Mora Linda B, Hough Morgan, Moscinski Lynn, Gomez Justin, Coppola Domenico
Department of Pathology, Florida Digestive Health Specialists, Lakewood Ranch, FL, U.S.A.
Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, U.S.A.
Cancer Diagn Progn. 2023 Jan 3;3(1):102-106. doi: 10.21873/cdp.10186. eCollection 2023 Jan-Feb.
BACKGROUND/AIM: Langerhans cell histiocytosis (LCH) is an uncommon disorder characterized by an abnormal monoclonal proliferation of pathologic Langerhans cells. The clinical presentation of LCH is very unpredictable, ranging from single-system limited disease to severe multi-organ disease with a high mortality rate. LCH usually affects children and very rarely adults. The most common body parts affected by LCH are the bones, skin, lungs, pituitary glands, and lymph nodes. Gastrointestinal tract involvement by LCH is exceptionally rare, and only a few cases have been reported.
We present the case of a 50-year-old woman who was referred to our clinic by her primary care physician for an upper endoscopy and colonoscopy and was diagnosed with H. pylori-related gastritis and a synchronous gastric LCH and primary colonic adenocarcinoma. We describe the histologic characteristics and clinical implications of the LCH diagnosis. A review of the published literature revealed that LCH presenting as a gastric solitary lesion is rare.
This case highlights the importance of recognizing this rare condition to ensure proper patient follow-up.
背景/目的:朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,其特征为病理性朗格汉斯细胞的异常单克隆增殖。LCH的临床表现极不可预测,范围从单系统局限性疾病到具有高死亡率的严重多器官疾病。LCH通常影响儿童,极少影响成人。LCH最常累及的身体部位是骨骼、皮肤、肺、垂体和淋巴结。LCH累及胃肠道极为罕见,仅有少数病例报道。
我们报告一例50岁女性,其初级保健医生将其转至我们诊所进行上消化道内镜检查和结肠镜检查,诊断为幽门螺杆菌相关性胃炎、同步性胃LCH和原发性结肠腺癌。我们描述了LCH诊断的组织学特征和临床意义。对已发表文献的回顾显示,以胃孤立性病变形式出现的LCH很罕见。
本病例强调了认识这种罕见疾病以确保对患者进行适当随访的重要性。