by biopsy specimen and diagnosis of amyloidosis in some cases was definitively made only after surgical resection of the stomach.
A 76-year-old Japanese woman presented to our facility with epigastric discomfort; she had previously undergone an esophagogastroduodenoscopy (EGD) procedure at another hospital. The EGD had revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, so our patient was referred to our hospital for further examination and treatment.
The term amyloidosis refers to a group of disorders characterized by extracellular accumulation of insoluble, fibrillar proteins in various organs and tissues . It can result from a heterogeneous group of disorders and cause impairment or even dysfunction of involved organs . Various subtypes exist, including primary systemic amyloidosis, reactive systemic amyloidosis associated with a chronic inflammatory condition and localized forms of aberrant amyloid deposition . Amyloidosis most often manifests as systemic involvement of multiple tissues and organs including the heart, liver, spleen, kidneys, lymph nodes, adrenals and thyroid in addition to many others. The clinical implication of a single organ or tissue is relatively unusual and reports of an amyloidal deposit confined to the stomach are extremely rare in the previously published literature [2, 3].
Review of the literature on cases of localized gastric amyloidosis
Author, year and reference
Rotondano et al., 2007 
Left flank pain
Died (Other disorder)
Yoshida et al., 1998 
Ishii et al., 1993 
Wu et al., 2003 
Survival (18 months)
Nishida et al., 1990 
Deniz et al., 2006 
Middle body to antrum
Survival (two months)
Kato et al., 1988 
Died (10 months)
Ikeda et al., 1988 
Nfoussi et al., 2010 
Upper to lower body
Survival (six years)
Present case, 2012
With regard to diagnosis of scirrhous type gastric cancer, histological confirmation using biopsy specimens is also sometimes difficult; accuracy of pre-operative histological diagnosis of scirrhous type gastric cancer has ranged from 58% to 70% in previously published reports [13, 14]. In the present case, therefore, the possibility of scirrhous type gastric cancer could not be excluded because of the similarity in gross appearance despite a biopsy specimen indicating amyloid deposits with no malignant findings. Strip biopsy was developed as an EMR technique for early gastric cancer in 1984  and makes it possible to obtain a larger resected specimen compared to a biopsy specimen that enables more precise histological diagnosis. It was particularly important in this case that scirrhous-like localized gastric amyloidosis could be diagnosed by evaluating such precise histological findings from the resected specimen, thereby excluding the existence of scirrhous type gastric cancer by only using EMR without the necessity of performing an invasive surgical resection.
Primary amyloidosis refers to the disorder in patients with no preceding or co-existing disease except immunocyte dyscrasis in which the extracellular substance is composed of AL protein produced by plasma cells, as typically seen in multiple myeloma. The major forms of systemic amyloidosis also include reactive systemic amyloidosis consisting of a non-immunoglobulin protein secreted by the liver in the setting of chronic inflammatory disorders or cancers . Localized gastric amyloidosis is characterized by AL type amyloid deposition in the mucosal or submucosal layer of the gastric wall [3, 16]. In our patient’s case, deposition of AL type amyloid in the stomach was revealed by detailed histological examination of the EMR specimens, including Congo red staining with prior potassium permanganate incubation. The diagnosis of localized gastric amyloidosis was then confirmed after ruling out systemic amyloid involvement in other organs. Obtaining sufficient tissue specimens from representative changes in our patient’s lesion using EMR, therefore, made it possible to initially perform a detailed histological evaluation including differential staining of elastic fibers and subsequently confirm the localized deposition of AL type amyloid in the stomach. In addition, we were also able to exclude gastric malignancies such as gastric cancer and malignant lymphoma by evaluating those same specimens, so EMR could well become an important diagnostic method for determining localized gastric amyloidosis.
Currently, there are no published reports that mention in particular any specific therapy for localized gastric amyloidosis. Some reports have documented that surgical resection with lymph node dissection may be a preferable therapeutic strategy to prevent possible complications such as bleeding and obstruction [2, 6]. In contrast, no further treatment is deemed to be necessary in other reports if the patient is symptom free on clinical follow-up. In addition, several reports indicate periodic controls should be scheduled to follow evolution of the disease and facilitate early recognition of multi-organ involvement [3, 7]. We did not perform any treatment in this case and there were no subsequent findings of disease progression, but an evaluation involving a large number of patients should be conducted in the future to determine the proper management of localized gastric amyloidosis.
In summary, this rare case of localized gastric amyloidosis was differentiated from scirrhous gastric cancer without performing invasive surgical resection as the use of EMR provided sufficient tissue specimens from the lesion to make an accurate diagnosis of localized deposition of AL type amyloid with no malignant findings possible.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We wish to express our appreciation to Christopher Dix for his assistance in editing this manuscript.
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