Neuroendocrine Tumor of the Jejunum in a Patient with Celiac Disease

Abstract

Purpose: Malignancy is a major complication of celiac disease. Small intestine lymphoma and carcinomas of the oropharynx, esophagus, and small intestine are the most common malignancies associated with celiac disease. We report a neuroendocrine tumor of the jejunum. Case Report: An 84 Y/O man who had undergone right hemicolectomy for colon cancer 16 years prior was referred for a second opinion for iron deficiency anemia (IDA). The anemia required weekly blood transfusions. One year prior, he had undergone an evaluation elsewhere. Significant findings included: elevated anti-gliadin IgG antibody; unremarkable colonoscopy; normal appearing duodenal mucosa on EGD with Marsh III findings on duodenal biopsies; and focal villous blunting of the proximal small bowel on WCE. The patient was not on a gluten free diet (GFD) at the time of the second opinion. Because of persistent IDA and weight loss, a repeat evaluation was done. Significant findings included: normal serum IgA level and normal tissue trans-glutaminase antibody; positive HLA DQ2 and negative HLA DQ8; unremarkable colonoscopy; and normal appearing duodenal mucosa and normal duodenal biopsies on EGD. The patient was started on a GFD for celiac disease, based on prior evaluations. At a 3 month follow-up visit, he noted an additional 30 lb weight loss, despite adherence to a strict GFD. A CT of the abdomen revealed abnormal small bowel wall thickening in the left pelvis. A small bowel follow through x-ray confirmed abnormal small bowel loops and abnormal small bowel mucosa. Antegrade double balloon enteroscopy was done and revealed a 4 cm fungating, necrotic jejunal mass. Biopsies revealed poorly differentiated neuroendocrine carcinoma. The patient underwent surgical resection of the mass. Final pathology revealed high grade malignant neuroendocrine carcinoma (WHO grade IV) with lymph node metastasis. The patient tolerated surgery well, had return of bowel function, and was discharged. Chemotherapy was not offered due to poor functional status. Discussion: Celiac disease is associated with an increased risk of malignancy. Neuroendocrine tumors in patients with celiac disease are reported infrequently. This case reports the first neuroendocrine tumor of the jejunum in a patient with celiac disease.