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Abstract
BACKGROUND
Duodenal neuroendocrine tumors (D-NETs) are rare neoplasms accounting for approximately 2–5% of gastroenteropancreatic neuroendocrine tumors. They are commonly asymptomatic and incidentally detected; however, ulcerated lesions may rarely present with vert gastrointestinal bleeding and severe iron deficiency anemia.
CASE PRESENTATION
Despite taking oral and parenteral iron supplements, a 62-year-old woman developed recurring melena and severe iron deficiency anemia that required transfusions. Microcytic hypochromic anemia with hemoglobin of 7.5 g/dL, decreased mean corpuscular volume, and peripheral smear results indicative of chronic blood loss were found in the laboratory assessment. Erosive pangastritis, a Helicobacter pylori-associated gastric ulcer, and over eight sessile and sub-pedunculated polypoidal lesions extending from the first to third parts of the duodenum were discovered during upper gastrointestinal endoscopy. Several of these lesions showed deep ulceration with active bleeding. Four bigger lesions underwent endoscopic band ligation. Several modestly hyper-enhancing duodenal polyps were seen on contrast-enhanced CT enterography; the biggest one was around 15 × 15 mm. Brunner gland hyperplasia with questionable neuroendocrine foci was revealed by histopathological analysis, however, a well-differentiated Grade I neuroendocrine tumor with widespread synaptophysin positivity and a Ki-67 proliferation index of 2% was verified by immunohistochemistry. With SUVmax values of 5.0 on early imaging and 10.0 on delayed imaging, Ga-68 DOTATOC PET-CT showed somatostatin receptor-positive multifocal duodenal lesions up to 1 cm in size. There was no indication of solid organ involvement, distant metastases, or locoregional lymphadenopathy.
CONCLUSION
This case illustrates a rare manifestation of multifocal Grade I duodenal neuroendocrine tumors linked to overt upper gastrointestinal hemorrhage, severe iron deficiency anemia, and Brunner gland hyperplasia. Accurate staging and prompt care are made possible by early detection employing a multidisciplinary approach that includes endoscopy, histology, immunohistochemistry, and somatostatin receptor imaging. This improves clinical results.
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References
- Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol 2017;3(10):1335-42.
- Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 2008;9(1):61-72.
- Yao JC, Hassan M, Phan A, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26(18):3063-72.
- Pape UF, Niederle B, Costa F, et al. ENETS Consensus Guidelines for neuroendocrine neoplasms of the appendix, colon, rectum, and duodenum. Neuroendocrinology 2016;103(2):144-52.
- Delle Fave G, O'Toole D, Sundin A, et al. ENETS Consensus Guidelines Update for gastroduodenal neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):119-24.
- Klöppel G, Couvelard A, Hruban RH, et al. Neoplasms of the neuroendocrine pancreas. In: WHO Classification of Tumours Editorial Board. Digestive System Tumours. 5th edn. Lyon: International Agency for Research on Cancer 2019.
- Nagtegaal ID, Odze RD, Klimstra D, et al. The 2019 WHO classification of tumours of the digestive system. Histopathology 2020;76(2):182-8.
- Klimstra DS, Kloppel G, La Rosa S, et al. Classification of neuroendocrine neoplasms of the digestive system. WHO Classification of Tumours Editorial Board. Digestive System Tumours. 5th edn. Lyon: IARC; 2019:16-9.
- Rindi G, Klersy C, Albarello L, et al. Competitive testing of the WHO grading and AJCC/UICC staging systems in a large series of neuroendocrine tumors of the gastroenteropancreatic tract. Ann Oncol 2012;23(10):2721-8.
- Pavel M, O'Toole D, Costa F, et al. ENETS Consensus Guidelines Update for the management of distant metastatic disease of intestinal, pancreatic, bronchial neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):172-85.
- Hope TA, Bergsland EK, Bozkurt MF, et al. Appropriate use criteria for somatostatin receptor PET imaging in neuroendocrine tumors. J Nucl Med 2018;59(1):66-74.
- Falconi M, Eriksson B, Kaltsas G, et al. ENETS Consensus Guidelines Update for the management of patients with digestive neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):153-71.
- Basuroy R, Srirajaskanthan R, Ramage JK. A multimodal approach to the management of neuroendocrine tumours. BMJ 2018;363:k3229.
- Scherübl H, Jensen RT, Cadiot G, et al. Neuroendocrine tumors of the small bowels are on the rise: early aspects and management. World J Gastrointest Endosc 2010;2(10):325-34.
- Ahmed M. Gastrointestinal neuroendocrine tumors in 2020. World J Gastrointest Oncol 2020;12(8):791-807.
References
Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol 2017;3(10):1335-42.
Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 2008;9(1):61-72.
Yao JC, Hassan M, Phan A, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26(18):3063-72.
Pape UF, Niederle B, Costa F, et al. ENETS Consensus Guidelines for neuroendocrine neoplasms of the appendix, colon, rectum, and duodenum. Neuroendocrinology 2016;103(2):144-52.
Delle Fave G, O'Toole D, Sundin A, et al. ENETS Consensus Guidelines Update for gastroduodenal neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):119-24.
Klöppel G, Couvelard A, Hruban RH, et al. Neoplasms of the neuroendocrine pancreas. In: WHO Classification of Tumours Editorial Board. Digestive System Tumours. 5th edn. Lyon: International Agency for Research on Cancer 2019.
Nagtegaal ID, Odze RD, Klimstra D, et al. The 2019 WHO classification of tumours of the digestive system. Histopathology 2020;76(2):182-8.
Klimstra DS, Kloppel G, La Rosa S, et al. Classification of neuroendocrine neoplasms of the digestive system. WHO Classification of Tumours Editorial Board. Digestive System Tumours. 5th edn. Lyon: IARC; 2019:16-9.
Rindi G, Klersy C, Albarello L, et al. Competitive testing of the WHO grading and AJCC/UICC staging systems in a large series of neuroendocrine tumors of the gastroenteropancreatic tract. Ann Oncol 2012;23(10):2721-8.
Pavel M, O'Toole D, Costa F, et al. ENETS Consensus Guidelines Update for the management of distant metastatic disease of intestinal, pancreatic, bronchial neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):172-85.
Hope TA, Bergsland EK, Bozkurt MF, et al. Appropriate use criteria for somatostatin receptor PET imaging in neuroendocrine tumors. J Nucl Med 2018;59(1):66-74.
Falconi M, Eriksson B, Kaltsas G, et al. ENETS Consensus Guidelines Update for the management of patients with digestive neuroendocrine neoplasms. Neuroendocrinology 2016;103(2):153-71.
Basuroy R, Srirajaskanthan R, Ramage JK. A multimodal approach to the management of neuroendocrine tumours. BMJ 2018;363:k3229.
Scherübl H, Jensen RT, Cadiot G, et al. Neuroendocrine tumors of the small bowels are on the rise: early aspects and management. World J Gastrointest Endosc 2010;2(10):325-34.
Ahmed M. Gastrointestinal neuroendocrine tumors in 2020. World J Gastrointest Oncol 2020;12(8):791-807.