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Adv Ther 2021 May 4. Epub 2021 May 4.

Device Development, Sun Pharmaceutical Industries Ltd, Tandalja, Vadodara, Gujarat, India.

Introduction: Octreotide acetate subcutaneous injection is indicated to treat acromegaly and the symptoms of carcinoid tumors and vasoactive intestinal peptide tumors (VIPomas). This formative human factors study assessed the octreotide acetate pen injector and accompanying instructions for use (IFU) with self-trained participants.

Methods: The study enrolled patients with diagnoses of acromegaly, carcinoid tumors, or VIPomas and healthcare practitioners (HCPs) who treat patients with these diagnoses. Read More

Cureus 2021 Mar 19;13(3):e14006. Epub 2021 Mar 19.

Internal Medicine, Wayne State University Detroit Medical Center, Detroit, USA.

Neuroendocrine tumors (NETs) are epithelial neoplasms with predominant neuroendocrine differentiation and the ability to synthesize and secrete variable hormones and monoamines. They are relatively rare, accounting for 2% of all malignancy cases in the United States. The most common system affected by NETs is the gastrointestinal tract. Read More

Am J Case Rep 2021 Mar 28;22:e929618. Epub 2021 Mar 28.

Department of Internal Medicine, São Paulo State University (Unesp), Medical School, Botucatu, SP, Brazil.

BACKGROUND Hemangiomas are benign vascular neoplasms that originate from fast-growing embryonic mesodermal tissue and have a proliferation of endothelial cells, which manifest themselves in different forms, locations, and dimensions. Owing to its rarity and similarity of symptoms with other chronic bowel diseases, intestinal hemangioma is a differential diagnosis to be considered in patients presenting with symptoms such as abdominal pain and anemia. CASE REPORT A 46-year-old woman with a history of diffuse abdominal pain and abdominal distension for 20 years presented with a worsening of symptoms in the past year. Read More

Front Oncol 2021 24;11:629665. Epub 2021 Feb 24.

Regenerative Medicine and Fibrosis Group, Institute for Liver and Digestive Health, Royal Free Hospital, University College London, London, United Kingdom.

Aim: Analysis of the pathophysiology of mesenteric fibrosis (MF) in small intestinal neuroendocrine tumors (SI-NETs) in an paracrine model and in human SI-NET tissue samples.

Methods: An indirect co-culture model of SI-NET cells KRJ-I and P-STS with stromal cells HEK293 was designed to evaluate the paracrine effects on cell metabolic activity, gene expression by RT2 PCR Profilers to analyse cancer and fibrosis related genes, and RNA sequencing. The integrin signaling pathway, a specific Ingenuity enriched pathway, was further explored in a cohort of human SI-NET tissues by performing protein analysis and immunohistochemistry. Read More

Zhonghua Bing Li Xue Za Zhi 2021 Jan;50(1):21-25

Department of Pathology, Peking University First Hospital, Beijing 100034, China.

To investigate the clinicopathological characteristics of goblet cell adenocarcinoma (GCA) of the appendix. Seven cases of GCA were collected at the First Hospital of Peking University, Beijing, China from 2015 to 2018. Hematoxylin and eosin staining and immunohistochemical studies (EnVision method) were carried out on all cases. Read More

Acta Gastroenterol Belg 2020 Oct-Dec;83(4):643-653

Digestive Oncology, University Hospitals Leuven, Leuven, Belgium.

Background And Study Aims: Neuroendocrine neoplasms (NENs) are relatively rare, with marked clinical and biological heterogeneity. Consequently, many controversial areas remain in diagnosis and optimal treatment stratification for NEN patients. We wanted to describe current clinical practice regarding controversial NEN topics and stimulate critical thinking and mutual learning among a Belgian multidisciplinary expert panel. Read More

Neuroendocrinology 2020 Nov 20. Epub 2020 Nov 20.

Background: Neuroendocrine neoplasia (NEN) are a rare group of tumors with different prognosis and response to therapy. Their heterogeneity is dependent on the site of origin, morphology and Ki67. Temozolomide (TEM) appears to be active in metastatic NENs (mNENs) but there is limited evidence about its efficacy in gastrointestinal NENs. 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

Neuroendocrinology 2020 Sep 25. Epub 2020 Sep 25.

Objective: To provide a real-world, population-based assessment of the incidence and outcomes of neuroendocrine neoplasms (NENs) of unknown primary.

Methods: Surveillance, Epidemiology and End Results (SEER) database was accessed and cases with NENs of unknown primary were reviewed. Rates of NENs diagnosis 1975-2017 according to primary tumor site were also reviewed. Read More

Surg Oncol 2020 Dec 18;35:336-343. Epub 2020 Sep 18.

Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology (NTNU), Trondheim, Norway; Department of Gastroenterology and Hepatology, St Olav's Hospital - Trondheim University Hospital, Trondheim, Norway. Electronic address:

Background And Objectives: Small intestinal neuroendocrine tumors (SI-NETs) are slow growing but have frequently metastasized at the time of diagnosis. Most patients are operated with either curative intent or with intent to prolong overall survival. In the current study we have examined overall and disease-free survival in patients operated for SI-NETs. Read More

World J Gastrointest Oncol 2020 Aug;12(8):791-807

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Thomas Jefferson University, Philadelphia, PA 19107, United States.

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. Read More

Curr Treat Options Oncol 2020 Aug 29;21(11):88. Epub 2020 Aug 29.

Section of Hepatobiliary Tumors, Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL, 33612, USA.

Opinion Statement: Small bowel neuroendocrine tumors (SB NETs) are increasing in frequency and becoming more common in surgical practice. It is often difficult to make the diagnosis of a SB NET at an early stage, as the primary tumor tends to be small and patients are asymptomatic until there is regional or distant metastasis, when they develop abdominal pain, partial obstruction, or bleeding and/or develop carcinoid syndrome. Despite this advanced presentation at the time of diagnosis, patients with metastatic SB NETs, as compared to other gastrointestinal malignancies, have favorable survival, which can be improved by appropriate surgical interventions. Read More

Interact Cardiovasc Thorac Surg 2020 09;31(3):408-410

Department of Surgery, Division of Cardiothoracic Surgery, University of Minnesota, Minneapolis, MN, USA.

Carcinoid tumours of the heart occur most commonly as a result of metastatic disease and usually affect the right side of the heart. We report a case of a solitary carcinoid metastasis to the interventricular septum without hepatic involvement in a 74-year-old man. Read More

Am Surg 2021 Jan 28;87(1):128-130. Epub 2020 Aug 28.

Department of Surgery, Ralph H. Johnson VA Medical Center, Charleston, SC, USA.

Neurofibromatosis type I (NF1) is an autosomal dominant genetic disorder associated with characteristic skin findings, as well as a fourfold increase in risk of malignancy. NF1 patient malignancies commonly include the central and peripheral nervous system, but these patients are also at high risk of developing gastrointestinal (GI) tumors. While most often these GI tumors are benign upper GI neurofibromas; clinicians should have a high suspicion for malignant tumors, degeneration into a malignant peripheral nerve sheath tumor or less common associated malignancies such as well-differentiated neuroendocrine tumor (formerly carcinoid tumor), when patients present with multiple GI tumors. Read More

Am J Physiol Gastrointest Liver Physiol 2020 10 26;319(4):G494-G501. Epub 2020 Aug 26.

Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.

Small intestinal neuroendocrine tumors (SI-NET) are serotonin-secreting well-differentiated neuroendocrine tumors of putative enterochromaffin (EC) cell origin. Recent studies recognize a subset of EC cells that is label-retaining at the +4 position in the crypt and functions as a reserve intestinal stem cell. Importantly, this +4 reserve EC cell subset not only contributes to regeneration of the intestinal epithelium during injury and inflammation but also to basal crypt homeostasis at a constant rate. Read More

Intern Med 2020 Dec 4;59(23):3001-3007. Epub 2020 Aug 4.

Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan.

A 38-year-old Japanese man who had been diagnosed with appendiceal carcinoid and undergone ileocecal resection 8 years before presented with duodenal obstruction caused by a submucosal tumor-like appearance. He was diagnosed with long-term recurrence of appendiceal goblet cell carcinoid (GCC) with a multi-morphological pattern based on the histological assessment of a duodenal biopsy and his previously resected appendix. He underwent subtotal stomach-preserving pancreaticoduodenectomy combined with resection of an ileo-colic anastomotic lesion. Read More

J Clin Med 2020 Jul 21;9(7). Epub 2020 Jul 21.

Digestive Surgery Department, Reims University Hospital, Robert Debré Hospital, F-51092 Reims, France.

Small-intestinal neuroendocrine tumors (SI-NETs) are the most prevalent small bowel neoplasms with an increasing frequency. In the multimodal management of SI-NETs, surgery plays a key role, either in curative intent, even if R0 resection is feasible in only 20% of patients due to advanced stage at diagnosis, or palliative intent. Surgeons must be informed about the specific surgical management of SI-NETs according to their hormonal secretion, their usual dissemination at the time of diagnosis and the need for bowel-preserving surgery to avoid short bowel syndrome. Read More

Gastroenterology 2020 11 15;159(5):1686-1694.e2. Epub 2020 Jul 15.

Department of Medical Epidemiology and Biostatistics, Karolinska Institute, Stockholm, Sweden; Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York; Department of Paediatrics, Örebro University Hospital, Örebro, Sweden; Division of Epidemiology and Public Health, School of Medicine, University of Nottingham, Nottingham, UK.

Background & Aims: The incidence of small bowel cancers is increasing. Associations have been made between celiac disease (CD) and small bowel cancers, but there have been no detailed studies of large cohorts.

Methods: Through the nationwide Epidemiology Strengthened by Histopathology Reports in Sweden cohort study, we retrieved data from Sweden's 28 pathology departments on all individuals who received a diagnosis of CD from 1965 through 2017. Read More

Pol Arch Intern Med 2020 10 9;130(10):903-905. Epub 2020 Jul 9.

Department of Endocrinology, Metabolism and Internal Diseases, Poznan University of Medical Sciences, Poznań, Poland

Asian J Surg 2020 Oct 4;43(10):1033-1034. Epub 2020 Jul 4.

Department of Surgery, Fujita Health University School of Medicine, Japan. Electronic address:

Gan To Kagaku Ryoho 2020 May;47(5):839-842

Dept. of Surgery, Kurashiki Central Hospital.

A 67-year-old man presented with abdominal distention and vomiting.Computed tomography revealed bowel obstruction due to a cecal tumor.We performed laparoscopic ileocecal resection after decompression with an ileus tube. Read More

Surg Clin North Am 2020 Jun 1;100(3):635-648. Epub 2020 Apr 1.

Section of General, Vascular, and Thoracic Surgery, Virginia Mason Medical Center, 1100 Ninth Avenue, CS-G6, Seattle, WA 98101, USA; University of Washington, Seattle, WA, USA. Electronic address:

Incidence of neuroendocrine tumors (NETs) is increasing, including those of the gastroenteropancreatic tract. A proper understanding of the management of this disease has become necessary for the general surgeon. This article addresses current guidelines for diagnosis and localization of NETs, including somatostatin receptor PET. Read More

World J Gastroenterol 2020 Apr;26(13):1513-1524

Department of Nuclear Medicine, Santiago de Compostela´s University Hospital, Santiago de Compostela 15706, A Coruña, Spain.

Background: Lu peptide receptor radionuclide therapy (PRRT) is a recently approved therapy in Spain that has been demonstrated to be a well-tolerated therapy for positive somatostatin receptor advanced gastroenteropancreatic neuroendocrine tumors.

Aim: To determine the impact of PRRT on quality of life, radiologic and metabolic response, overall survival, prognostic factors and toxicity.

Methods: Thirty-six patients treated with Lu-PRRT from 2016 to 2019 were included. Read More

Neuro Endocrinol Lett 2019 Dec;40(7-8):315-318

Poznan University of Medical Sciences, Department of Endocrinology, Metabolism and Internal Medicine, Przybyszewskiego 49, 60-355 Poznan, Poland.

Background: The assessment of hormonal function of neuroendocrine neoplasm (NEN) is an important stage in the diagnosis and monitoring of these diseases treatment. Objective of this study was to analyze the results of urinary excretion of 5-hydroxyindoloacetic acid (5-HIAA) in patients with carcinoid syndrome treated with somatostatin analogues, depending on the histologic maturity, degree of liver involvement and stage of the disease.

Methods: The final group comprised of 41 patients. Read More

ANZ J Surg 2020 10 9;90(10):1975-1978. Epub 2020 Apr 9.

Department of General Surgery, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.

Background: Carcinoid tumours of the appendix are the most common primary malignant lesion of the appendix. However, the overall incidence remains low; found in as few as 0.3-0. Read More

Gan To Kagaku Ryoho 2019 Dec;46(13):2413-2415

Dept. of Gastrointestinal and Pediatric Surgery, Tokyo Medical University.

Although there are reports of goblet cell carcinoid(GCC)treated by chemotherapy using the treatment protocol for colon cancer, the benefit of chemotherapy for GCC remains controversial and unclear. Herein we report 2 cases of patients with GCC who were successfully treated by surgical resection and oxaliplatin-based adjuvant chemotherapy, without evidence of recurrence. The first case was a 57-year-old man who underwent laparoscopic ileocecal resection after being diagnosed with adenocarcinoma of the appendix by biopsy via colonoscopy. Read More

Chirurgia (Bucur) 2020 Jan-Feb;115(1):102-111

Goblet cell carcinoids (GCC) are extremelyrare neuroendocrine tumours, and characterised by their unique combination of two types of cancer cells âÃÂ" neuroendocrine (carcinoid) and epithelial (adeno-carcinoma). In spite of the fact that GCC is regarded as Neuro-Endocrine Tumour (NET), it does not illicit carcinoid syndrome. GCC usually arises in the appendix and accounting for less than 14% of all appendiceal tumours. Read More

Am J Surg 2020 05 25;219(5):795-799. Epub 2020 Feb 25.

Division of Surgical Oncology, Oregon Health & Science University, USA. Electronic address:

Introduction: Neuroendocrine tumors (NETs) metastatic to the ovary are traditionally considered rare, but data are lacking. This study seeks to better characterize the prevalence and outcomes of patients with neuroendocrine ovarian metastases (NOM).

Methods: Women with well-differentiated lung and gastroenteropancreatic NETs 2007-2017 were identified by medical record query. Read More

Int J Mol Sci 2020 Feb 29;21(5). Epub 2020 Feb 29.

Department of Endocrinology, "La Paz" University Hospital. Paseo de la Castellana, 261, 28046 Madrid, Spain.

Somatostatin analogs are an invaluable therapeutic option in the diagnosis and treatment of somatotropinomas, thyrotropinomas, and functioning and non-functioning gastroenteropancreatic neuroendocrine tumors. They should also be considered an effective and safe therapeutic alternative to corticotropinomas, gonadotropinomas, and prolactinomas resistant to dopamine agonists. Somatostatin analogs have also shown to be useful in the treatment of other endocrine diseases (congenital hyperinsulinism, Graves' orbitopathy, diabetic retinopathy, diabetic macular edema), non-endocrine tumors (breast, colon, prostate, lung, and hepatocellular), and digestive diseases (chronic refractory diarrhea, hepatorenal polycystosis, gastrointestinal hemorrhage, dumping syndrome, and intestinal fistula). Read More

Adv Exp Med Biol 2020 ;1226:87-95

Department of Medical and Surgical Sciences, Section of Surgery, University Hospital of Modena, Modena (MO), Italy.

The pathological features of the appendix tumors fundamentally recall those of the more frequent colorectal neoplasms, although with a higher relative incidence of carcinoids, due to the abundant presence of enteroendocrine cells in the appendix wall. Moreover, different types of lymphomas, Hodgkin and non-Hodgkin, arising from the extra-nodal mucosal-associated lymphatic tissue, can be encountered. The appendix tumor microenvironment (TME) consists of a cellular component and of a noncellular component: the former includes the immunocompetent cells, while the latter represents the support stroma. Read More