Work

Case Report: Systematic endoscopic characterization of synchronous esophageal, gastric, and colorectal involvement in multisystem Langerhans cell histiocytosis

Year: 2025

Type: article

Abstract: Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder derived from dendritic cells, predominantly affecting pediatric populations. Adult-onset LCH involving the gastrointestinal (GI) tract is exceedingly rare, with limited endoscopic descriptions. We describe a 34-year-old male with gingival swelling and mandibular osteolysis, ultimately diagnosed with multisystem LCH. Imaging revealed pulmonary nodules and diffuse gastrointestinal involvement, confirmed through high-resolution endoscopy. Characteristic findings included barnacle-like esophageal plaques, ulcerated gastric nodules, and clustered subepithelial masses in the colon. A novel 'mucosal fragmentation sign' was identified during resection-abrupt mucosal detachment revealing white, fish-flesh-like tumor tissue. Histology confirmed LCH with CD1a, S100, and langerin (CD207) positivity, alongside detection of a BRAF V600E mutation. This case is the first to systematically characterize concurrent esophageal, gastric, and colonic LCH involvement in an adult. Distinctive endoscopic patterns may support early diagnosis and optimized biopsy strategies in atypical clinical scenarios.

Source: Frontiers in Medicine

DOI: 10.3389/fmed.2025.1674312

Authors: Xueman Wang, Quan Luo, Tiannu Ding, Bo Lian, Xintong Jiang, Jian Dong

Institutions: Shaoxing University, Shaoxing People's Hospital

Language: English

Topic: Histiocytic Disorders and Treatments

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,801