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Front Oncol 2021 31;11:646992. Epub 2021 Mar 31.

Predictive Oncology Laboratory, Department of Medical Oncology, CRCM, Institut Paoli-Calmettes, Aix-Marseille Université, Marseille, France.

Temporal and spatial tumor heterogeneity can be observed in pancreatic neuroendocrine tumor. We report the case of a young woman with long term stabilization of a G2 metastatic pancreatic NET that, after pregnancy, suddenly progressed into one single liver metastasis corresponding to a transformation into G3 large-cell neuroendocrine cancer. The patient underwent liver resection (the progressive and one dormant metastasis). Read More

J Cancer Res Clin Oncol 2021 Apr 27. Epub 2021 Apr 27.

Department of Hepatobiliary and Pancreatic Surgery, Graduate School of Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

Purpose: In patients with metastatic functional gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs), it is unknown what degree of tumor reduction is required to eliminate hormonal symptoms. We aimed to reduce hormonal symptoms derived from advanced GEP-NENs by efficient minimal intervention, constructing a hormonal tumor map of liver metastases.

Methods: Between 2013 and 2019, we treated 12 insulinoma or gastrinoma patients with liver metastases. Read More

Mol Cancer Res 2021 Mar 26. Epub 2021 Mar 26.

Department of Pathology, University Medical Center Utrecht, the Netherlands.

Patients with multiple endocrine neoplasia 1 (MEN1) syndrome have a germline mutation in the gene. Loss of the wild-type allele can initiate endocrine tumorigenesis. Microscopic and macroscopic pituitary, parathyroid, and pancreatic tumors (referred to as the 3 P's) show loss of the wild-type MEN1 allele up to 100%. Read More

Endokrynol Pol 2021 Mar 22. Epub 2021 Mar 22.

Department of Gastroenterology,Pomeranian Medical University, Unii Lubelskiej 1, 71-252 Szczecin, Poland.

Not required for Clinical Vignette. Read More

World J Nucl Med 2020 Oct-Dec;19(4):417-420. Epub 2020 Aug 22.

Department of Biophysics and Medical Physics, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania.

Gastrin-secreting tumors, hypergastrinemia and severe ulcer disease form the trademarks of Zollinger-Ellison syndrome (ZES). We report a case of gastrinoma, in a patient who was misdiagnosed for almost five years. The case emphsizes the the special role of functional imaging in the personalized approach to the patient with suggestive symptomatology for NETs. Read More

Mayo Clin Proc 2021 02;96(2):285-286

Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN.

World J Gastroenterol 2021 Jan;27(1):129-142

Department of Gastroenterology, St. Luke's International Hospital, Tokyo 104-8560, Japan.

Background: Gastric gastrinoma and spontaneous tumor regression are both very rarely encountered. We report the first case of spontaneous regression of gastric gastrinoma.

Case Summary: A 37-year-old man with a 9-year history of chronic abdominal pain was referred for evaluation of an 8 cm mass in the lesser omentum discovered incidentally on abdominal computed tomography. Read More

Am J Case Rep 2021 Jan 16;22:e927761. Epub 2021 Jan 16.

Department of Surgery, University of British Columbia, Vancouver, BC, Canada.

BACKGROUND Neurofibromatosis type 1 (NF1) is a multi-tumor syndrome in which affected patients develop malignancies that are rare in the overall population, such as tumors of neural or endocrine origin. CASE REPORT A 67-year-old woman with a clinical diagnosis of NF1 presented with abdominal pain and pneumoperitoneum. She underwent small-bowel resections for a perforated jejunal lesion and a second lesion in the ileum; pathology showed a neurofibroma at the site of the perforation and a 1-cm low-grade GIST, respectively. Read More

Pancreatology 2021 Jan 7;21(1):236-239. Epub 2020 Dec 7.

Department of Gastroenterology and Digestive Oncology, Reims University Hospital, Reims, France.

We report here the first case of life-threatening hypomagnesemia in a Zollinger-Ellison syndrome patient with multiple endocrine neoplasia type 1 (MEN1) syndrome. The severe symptomatic hypomagnesemia proved to be due to proton pump inhibitors (PPIs), but withdrawal of PPIs led to early severe peptic complications despite a substitution by histamine H2-receptor antagonist therapy. Simultaneous management of life-threatening hypomagnesemia, severe gastric acid hypersecretion and MEN1-associated gastrinomas was complex. Read More

Histol Histopathol 2020 Dec 11:18288. Epub 2020 Dec 11.

School of Medicine, Griffith University, Gold Coast, QLD, Australia.

The nomenclature and classification of pancreatic neuroendocrine neoplasms has evolved in the last 15 years based on the advances in knowledge of the genomics, clinical behaviour and response to therapies. The current 2019 World Health Organization classification of pancreatic neuroendocrine neoplasms categorises them into three groups; pancreatic neuroendocrine tumours (PanNETs)(grade 1 grade 2, grade 3), pancreatic neuroendocrine carcinomas and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) based on the mitotic rate, Ki-67 index, morphological differentiation and/or co-existing tissue subtype. PanNETs are also classified into non-functional NET, insulinoma, gastrinoma, VIPoma, glucagonoma, somatostatinoma, ACTH-producing NET and serotonin producing NET based on hormone production and clinical manifestations. Read More

Surg Case Rep 2020 Nov 18;6(1):290. Epub 2020 Nov 18.

Department of Surgery, Fukuyama City Hospital, 5-23-1 Zao-cho, Fukuyama, Hiroshima, 721-8511, Japan.

Background: A majority of gastrinomas causing Zollinger-Ellison syndrome are located in the duodenum or pancreas. Primary hepatic gastrinomas are rare and difficult to diagnose. We report a rare case of primary hepatic gastrinoma, which could be diagnosed preoperatively. Read More

Int J Endocrinol 2020 6;2020:1030518. Epub 2020 Nov 6.

Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.

Background: Functional pancreatic neuroendocrine neoplasms (PanNENs) are very rare disorders but have complex spectrum, including insulinoma, gastrinoma, glucagonoma, somatostatinoma, and VIPoma. Patients with PanNENs usually present with characteristic symptoms caused by corresponding hormone hypersecretion. It has always been challenging in dealing with such rare but complicated disorders. Read More

Cell 2020 Nov 6;183(5):1420-1435.e21. Epub 2020 Nov 6.

Department of Pulmonary Medicine, Keio University School of Medicine, Tokyo 160-8582, Japan.

Gastroenteropancreatic (GEP) neuroendocrine neoplasm (NEN) that consists of neuroendocrine tumor and neuroendocrine carcinoma (NEC) is a lethal but under-investigated disease owing to its rarity. To fill the scarcity of clinically relevant models of GEP-NEN, we here established 25 lines of NEN organoids and performed their comprehensive molecular characterization. GEP-NEN organoids recapitulated pathohistological and functional phenotypes of the original tumors. Read More

Am Surg 2020 Oct 30:3134820951493. Epub 2020 Oct 30.

Department of Surgery, Walter Reed National Military Medical Center, Bethesda, MD, USA.

Front Oncol 2020 25;10:555963. Epub 2020 Sep 25.

Department of Oncology, Yokohama City University Hospital, Yokohama, Japan.

Neuroendocrine tumors (NETs) are rare, but their worldwide incidence is gradually increasing. NETs are generally heterogeneous; however, in rare cases, they have been shown to change their phenotype (i.e. Read More

BMC Med Genet 2020 09 29;21(1):190. Epub 2020 Sep 29.

Department of Endocrinology and Diabetology, University Hospital Centre Split, Soltanska 1, Split, Croatia.

Background: Multiple endocrine neoplasia type 1 (MEN1) syndrome is usually accompanied by endocrine tumors, but non-endocrine tumors can occur as well. However, the coexistence of MEN1 syndrome and malignant tumor such as low-grade fibromyxoid sarcoma has not been described in the literature. Moreover, the MEN1 gene mutations have not been identified in patients with fibromyxoid sarcoma, so far. Read More

Int J Surg Case Rep 2020 19;77S:S105-S108. Epub 2020 Sep 19.

Department of Surgical, Oncological and Oral Sciences, Section of General and Urgent Surgery, University of Palermo, Italy. Electronic address:

Introduction: Neuroendocrine tumors (NETs) represent uncommon neoplasms with different characteristics. They can be asymptomatic and benign or they can also proliferate and manifest themselves with neoplastic mass symptoms such as intestinal occlusion or with carcinoid syndrome. Gastric neuroendocrine neoplasms (g-NENs) are the most frequent digestive NENs while duodenal neuroendocrine neoplasms (d-NENs) may be sporadic or associated with multiple endocrine neoplasia type 1 (MEN-1) and present a functional syndrome (e. Read More

Abdom Radiol (NY) 2021 03 22;46(3):919-935. Epub 2020 Sep 22.

Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.

Neuroendocrine neoplasms (NENs) are a group of neoplasms arising from the diffuse endocrine system (DES). The gastrointestinal tract (GIT) is the most common site of NEN. The WHO classification divides NEN into three broad categories viz. Read More

J Endocrinol Invest 2020 Sep 9. Epub 2020 Sep 9.

General Surgery Service, Endocrine Surgery Unit, Instituto Murciano de Investigación Biosanitaria (IMIB), Virgen de La Arrixaca University Hospital, Crta./Madrid-Cartagena, S/N, 30120, El Palmar, Murcia, Spain.

Introduction: The prognosis of MEN 1 patients is not only determined by pancreatic disease; it is also related to other uncommon tumors. The objective of this study is to analyze the tumors associated with MEN 1 outside the classic triad and to investigate their relationship with mortality.

Materials And Methods: One hundred and five MEN 1 patients were studied in a tertiary referral hospital (1980-2019). Read More

Nihon Shokakibyo Gakkai Zasshi 2020 ;117(8):706-712

Department of Pathology, Osaka City General Hospital.

A 42-year-old man, after remission of MALT lymphoma of the small intestine, was repeatedly hospitalized because of abdominal pain and severe dehydration caused by frequent vomiting and watery diarrhea. His symptoms would improve quickly every time when he was fasted and inserted a nasogastric tube. We were unable to find abnormalities on endoscopic examination and computed tomography. Read More

AJR Am J Roentgenol 2020 10 13;215(4):885-895. Epub 2020 Jul 13.

Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030.

Multiple endocrine neoplasia (MEN) syndromes are autosomal-dominant genetic disorders that predispose two or more organs of the endocrine system to tumor development. Although the diagnosis relies on clinical and serologic findings, imaging provides critical information for surgical management with the ultimate goal of complete tumor resection. This article reviews abdominal neoplasms associated with the various subtypes of MEN syndromes, with a focus on clinical presentation and characteristic imaging features. Read More

J Gastrointest Cancer 2021 Mar;52(1):351-354

Department of Tumor Pathology, Hamamatsu University School of Medicine, Hamamatsu, Japan.

Histopathology 2020 Dec 20;77(6):865-876. Epub 2020 Sep 20.

Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Aims: Proton pump inhibitors (PPIs) are among the most widely used medications in the United States. Most PPI users have persistent hypergastrinaemia during treatment. However, gastric neuroendocrine tumours diagnosed in long-term PPI users are rarely reported. Read More

Eur Heart J 2020 09;41(35):3376

Department of Cardiology, Hospital del Mar, Passeig Marítim de la Barceloneta, 25-29, 08003 Barcelona, Spain.

Cureus 2020 Jun 6;12(6):e8471. Epub 2020 Jun 6.

Department of Gastroenterology and Hepatology, Carol Davila University of Medicine and Pharmacy, Floreasca Emergency Hospital, Bucharest, ROU.

We present a case of a 60-year-old male diagnosed with Zollinger-Ellison syndrome (ZES) after a protracted multicentric workup for chronic diarrhea and unexplained weight loss. ZES is intrinsically difficult to diagnose due to nonspecific symptoms, which are mimicked by other more frequent pathologies, such as peptic ulcer disease secondary to Helicobacter pylori or nonsteroidal anti-inflammatory drugs. The diagnostic challenge can be further complicated by patient noncompliance, resulting in delayed management and unnecessary health care. Read More

Clin Nucl Med 2020 Oct;45(10):813-816

Division of Endocrinology, UCLA David Geffen School of Medicine, Los Angeles, CA.

Incidental tracer uptake on DOTATATE PET is increasingly encountered in clinical practice. We describe 3 patients with suspected or known neuroendocrine tumor who were found to have diffuse and intense stomach uptake on DOTATATE PET. All patients underwent esophagogastroduodenoscopy and/or endoscopic ultrasound; the cause of the stomach uptake was attributed to proton-pump inhibitor use, chronic gastritis, and gastrinoma, respectively. Read More

Med Sci Monit 2020 Jun 21;26:e922613. Epub 2020 Jun 21.

Department of Gastroenterology, Yijishan Hospital of Wannan Medical College, Wuhu, Anhui, China (mainland).

BACKGROUND This study was designed to predict prognosis of patients with primary duodenal neuroendocrine neoplasms (D-NENs) by developing nomograms. MATERIAL AND METHODS Patients diagnosed with D-NENs between 1988 and 2015 were queried from the SEER database and a total of 965 appropriate cases were randomly separated into the training and validation sets. Kaplan-Meier analysis was used to generated survival curves, and the difference among the groups was assessed by the log-rank test. Read More

GE Port J Gastroenterol 2020 Apr 9;27(3):192-196. Epub 2019 Oct 9.

Internal Medicine Department, Centro Hospitalar São João, Porto, Portugal.

Gastrinomas are neuroendocrine tumors characterized by gastrin overexpression - 80% are sporadic and 20% are associated with multiple endocrine neoplasia type 1. A 75-year-old male patient, surgically treated at the age of 50 years for gastrinoma, followed on an outpatient basis because of chronic non-bloody diarrhea, was admitted to our hospital because of abdominal pain, watery diarrhea, and nonbiliary vomits. He was hypotensive and showed no response to fluids. Read More

Nutrients 2020 May 15;12(5). Epub 2020 May 15.

Department of Human Nutrition and Metabolomic, Pomeranian Medical University in Szczecin, Broniewskiego 24 str, 71-460 Szczecin, Poland.

Nuroendocrine neoplasms (NENs) are a group of rare neoplasms originating from dispersed neuroendocrine cells, mainly of the digestive and respiratory tract, showing characteristic histology and immunoprofile contributing to classification of NENs. Some NENs have the ability to produce biogenic amines and peptide hormones, which may be associated with clinical syndromes like, e.g. Read More

World J Surg Oncol 2020 Apr 28;18(1):80. Epub 2020 Apr 28.

National Institute of Gastroenterology "S. de Bellis", Research Hospital Castellana Grotte Bari, Via Turi 27, 70013 Castellana Grotte (Ba), Bari, Italy.

Background: Gastrinoma is a rare form of neuroendocrine neoplasm. The presence of a primary lymph node localization of gastrinoma is a much debated and controversial topic in the literature, as regards whether these cases represent metastatic disease from an as yet unidentified primary tumor, or the de novo occurrence of a gastrinoma in a lymph node.

Case Presentation: We report the case of a 24-year-old male with intense epigastric pain treated at the beginning with high dose proton pump inhibitors. Read More