title: Langerhans cell histiocytosis of the stomach with atypical morphological features.
abstract: We report the unique case of a 52-year-old patient with atypical morphological features of gastric Langerhans cell histiocytosis. The man was admitted because of increasing upper abdominal pain and weight loss. The upper gastrointestinal endoscopy showed a submucous, hemispherical tumor of the stomach wall along the lesser curvature. The tumor was completely removed and the patient was discharged 11 days later. Two months later, he died at home. The cause of death is unknown, because the autopsy was denied by the relatives. Macroscopically, the stomach showed a 4.5 x 2.5 cm large, spherical tumor of the lesser curvature of the corpus, which infiltrated the perigastric omenta and lymph nodes, the distal pancreas and the glissonian liver capsule. The vast majority of neoplastic cells were intensely positive for S-100 and CD1a. Typical Birbeck granules could be identified in almost all cells. Cell nuclei analyzed by flow cytometry showed an aneuploid peak, a feature typically associated with malignant disease.
authors.affiliation: Institute of Pathology, University Hospital of Basel, Switzerland. lterracciano@uhbs.ch
authors.forename: L
authors.initials: L
authors.lastname: Terracciano
authors.forename: T
authors.initials: T
authors.lastname: Kocher
authors.forename: G
authors.initials: G
authors.lastname: Cathomas
authors.forename: L
authors.initials: L
authors.lastname: Bubendorf
authors.forename: F S
authors.initials: FS
authors.lastname: Lehmann
mesh_terms: D003594:Cytoplasmic Granules / Q000648:ultrastructure; D017809:Fatal Outcome; D005434:Flow Cytometry; D005773:Gastroscopy; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007801:Langerhans Cells / Q000648:ultrastructure; D008297:Male; D008875:Middle Aged; D013270:Stomach / Q000473:pathology*; D013272:Stomach Diseases / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed