Duodenal neuroendocrine tumors (d-NETs): challenges in diagnosis and treatment

Strinović, Mateja; Kruljac, Ivan; Dabelić, Nina; Nikolić, Marko; Ljubičić, Neven; Čugura, Jakša Filipović; Vazdar, Ljubica; Brozić, Jasmina Marić; Kust, Davor; Ovčariček, Petra Petranović; Nenadić, Vedrana Gladić; Marjan, Domagoj; Ulamec, Monika; Demirović, Alma; Miculinić, Andrija; Vrkljan, Milan

doi:10.21040/eom/2016.2.3.3

Cited by 4 publications

(15 citation statements)

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“…They most commonly occur in the 6th decade of life with male preponderance. Most of the GEP-NETs are nonfunctional and present clinically with symptoms like abdominal pain, upper gastrointestinal bleeding, anaemia or jaundice [6]. Our patient was a 52-year-old male, presenting with abdominal pain.…”

Section: Discussionmentioning

confidence: 91%

“…Duodenal NET is extremely rare and accounts for only 2.6% of all NETs [3,5]. Duodenal NETs (d-NETs) can be classified into five different tumour types: non-functional d-NETs, duodenal gangliocytic paraganglioma, high-grade poorly [6]. Broadly, it is important to note that 90% of d-NETs are not associated with a functional clinical syndrome and more than 90% occur in the first or second part of the duodenum [5].…”

Section: Discussionmentioning

confidence: 99%

“…Duodenal NETs account for only 2.6% of all the neuroendocrine neoplasms [3,5]. They most commonly occur in the 6th decade of life with male preponderance [6]. Most of the GEP-NET are non-functional and are discovered incidentally on endoscopy, done for symptoms like abdominal pain, upper gastrointestinal bleeding, anaemia or jaundice [5,7].…”

Section: Introductionmentioning

confidence: 99%

“…Most of the GEP-NET are non-functional and are discovered incidentally on endoscopy, done for symptoms like abdominal pain, upper gastrointestinal bleeding, anaemia or jaundice [5,7]. Various investigations that can be performed for the diagnosis of NET are upper gastrointestinal endoscopy which is the gold standard [6]. Ultrasound abdomen, endoscopic ultrasonography (EUS) and EUS-fine-needle aspiration and biopsy (EUS-FNAB) can also be done along with other radiological modalities such as computerised tomography (CT) and magnetic resonance imaging (MRI) [8,9].…”

Section: Introductionmentioning

confidence: 99%

“…Surgical intervention includes Whipple's procedure or pancreaticoduodenectomy with Roux-enY anastomosis. A 5-year survival rate for patients with well differentiated NET is 85%, but patients with poorly differentiated NEC have unfavourable prognosis [6]. Histologically, NETs are small blue round cell tumours showing uniform cells having round to oval nucleus with stippled chromatin and eosinophilic granular cytoplasm.…”

Section: Introductionmentioning

confidence: 99%

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Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

Dewan

Bhat

Prasad

et al. 2018

Indian J Surg Oncol

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Neuroendocrine carcinoma rarely occurs in the duodenum, and most cases of neuroendocrine carcinoma in the duodenum show rapid progression of the disease. Such cases have poor prognosis even with radical surgery with or without chemotherapy with low 5-year survival rate. We present a case of a 52-year-old man who presented with abdominal pain of 1-month duration and one episode of vomiting. Upper gastrointestinal endoscopy revealed polypoidal lesions in the first and second part of the duodenum. Whipple's procedure was performed. Diagnosis of poorly differentiated neuroendocrine carcinoma was made with extension to pancreas with peripancreatic lymph node metastases. The patient expired on post operative day 17 following cardiac arrest.

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

Dewan

Bhat

Prasad

et al. 2018

Indian J Surg Oncol

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Sporadic, non‐functional, gastrin‐producing duodenal neuroendocrine tumors: A retrospective study of an infrequent disease

Huerta

Fernández

Sánchez-Moreno

et al. 2022

J of Digest Diseases

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Objectives Non‐functioning gastrin‐producing neuroendocrine neoplasms (NEN) of the duodenum are rare gastrointestinal tumors without a clinical syndrome due to gastrin production. Their incidence has significantly increased as an incidental finding during endoscopic studies. The aim of this study was to describe the characteristics and prognostic factors of this emergent and infrequent neoplasm. Methods We performed a retrospective observational study based on the duodenal NENs samples with positive staining for gastrin at the Department of Pathology, University Hospital 12‐de‐Octubre (Madrid, Spain) between 2000 and 2017. Patients with clinically functional tumors ([Zollinger–Ellison syndrome] or gastrin >1000 pg/mL), with previously diagnosed multiple endocrine neoplasia (MEN) syndrome or synchronous neoplasia were excluded. Clinicopathological and therapeutic variables, follow‐up, recurrence, and mortality data were collected. Results In all, 21 patients were included. Most of the tumors were diagnosed incidentally as a single small polypoid lesion limited to mucosa/submucosa and with a low histological grade. Four (19.0%) patients presented with metastatic involvement at diagnosis (lymphatic and/or hepatic). These four patients also had a high or intermediate mitotic grade and infiltration further than submucosa. Local resection was applied in most cases as curative treatment. There were two cases of tumor recurrence and two tumor‐related deaths with a 5‐year disease‐free survival of 81.0%. Conclusions The majority of these tumors were diagnosed at a localized stage and had a good prognosis with treatment. Nevertheless, given the potential metastatic risk, a close follow‐up is necessary, especially in those with aggressive pathological factors such as deep infiltration or high histological grade.

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Gastrointestinal neuroendocrine tumors in 2020

Ahmed

2020

WJGO

160

135

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Gastrointestinal neuroendocrine tumors are rare slow-growing tumors with distinct histological, biological, and clinical characteristics that have increased in incidence and prevalence within the last few decades. They contain chromogranin A, synaptophysin and neuron-specific enolase which are necessary for making a diagnosis of neuroendocrine tumor. Ki-67 index and mitotic index correlate with cellular proliferation. Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors. Most of the gastrointestinal neuroendocrine tumors are non-functional. World Health Organization updated the classification of neuroendocrine tumors in 2017 and renamed mixed adenoneuroendocrine carcinoma into mixed neuroendocrine neoplasm. Gastric neuroendocrine tumors arise from enterochromaffin like cells. They are classified into 4 types. Only type I and type II are gastrin dependent. Small intestinal neuroendocrine tumor is the most common small bowel malignancy. More than two-third of them occur in the terminal ileum within 60 cm of ileocecal valve. Patients with small intestinal neuroendrocrine tumors frequently show clinical symptoms and develop distant metastases more often than those with neuroendocrine tumors of other organs. Duodenal and jejuno-ileal neuroendocrine tumors are distinct biologically and clinically. Carcinoid syndrome generally occurs when jejuno-ileal neuroendocrine tumors metastasize to the liver. Appendiceal neuroendocrine tumors are generally detected after appendectomy. Colonic neuroendocrine tumors generally present as a large tumor with local or distant metastasis at the time of diagnosis. Rectal neuroendocrine tumors are increasingly being diagnosed since the implementation of screening colonoscopy in 2000. Gastrointestinal neuroendocrine tumors are diagnosed and staged by endoscopy with biopsy, endoscopic ultrasound, serology of biomarkers, imaging studies and functional somatostatin scans. Various treatment options are available for curative and palliative treatment of gastrointestinal neuroendocrine tumors.

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Duodenal neuroendocrine tumors (d-NETs): challenges in diagnosis and treatment

Cited by 4 publications

References 0 publications

Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

Sporadic, non‐functional, gastrin‐producing duodenal neuroendocrine tumors: A retrospective study of an infrequent disease

Gastrointestinal neuroendocrine tumors in 2020

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