Upper Gastrointestinal Langerhans Cell Histiocytosis: A Report of 2 Adult Cases and a Literature Review

• Published in: International journal of surgical pathology 2021-08, Vol.29 (5), p.550-556
• Main Authors: Matsuoka, Yui, Iemura, Yoshiki, Fujimoto, Masakazu, Shibuya, Shinsuke, Yamada, Atsushi, Fujii, Shigehiko, Kusaka, Toshihiro, Shindo, Takero, Minamiguchi, Sachiko, Haga, Hironori
• Format: Article
• Language: English
• Subjects: Autoimmune diseases; Endoscopy
• ISSN: 1066-8969; 1940-2465
• DOI: 10.1177/1066896920964566

Langerhans cell histiocytosis (LCH) with primary involvement of the upper gastrointestinal (GI) tract is rare. We report 2 adult cases of localized LCH in the upper-GI tract, including the second reported adult case of esophageal LCH and review 11 previously reported cases. Case 1 involved the esophagus of a 61-year-old man; histiocytosis was detected when endoscopy was performed for an examination of epigastric pain. Case 2 involved the stomach of a 56-year-old woman wherein the lesion was detected during a follow-up endoscopy after Helicobacter pylori infection. Both biopsy specimens exhibited diffuse proliferation of mononuclear cells with nuclear convolution and a background of eosinophilic infiltrate. The cells were immunohistochemically positive for CD1a and langerin, and BRAF V600E mutation was detected in Case 2. Follow-up endoscopy for both cases revealed that the lesions disappeared without any treatment. It is important to avoid misdiagnosing LCH of the upper-GI tract as a malignant neoplasm.