Granular cell tumors (GCTs) are rare and benign tumors that can occur at any anatomical site. GCTs are thought to originate from nerve cells, particularly Schwann cells. Their name derives from the fact that an accumulation of cytoplasmic lysosomes imparts the tumor with a granular appearance. They are most commonly observed in the oral cavity, skin and subcutaneous tissue, breast, and respiratory tract. GCTs rarely affect the gastrointestinal tract. We report a 56-year-old female with a medical history of human immunodeficiency virus, hepatitis C, and cholelithiasis, who presented with abdominal pain. Upper endoscopy revealed a 1 - 2 cm solitary yellowish appearing nodule just distal to the GE junction. Biopsy of the nodule followed by histopathology was positive for S100, but negative for pancytokeratin immunostains. PAS staining highlighted cytoplasmic granules, further supporting the diagnosis of gastrointestinal GCT.
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