periampullary duodenal neuroendocrine tumor in a patient with neurofibromatosis-1: case report and bibliometric analysis

Abstract

Abstract Background: Patients with Neurofibromatosis type 1 (NF1) have an increased risk of developing neuroendocrine tumors, and the most frequent target organs of periampullary neuroendocrine neoplasms (NENs) in NF1 patients are the duodenum and peri-ampullary region. Case presentation: A 50-year-old male patient was admitted to our hospital after experiencing progressive skin and scleral yellowing for over six months. An abdominal contrast-enhanced computed tomography (CT) scan showed a tumor in the periampullary region, which measures 1.2×1.4cm in size and showed a progressive enhancement. Magnetic resonance cholangiopancreatography (MRCP) showed the dilation of intra- and extrahepatic bile ducts. The patient was diagnosed with an ampullary tumor with the possibility of malignancy. A Whipple procedure was performed. Microscopically, the duodenum tumor invades the mucosa, sphincter, and muscular layer of the duodenal papilla. Histologic hematoxylin and eosin (HE)stain confirmed the presence of duodenal G1 neuroendocrine tumor (NET). In order to get the whole picture of NENs, a bibliometric analysis was performed, which showed that publications about periampullary NENs increase gradually every year, and most of them focus on the treatment and diagnosis of NENs. Conclusions: We reported a case of periampullary duodenal NET in a patient with NF1, and carried out a bibliometric analysis on periampullary NENs.