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We report herein the extremely unusual case of a 71-year-old woman with signet-ring cell carcinoma of the ileum. She originally presented with a 6-month history of intermittent nausea and abdominal distention, but initial examinations, including gastrointestinal fiberscopy, ultrasonography, and computed tomography (CT) scan, failed to reveal any cause of her symptoms. A barium-enema study performed 11 months after her initial visit demonstrated a narrow portion of the terminal ileum. An ileocecal resection was subsequently performed, and an epigastric subcutaneous tumor was simultaneously excised. The specimen contained a tumor with a stenotic lumen resembling a "lead pipe", an ulcerative portion, and mucosa with a granular appearance adjoining its proximal site. Many small aphthous lesions with IIa + IIc appearance were seen in the apparently normal mucosa. Histopathological examination confirmed a diagnosis of signet-ring cell carcinoma. The small aphthous lesions seemed to be metastases spread via the lymphatic vessels. Our review of the medical literature revealed three cases of signet-ring cell carcinoma of the jejunum; however, this is the first reported case of signet-ring cell carcinoma of the ileum.

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