成人胃孤立性朗格汉斯细胞组织细胞增生症1例并文献复习OA

Objective To investigate the clinical features,endoscopic manifestations,pathological characteristics,treatment and prognosis of isolated Langerhans cell histiocytosis(LCH)in adults.Methods The clinical data of one adult patient with isolated gastric LCH were retrospectively analyzed.Relevant literatures published in PubMed,China National Knowledge Infrastructure(CNKI),and Wanfang Databases were retrieved to summarize the clinical features,endoscopic manifestations,pathological characteristics,treatment,and prognosis of this disease.Results A 61-year-old female patient was admitted to hospital due to intermittent epigastric pain for 1 month,which worsened over for 15 days.Gastroscopy revealed a congestive and erosive lesion on the greater curvature of the gastric antrum.Pathological examination showed focal and patchy distribution of abnormally proliferated Langerhans cells in the lamina propria of gastric mucosa.The cells were medium-sized with slightly eosinophilic cytoplasm,oval nuclei,indistinct nucleoli and visible nuclear grooves.Inflammatory cells including eosinophils,lymphocytes,and plasma cells were observed in the background.Immunohistochemistry demonstrated positivity for CD1α,S-100,Langerin,and Cyclin D1;CD68 was also positive;p53 showed positivity in scattered individual cells and the Ki-67 proliferation index was 20%.A follow-up gastroscopy 2 months later revealed near-complete healing of the original erosive lesion on the greater curvature of the gastric antrum,with scattered erosive lesions observed at the greater curvature of the antral-body junction.The literature review identified 28 reported cases of isolated gastric LCH in adults,with a male predominance and a mean age of 45 years.Endoscopically,elevated or polypoid lesions were the main manifestations.Pathology combined with immunohistochemistry serves as the gold standard for diagnosis,with positive expression of CD1α,S-100,Langerin,and Cyclin D1.Conclusions Isolated gastric LCH is a rare disease with non-specific clinical symptoms,and diverse endoscopic manifestations,and it presents a favorable prognosis.Endoscopists and pathologists should make a definite diagnosis combined with pathological and immunohistochemical findings to avoid missed diagnosis and misdiagnosis.