PDF(Pubmed)
Abstract：
Langerhans cell histiocytosis (LCH) of the stomach is rare. Moreover, it is usually found in pediatric patients with systemic diseases and may be associated with a poor prognosis. Solitary gastric LCH in adults is extremely rare and is often misdiagnosed or missed. The aim of our study was to review cases of gastric LCH and explore the characteristics of the disease further. A retrospective study of all patients admitted with solitary gastric LCH was conducted between 2013 and 2023. Clinical manifestations, endoscopic and pathological features, immunophenotypes, and molecular changes were collected from medical records. We examined four cases (one female, three males) of gastric LCH. The affected patients were between 33 and 70 years of age. Endoscopically, three patients presented with a solitary polyp or elevated lesions, whereas one patient showed no abnormalities. Under a microscope, all cases showed abnormal proliferation of histiocytoid cells infiltrating in a nested or sheet-like fashion. The tumor cells were medium-sized, with a slightly eosinophilic cytoplasm, irregular or renal-shaped nuclei, folded nuclear membranes, visible nuclear grooves, and the infiltration of inflammatory cells in the background. Immunohistochemically, all lesions expressed CD1a, S-100, langerin, and cyclinD1. One case showed diffuse BRAF V600E positivity. Follow-up data were available for all patients from 4 to 36 months, and all patients were alive without recurrence or progress at the time of manuscript preparation. Combined with previously reported data, solitary adult gastric LCH is more common in male patients, most of whom are asymptomatic or exhibit only mild gastrointestinal symptoms, with a good prognosis. Endoscopy often reveals solitary polyps or protruding lesions; rare cases may progress to multifocal/multisystem lesions, necessitating long-term close follow-up.
摘要：
胃的朗格汉斯细胞组织细胞增生症（LCH）很少见。此外，它通常在患有全身性疾病的儿科患者中发现，并且可能与不良预后有关。成人单发胃LCH极为罕见，常被误诊或漏诊。我们研究的目的是回顾胃LCH的病例并进一步探讨该疾病的特征。在2013年至2023年期间，对所有单发胃LCH患者进行了回顾性研究。临床表现,内窥镜和病理特征，免疫表型,和分子变化是从医疗记录中收集的。我们检查了四例(一名女性，三个男性）胃LCH。受影响的患者年龄在33至70岁之间。内窥镜检查,三名患者表现为孤立性息肉或隆起性病变，而一名患者没有表现出异常。在显微镜下,所有病例均显示组织细胞样细胞异常增殖，呈巢状或片状浸润。肿瘤细胞是中等大小的,有轻微嗜酸性的细胞质，不规则或肾形细胞核，折叠的核膜，可见的核沟，以及背景中炎性细胞的浸润。免疫组织化学，所有病变均表达CD1a，S-100Langerin,和cyclinD1。1例弥漫性BRAFV600E阳性。所有患者的随访数据均为4至36个月，所有患者在手稿制备时都活着，没有复发或进展。结合以前报告的数据，单发成人胃LCH在男性患者中更为常见，大多数人无症状或仅表现出轻微的胃肠道症状，预后良好。内镜检查常显示孤立性息肉或突出病变；罕见病例可进展为多病灶/多系统病变，需要长期密切跟进。
