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Acta Med Litu 2020 ;27(1):17-24

Centre of Abdominal Surgery, Clinic of Gastroenterology, Nephrourology and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania.

High-grade well-differentiated neuroendocrine tumour tumours (NETs) of gastrointestinal tract are rare; they can arise in any part of the digestive system and usually present in advanced stages. Low incidence and wide heterogeneity in the biological behaviour of such lesions pose a diagnostic and therapeutic challenge. Loco-regional NETs should be resected whenever feasible, potentially with a curative intent. Read More

Balkan Med J 2020 Jun 23. Epub 2020 Jun 23.

Department of Medical Oncology, Hacettepe University Cancer Institute, Ankara, Turkey.

Background: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogenous group of neoplasms originating from the neuroendocrine system of the gastrointestinal tract and pancreas. There aren't many large well-designed studies investigating NETs in Turkey.

Aims: To define the clinicopathologic, demographic and survival features of patients with GEPNETs. Read More

Radiographics 2020 Jul-Aug;40(4):1020-1038. Epub 2020 Jun 19.

From the Department of Radiology, Virginia Commonwealth University Health System, 1250 E Marshall St, Richmond, VA 23298.

Small bowel tumors are rare and account for approximately 3% of all gastrointestinal tumors in the United States. The incidence of small bowel neoplasms is rising-in particular, there is a rising incidence of small bowel carcinoid tumors. This may in part be due to small bowel lesion identification in an increasing number of cross-sectional imaging studies performed for other indications as well as increased use of advanced imaging techniques to assess specifically for small bowel disease. Read More

Ophthalmic Plast Reconstr Surg 2020 Jun 2. Epub 2020 Jun 2.

Division of Orbital and Ophthalmic Plastic Surgery and*Department of Ophthalmology, Columbia University College of Physicians and Surgeons, New York, New York.

Purpose: Well-differentiated neuroendocrine or carcinoid tumors are found most commonly in the gastrointestinal tract. When metastatic to the orbit, they tend to have a propensity for the extraocular muscles. The purpose of this study was to better understand the diversity in presentation of orbital carcinoid disease and to determine predictors for survival. Read More

Lung Cancer 2020 Jul 5;145:85-94. Epub 2020 May 5.

Division of Chemotherapy and Clinical Research, National Cancer Center Research Institute, Tokyo, Japan; Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, Tokyo, Japan.

Introduction: The majority of pulmonary carcinoid (PC) tumors can be cured by surgical resection alone, but a significant proportion of patients experience recurrence. As PC is insensitive to conventional chemotherapy, further clarification of the molecular mechanisms of metastasis is needed in order to develop targeted therapeutics.

Methods: We performed comprehensive whole-exome sequencing (WES) of primary tumors and corresponding normal lung tissues from 14 PC patients (including 4 patients who developed postsurgical distant metastasis) and RNA sequencing of primary tumors from 6 PC patients (including 4 patients who developed postsurgical distant metastasis). 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

Curr Treat Options Oncol 2020 Apr 30;21(6):50. Epub 2020 Apr 30.

Gastrointestinal Oncology, Intermountain Healthcare, 5171 S. Cottonwood Street, Bldg. 1. Ste. 610, Murray, UT, 84107, USA.

Opinion Statement: Oncologists should be able to discern the salient clinical features of the most common germline mutations that give rise to neuroendocrine tumors. Astute recognition of an index patient affected by a hereditary syndrome can lead to a "tip-of-the-iceberg" phenomenon whereby their entire kindred can then be proactively monitored and managed potentially with substantial reduction of morbidity and mortality. Through careful history-taking, as well as thoughtful assimilation of findings from the physical exam, biochemical laboratories, scans, and pathology reports, the clinician can spot phenotypic clues that distinguish these familial patterns from sporadic cases of tumorigenesis. 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

Diagn Pathol 2020 Apr 3;15(1):32. Epub 2020 Apr 3.

Department of Pathology, The Affiliated Hospital of Zunyi Medical University, Zunyi, 563000, Guizhou, China.

Background: Neuroendocrine tumors (NETs) can secrete bioactive amines in the bloodstream, resulting in the carcinoid syndrome characterized by diarrhea and flushing. The frequency of occurrence of primary cardiac neuroendocrine neoplasms is lesser than that of metastases, and hence, metastases must be adequately ruled out before diagnosis. Cardiac tumors, both primary and metastatic, mainly result in heart-related symptoms, such as heart failure and acquired valvular dysfunction. Read More

Clin J Oncol Nurs 2020 Apr;24(2):129-133

University of Kentucky.

Neuroendocrine tumors (NETs) are comprised of biologically diverse neoplasms. The presence of systemic symptoms is dependent on NET location and differentiation. New treatment modalities have become available, offering patients improved symptom management and survival. 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

Pancreas 2020 03;49(3):408-412

Medical Affairs, Lexicon Pharmaceuticals, Inc, The Woodlands, TX.

Objectives: We evaluated carcinoid syndrome (CS) symptoms and the real-world effectiveness of telotristat ethyl (TE) among patients with ≤3 bowel movements (BM) per day.

Methods: Patients with CS initiating TE between March and November 2017 could participate in a nurse support program collecting demographic and CS symptom data before TE initiation (baseline) and during ≥1 monthly follow-up within 3 months. Symptoms for patients averaging ≤3 BM/d at baseline were evaluated using pre/post-Student t tests. 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

Horm Metab Res 2020 Feb 27. Epub 2020 Feb 27.

1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece.

Neuroendocrine neoplasms (NENs) are rare tumours that arise mainly in the gastrointestinal or pulmonary system. Most NENs are well-differentiated and may obtain prolonged survival besides the presence of metastatic disease; however, a subset (poorly differentiated NENs) may display a truly aggressive behaviour exhibiting a poor prognosis. The recently developed classification systems along with advances in functional imaging have helped stratify patients to the administration of appropriate therapeutic options. Read More

Cardiol Res 2020 Feb 26;11(1):56-60. Epub 2020 Jan 26.

Division of Cardiovascular Sciences, University of South Florida Morsani College of Medicine, Tampa, FL 33606, USA.

Background: Carcinoid heart disease (CaHD) is a rare condition that has a high impact on the morbidity and mortality of its patients. Once heart failure symptoms develop in the patient with CaHD, cardiac valve surgery is often the only effective treatment. Although atrioventricular block (AVB) is a known postoperative complication of the valve surgery, the incidence of AVB in this population has not been well described. Read More

Int J Surg Case Rep 2020 11;67:262-266. Epub 2020 Feb 11.

Gastrointestinal Clinical Institute, Epworth Healthcare, Victoria, Australia; Department of Cancer Surgery, Peter MacCallum Cancer Centre, Victoria, Australia; Department of Surgery, Alfred Health, Victoria, Australia. Electronic address:

Introduction: Neuroendocrine tumours are the most common type of primary small bowel neoplasm. Consensus guidelines recommend a multimodal approach to treatment of such tumours, with aggressive surgical resection remaining the mainstay of management. There is evidence that complete mesocolic excision (CME) of lymph nodes is associated with superior oncological outcomes including longer disease-free survival in patients with colorectal cancer than standard lymph node dissection and there is increasing evidence to suggest that the robotic approach may be superior to laparoscopic or open CME. Read More

Cureus 2020 Jan 13;12(1):e6641. Epub 2020 Jan 13.

Gastroenterology, AdventHealth, Orlando, USA.

Small bowel neoplasms are rare, accounting for only 3%-6% of all gastrointestinal neoplasms. Carcinoid tumors represent a large portion of these (20%-30%), making them the second most common small bowel malignancy after adenocarcinoma. Gastrointestinal carcinoids constitute 70% of all neuroendocrine tumors, and out of those, 42% originate in the small bowel. Read More

Surg Case Rep 2020 Feb 13;6(1):36. Epub 2020 Feb 13.

The Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan.

Background: Neuroendocrine tumor (NET) is a relatively rare tumor and can develop in almost any organ, but primary mesenteric NETs are extremely rare. In addition, liver metastases from synchronous double cancer of neuroendocrine tumor graded as G1 and second primary malignancies (SPMs) have never been reported before. We herein report a case of multiple liver metastases from synchronous double cancer of NET (G1) at the ileal mesentery and rectal cancer. Read More

Int J Mol Sci 2020 Jan 19;21(2). Epub 2020 Jan 19.

Departments of Medicine & Biochemistry, Division of Gastroenterology and Hepatology, University of New Mexico School of Medicine and Raymond G. Murphy Veterans Administration Medical Center, 1501 San Pedro Blvd. SE, Albuquerque, NM 87108, USA.

Neuroendocrine tumors (NETs) throughout the body are the focus of much current interest. Most occur in the gastrointestinal tract and have shown a major increase in incidence over the past 30 years, roughly paralleling the world-wide increase in the use of proton pump inhibitor (PPI) drugs. The greatest rise has occurred in gastric carcinoids (g-NETs) arising from enterochromaffin-like (ECL) cells. Read More

Case Rep Cardiol 2019 5;2019:8746413. Epub 2019 Dec 5.

Department of Diagnostic Imaging and Interventional Radiology, H. Lee Moffitt Cancer Center and Research Institute, Tampa FL, USA.

A 76-year-old male with a small bowel neuroendocrine tumor with hepatic metastases presented with new onset lower extremity swelling, bloating, and weight gain which ultimately lead to cardiac magnetic resonance (CMR) to evaluate for cardiac involvement of disease. CMR showed right and left ventricular myocardial metastases along with findings suggestive of carcinoid heart disease. The patient had severe tricuspid valve regurgitation necessitating surgical valve repair. Read More

World J Gastroenterol 2019 Dec;25(47):6857-6865

Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.

Background: The burden of carcinoid syndrome (CS) among patients with neuroendocrine tumors is substantial and has been shown to result in increased healthcare resource use and costs. The incremental burden of CS diarrhea (CSD) is less well understood, particularly among working age adults who make up a large proportion of the population of patients with CS.

Aim: To estimate the direct medical costs of CSD to a self-insured employer in the United States. Read More

J Gastroenterol Hepatol 2020 Mar 1;35(3):359. Epub 2019 Dec 1.

Department of Gastroenterology and Hepatology, Kalinga Institute of Medical Sciences, Bhubaneswar, India.

BMJ Case Rep 2019 Nov 28;12(11). Epub 2019 Nov 28.

Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, USA.

Atypical pulmonary carcinoid (APC) is a lung neuroendocrine neoplasm (NEN), whose treatment draws from management of gastrointestinal NENs and small-cell lung carcinoma. We present a patient with recurrent metastatic APC and persistent mediastinal lymphadenopathy refractory to cisplatin and etoposide. After pursuing alternative treatments, he returned with significant progression, including diffuse subcutaneous nodules, weight loss and worsening cough. Read More

World J Gastrointest Endosc 2019 Nov;11(11):541-547

Department of Medical Gastroenterology, Government Stanley Medical College, Chennai 600001, Tamil Nadu, India.

Background: Oesophageal cancer is the fourth most common cause of cancer-related deaths in India. Esophageal squamous cell carcinomas (ESCCs) arise from the epithelial layer, and commonly present as polypoidal, ulcerative or ulceroproliferative growth in the oesophageal lumen. In contrast, oesophageal submucosal tumours are a distinct group of tumours arising from the mesenchyme (examples include leiomyoma, fibrovasculoma, lipoma, granular cell tumour or carcinoid), and mostly do not breach the mucosa. Read More

Endocr Connect 2019 Dec;8(12):R184-R199

Birmingham Neuroendocrine Tumour Centre, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK.

Carcinoid heart disease (CHD) is a consequence of valvular fibrosis triggered by vasoactive substances released from neuroendocrine tumours, classically in those with metastatic disease and resulting in tricuspid and pulmonary valve failure. CHD affects one in five patients who have carcinoid syndrome (CS). Valve leaflets become thickened, retracted and immobile, resulting most often in regurgitation that causes right ventricular dilatation and ultimately, right heart failure. Read More

Arch Pathol Lab Med 2020 Jun 12;144(6):764-768. Epub 2019 Nov 12.

From the Department of Pathology, University of Utah, Salt Lake City (Dr Jedrzkiewicz); the Department of Pathology, Yokohama City University, Yokohama, Japan (Dr Tateishi); and the Departments of Pathology (Drs Kirsch, Conner, Riddell, and Pollett) and Surgery (Drs Bischof, McCart, and Govindarajan, and Ms Taylor), Sinai Health System, University of Toronto, Toronto, Ontario, Canada.

Context.—: Data regarding the clinical impact of subspecialist pathology review of appendiceal neoplasms are limited.

Objective. Read More