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Pancreas 2022 Apr;51(4):319-324

Surgery, City of Hope National Medical Center, Duarte, CA.

Objectives: There is a lack of effective patient education regarding diagnosis/treatment of neuroendocrine tumors (NETs), possibly related to their rare incidence.

Methods: In this cross-sectional survey study, NET patients attending the 2019 Annual Los Angeles NET Education Conference were approached to complete NET VITALS, a self-assessment tool gauging patients' perception/awareness of their NET diagnosis/treatment, and a satisfaction survey. Feasibility of NET VITALS, patient satisfaction with NET VITALS, and patients' perception/awareness of their NET diagnosis/treatment were evaluated. Read More

Front Oncol 2022 7;12:911294. Epub 2022 Jun 7.

Department of Medical Oncology and Hematology, University Hospital Zurich, Zurich, Switzerland.

Background: () rearrangements are known oncogenic drivers in non-small cell lung cancer (NSCLC). Few case reports described the occurrence of such rearrangements in large cell neuroendocrine carcinomas (LCNECs) of the lung without information on clinical responses to ALK tyrosine kinase inhibitors (TKIs) in these cases. Currently, neuroendocrine tumors of the lungs are not screened for rearrangements. Read More

Proc (Bayl Univ Med Cent) 2022 19;35(4):537-539. Epub 2022 Apr 19.

Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas.

This case presents a patient with ectopic secretion of adrenocorticotrophic hormone from a carcinoid tumor resulting in severe hypertension, posterior reversible encephalopathy syndrome (PRES), and refractory status epilepticus. Secondary hypertension may be difficult to identify in critically ill patients. Severely uncontrolled hypertension presenting with PRES caused by Cushing syndrome is rare but should be considered. Read More

BMJ Case Rep 2022 Jun 24;15(6). Epub 2022 Jun 24.

Gynaecologic Oncology, Dr B Borooah Cancer Society Trust, Guwahati, Assam, India.

Carcinoid tumours are present in a wide range of organs but most frequently involve the gastrointestinal tract and rarely reported in gynaecological organs. Literature reports that the prevalence of ovarian carcinoid is 0.3%-1% of ovarian neoplasms and accounts for only 5% of ovarian teratomas. Read More

Cancers (Basel) 2022 Jun 16;14(12). Epub 2022 Jun 16.

Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL 33612, USA.

Background: Surgery is the only curative option for patients with neuroendocrine tumors (NET) and is also indicated for debulking of liver metastasis. Intraoperative carcinoid crisis (CC) is thought to be a potentially lethal complication. Though perioperative octreotide is often recommended for prevention, recent NET society guidelines raised concerns regarding limited data supporting its use. Read More

Cancers (Basel) 2022 Jun 8;14(12). Epub 2022 Jun 8.

Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, UT 84132, USA.

PET-CT is an advanced imaging modality with many oncologic applications, including staging, therapeutic assessment, restaging and surveillance for recurrence. The goal of this series of six review articles is to provide practical information to providers and imaging professionals regarding the best use of PET-CT for specific oncologic indications, the potential pitfalls and nuances that characterize these applications, and guidelines for image interpretation. Tumor-specific clinical information and representative PET-CT images are provided. Read More

JCO Precis Oncol 2022 Jun;6:e2200009

Division of Hematology and Oncology, Department of Medicine, University of California, San Francisco, San Francisco, CA.

Curr Med Sci 2022 Jun 21. Epub 2022 Jun 21.

Department of Pulmonology, Children's Hospital of Chongqing Medical University, Chongqing, 400014, China.

Objective: To determine the clinical characteristics and prognosis of primary tracheobronchial tumors (PTTs) in children, and to explore the most common tumor identification methods.

Methods: The medical records of children with PTTs who were hospitalized at the Children's Hospital of Chongqing Medical University from January 1995 to January 2020 were reviewed retrospectively. The clinical features, imaging, treatments, and outcomes of these patients were statistically analyzed. Read More

World J Gastrointest Endosc 2022 May;14(5):267-290

Division of Gastroenterology Hepatology and Endoscopy, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, United States.

Gastroenteropancreatic neuroendocrine neoplasms are a heterogenous group of rare neoplasms that are increasingly being discovered, often incidentally, throughout the gastrointestinal tract with varying degrees of activity and malignant potential. Confusing nomenclature has added to the complexity of managing these lesions. The term carcinoid tumor and embryonic classification have been replaced with gastroenteropancreatic neuroendocrine neoplasm, which includes gastrointestinal neuroendocrine and pancreatic neuroendocrine neoplasms. Read More

Cancer Epidemiol 2022 Jun 16;79:102197. Epub 2022 Jun 16.

Faculty of Medicine, University of Iceland, Reykjavik, Iceland; Division of Gastroenterology and Hepatology, Department of Internal Medicine, Landspitali University Hospital of Iceland, Reykjavik, Iceland. Electronic address:

Background & Aims: Small intestinal neuroendocrine tumours (SI-NETs) are the most frequent malignant tumours of the small intestine. Population based studies on SI-NETs are scarce. We aimed to examine the incidence, presentation of disease and prognosis of SI-NET and to determine patient prognosis in those undergoing emergency or elective surgery. Read More

Surg Pathol Clin 2022 Jun 18;15(2):407-420. Epub 2022 May 18.

Department of Cellular Pathology, Birmingham Women's Hospital, Birmingham, United Kingdom.

Neuroendocrine neoplasia is relatively uncommon in the female genital tract (FGT) and occurs at any site, most often the ovary and cervix. A unified dichotomous nomenclature, introduced by the World Health Organization Classification of Tumors in all fifth edition volumes, divides neuroendocrine neoplasms (NENs) into well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The term carcinoid tumor is retained in the ovary and represents the commonest FGT NEN. Read More

Medicine (Baltimore) 2022 Jun 17;101(24):e29464. Epub 2022 Jun 17.

General Surgery Department, IRCCS MultiMedica, Italy.

Introduction: Neuroendocrine tumours (NETs) are rare tumors. 55% of NETs originate in the gastrointestinal tract and the liver is the most common site of distant metastases. Serum chromogranin A is the most common biomarker for assessing the extent of disease and monitoring treatment; carcinoid syndrome occurs in 19% of NETs and is characterized by chronic diarrhea or flushing. Read More

J Am Coll Surg 2022 Jun 17;234(6):1082-1089. Epub 2022 Mar 17.

Division of General Surgery, Trauma, and Surgical Critical Care, Department of Surgery (Schuster), Yale School of Medicine, New Haven, Connecticut.

Background: Nonoperative management of acute appendicitis is increasingly common. However, small studies have demonstrated high rates of appendiceal cancer in interval appendectomy specimens. Therefore, we sought to identify national trends in appendiceal cancer incidence and histology. Read More

J Endocr Soc 2022 Jul 3;6(7):bvac083. Epub 2022 Jun 3.

Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK.

Acromegaly due to ectopic secretion of growth hormone-releasing hormone (GHRH) is rare. Treatment consists of surgical removal of the primary tumor, cytostatic therapy, "cold" or radioactive somatostatin analogue treatment, and medical therapy for acromegaly, if needed. A 53 year-old female had an ocular lesion noted on a routine optician visit, originally considered to be an ocular melanoma. Read More

Endocrine 2022 Jun 13. Epub 2022 Jun 13.

Medical Research Center Oulu, Oulu University Hospital, Research Unit of Internal Medicine, University of Oulu, Oulu, Finland.

Objective: MEN1 is associated with an increased risk of developing tumors in different endocrine organs. Neuroendocrine tumors of the thymus (TNETs) are very rare but often have an aggressive nature. We evaluated patients with MEN1 and TNET in three university hospitals in Finland. Read More

BMC Surg 2022 Jun 11;22(1):226. Epub 2022 Jun 11.

Department of Thoracic Surgery, University Medical Center Mainz, Johannes Gutenberg University Mainz, Mainz, Germany.

Background: Ectopic Cushing syndrome (ECS) is a sporadic condition. Even uncommon is an ECS that derives from a carcinoid tumor of the thymus. These tumors may pose several diagnostic and therapeutic conundrums. Read More

Oncotarget 2022 1;13:800-809. Epub 2022 Jun 1.

Department of Pathology, Antwerp University Hospital, Edegem, Belgium.

Background: Novel targets in neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) are needed to improve outcome. The presence of O6-Methylguanine-DNA methyltransferase (MGMT) promoter methylation in NETs and NECs may act as a predictive marker for response on treatment with temozolomide. As anaplastic lymphoma kinase (ALK) plays an important role in the nervous system we hypothesized that ALK rearrangement can act as a biomarker in patients with NETs and NECs. Read More

Neuroendocrinology 2022 Jun 7. Epub 2022 Jun 7.

Introduction: The European Neuroendocrine Tumor Society, ENETS, reports variables of prognostic significance in bronchopulmonary neuroendocrine neoplasms. The aim of this study was to investigate prognostic factors, recurrence free survival (RFS) and overall survival (OS) for patients with typical carcinoid (TC), atypical carcinoid (AC) and large cell neuroendocrine carcinoma (LCNEC). Current follow-up practices vary as the evidence is sparse and we aimed to explore the relevance of routine bronchoscopy in follow-up. Read More

J Chromatogr B Analyt Technol Biomed Life Sci 2022 Jul 30;1203:123315. Epub 2022 May 30.

Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, PR China. Electronic address:

Carcinoid tumors referred to neuroendocrine neoplasms that often are indolent and may not became clinical apparent until there has been metastatic spread. Urinary 5-hydroxyindoleacetic acid (5-HIAA) was recommended as a first-line screening biomarker for the diagnosis and follow-up of carcinoid tumors. The measurement of this analyte is conventionally performed by spectrophotometer or high performance liquid chromatography, and has switched to liquid chromatography-tandem mass spectrometry (LC-MS/MS) recently. Read More

Onco Targets Ther 2022 27;15:587-596. Epub 2022 May 27.

Department of Urology, The Fifth Affiliated Hospital of Zunyi Medical University (Zhuhai Sixth People's Hospital), Zhuhai, People's Republic of China.

Primary carcinoid tumor of the kidney is an extremely rare well-differentiated neuroendocrine tumor, which is generally a low-grade malignant cancer with a good prognosis. Carcinoid tumors are rarely found in the urinary system. Here, we report a 34-year-old woman with primary renal well-differentiated neuroendocrine tumor who underwent nephron sparing surgery and no evidence of recurrence or distant metastasis was found during routine follow-up. Read More

World J Gastroenterol 2022 Apr;28(13):1304-1314

Department of General and Digestive Surgery, Hospital Universitario de La Princesa, Madrid 28006, Spain.

Appendiceal neuroendocrine tumors (aNETs) are an uncommon neoplasm that is relatively indolent in most cases. They are typically diagnosed in younger patients than other neuroendocrine tumors and are often an incidental finding after an appendectomy. Although there are numerous clinical practice guidelines on management of aNETs, there is continues to be a dearth of evidence on optimal treatment. Read More

Oncologist 2022 03;27(2):e116-e125

The Second Department of Thoracic Oncology, Hunan Cancer Hospital/The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, People's Republic of China.

Background: Pulmonary neuroendocrine tumors (pNETs) include typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC), and small cell lung carcinoma (SCLC). The optimal treatment strategy for each subtype remains elusive, partly due to the lack of comprehensive understanding of their molecular features. We aimed to explore differential genomic signatures in pNET subtypes and identify potential prognostic and therapeutic biomarkers. Read More

Radiol Imaging Cancer 2022 May;4(3):e220038

From the Department of Radiology, Mayo Clinic, 200 1st St SW, Rochester, MN 55902.

Front Oncol 2022 9;12:842703. Epub 2022 May 9.

Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Obstetric & Gynecologic Diseases, Beijing, China.

Objective: To investigate the clinical characteristics and survival outcomes of patients with malignant transformation arising from ovarian mature cystic teratoma (MT-MCT).

Methods: This retrospective study included patients with ovarian MCTs at Peking Union Medical College Hospital (PUMCH) during 1990.01-2020. Read More

J Neuroendocrinol 2022 Apr 25:e13146. Epub 2022 Apr 25.

Centre for Gastroenterology, Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free Hospital, London, UK.

J Surg Case Rep 2022 May 22;2022(5):rjac128. Epub 2022 May 22.

Chicago Medical School at Rosalind Franklin University of Medicine and Science, North Chicago, IL, USA.

Meckel's diverticulum is a congenital anomaly leading to the formation of a true diverticulum in the distal small intestine. Though most are asymptomatic and discovered incidentally, Meckel's diverticuli can give rise to a wide range of symptoms. Rarely, this can be a malignancy, most commonly a carcinoid tumor. Read More

Monaldi Arch Chest Dis 2022 May 24. Epub 2022 May 24.

Department of Respiratory Diseases, Bolzano Hospital.

Oxidized cellulose, used as hemostatic in thoracic surgery, may cause in some cases foreign body reactions, and simulate other diseases. We report the case of a 39-year-old man operated on a middle lobe lobectomy for atypical carcinoid. The follow up chest-CT showed enlarged mediastinal lymph nodes, so endobronchial ultrasound-guided transbronchial needle aspiration was performed suspecting recurrence of the tumor. Read More

N Engl J Med 2022 05 21;386(21):e56. Epub 2022 May 21.

MedStar Union Memorial Hospital, Baltimore, MD

J Surg Case Rep 2022 May 17;2022(5):rjac180. Epub 2022 May 17.

Divion of General Surgery, Department of Surgery, King Abdulaziz Medical City, National Guard Health Affairs, Riyadh, Saudi Arabia.

Carcinoid tumors have been treated with formal oncological surgical resection, which is considered the gold standard approach. In the past two decades, bronchoscopic has gained popularity for treatment of carcinoid tumors. A 34-year-old female with an unresolving chronic cough, underwent a chest CT that showed right endobronchial lesion. Read More

J Nucl Med 2022 May 19. Epub 2022 May 19.

Nuclear Medicine and Molecular Imaging, Institut de Cancérologie Strasbourg Europe (ICANS), France.

To compare the respective value of Ga-DOTATOC and F-DOPA PET/CT for initial staging or pre-surgical work-up of patients with small intestine neuroendocrine tumors (SiNET). This is a retrospective, multicenter, non-interventional investigation involving 53 non-operated SiNET patients who underwent both Ga-DOTATOC and F-DOPA PET/CT within a 6-months interval without therapeutic intervention or change between the two PET/CT studies. Detection rate (DR %) was calculated according to per-region and per-lesion analyses. Read More