Abstract

INTRODUCTION: Neuroendocrine tumors (NETs), historically known as carcinoid tumors, can arise throughout the gastrointestinal tract. However, duodenal NETs are very rare; the incidence is only 0.19 / 100,000 in the US. We present a patient who identifies as a Jehovah’s witness presenting with acute on chronic anemia and occult gastrointestinal bleeding due to a bleeding mass which was biopsied and confirmed as a duodenal NET. CASE DESCRIPTION/METHODS: A 69 year-old African American female, Jehovah’s witness, with medical history of end stage renal disease on hemodialysis and anemia of chronic disease who was brought to the hospital for asymptomatic acute on chronic anemia with a hemoglobin of 5.6 g/dL (baseline 9–10 g/dL). Rectal exam revealed melena. Patient underwent upper endoscopy which revealed a 2 cm duodenal bulb mass which was biopsied and pathology revealed neuroendocrine tumor. Patient had elevated serum gastrin level of 250 pg/mL and chromogranin A level of 1963 ng/mL. Octreotide scan confirmed radiotracer uptake in the duodenum and in no other areas. The patient refused blood products adamantly and was managed with darbepoetin alfa. Her hemoglobin gradually trended up several months later to 10 g/dL. For her NET, she was referred to a tertiary center for EUS, for staging and surgical evaluation. DISCUSSION: Duodenal NETs can be divided upon proliferative activity, (well differentiated and poorly differentiated) as well as upon hormonal capabilities (gastrinoma, somatostatinoma, nonfunctional, and paragangliomas). Most patients are incidentally diagnosed during endoscopy or present with late complications of an indolent tumor. Carcinoid syndrome only occurs in less than 10 % cases, when there is metastasis to the liver. Diagnosis is based upon histological staining for neuroendocrine tissue, such as chromogranin and synaptophysin along with checking for proliferative activity with Ki-67 staining. Our patient’s biopsy confirmed well differentiated neuroendocrine tumor, positive for the above markers and low Ki-67 staining. Serum chromogranin A is elevated in 80% of NETs, as in this case. Gastrin level was below 1000 pg/mL, ruling out a functional tumor. CT and octreotide scan excluded any metastasis. NETs which are nonfunctional, without metastasis, and are less than 1 cm can be endoscopically resected. Our patient’s mass was 2 cm, and therefore she was sent for surgical evaluation. Her hemoglobin improved gradually with erythropoietin therapy and her outcome did not change despite her beliefs.

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