Neuroendocrine Tumors of the Pancreas (Including Insulinoma, Gastrinoma, Glucogacoma, Vipoma, Somatostatinoma)

Total Page：16

File Type：pdf, Size：1020Kb

Neuroendocrine tumors of the pancreas (including insulinoma, gastrinoma, glucogacoma, VIPoma, somatostatinoma) Neuroendocrine pancreatic tumors (pancreatic NETs or pNETs) account for about 3% of all primary pancreatic tumors. They develop in neuroendocrine cells called islet cells. Neuroendocrine tumors of the pancreas may be nonfunctional (not producing hormones) or functional (producing hormones). Most pNETs do not produce hormones and, as a result, these tumors are diagnosed incidentally or after their growth causes symptoms such as abdominal pain, jaundice or liver metastasis. pNETs that produce hormones are named according to the type of hormone they produce and / or clinical manifestation: Insulinoma - An endocrine tumor originating from pancreatic beta cells that secrete insulin. Increased insulin levels in the blood cause low glucose levels in blood (hypoglycemia) with symptoms that may include sweating, palpitations, tremor, paleness, and later unconsciousness if treatment is delayed. These are usually benign and tend to be small and difficult to localize. Gastrinoma - a tumor that secretes a hormone called gastrin, which causes excess of acid secretion in the stomach. As a result, severe ulcerative disease and diarrhea may develop. Most gastrinomas develop in parts of the digestive tract that includes the duodenum and the pancreas, called "gastrinoma triangle". These tumors have the potential to be malignant. Glucagonoma is a rare tumor that secretes the hormone glucagon, which may cause a typical skin rash called migratory necrolytic erythema, elevated glucose levels, weight loss, diarrhea and thrombotic events. VIPoma - a tumor that secretes Vasoactive peptide (VIP) hormone causing severe diarrhea. The diagnosis is made by finding a pancreatic neuroendocrine tumor with elevated VIP hormone in the blood and typical clinical symptoms. Somatostatinoma – a tumor that secretes the somatostatin hormone. An excess of this hormone may cause gallstones, diarrhea and weight loss. In addition, neuroendocrine tumors in the pancreas can secrete other hormones such as calcitonin and PTH like peptide. .

Copy Link

Recommended publications

Endo4 PRINT.Indb
Contents 1 Tumours of the pituitary gland 11 Spindle epithelial tumour with thymus-like differentiation 123 WHO classifi cation of tumours of the pituitary 12 Intrathyroid thymic carcinoma 125 Introduction 13 Paraganglioma and mesenchymal / stromal tumours 127 Pituitary adenoma 14 Paraganglioma 127 Somatotroph adenoma 19 Peripheral nerve sheath tumours 128 Lactotroph adenoma 24 Benign vascular tumours 129 Thyrotroph adenoma 28 Angiosarcoma 129 Corticotroph adenoma 30 Smooth muscle tumours 132 Gonadotroph adenoma 34 Solitary fi brous tumour 133 Null cell adenoma 37 Haematolymphoid tumours 135 Plurihormonal and double adenomas 39 Langerhans cell histiocytosis 135 Pituitary carcinoma 41 Rosai–Dorfman disease 136 Pituitary blastoma 45 Follicular dendritic cell sarcoma 136 Craniopharyngioma 46 Primary thyroid lymphoma 137 Neuronal and paraneuronal tumours 48 Germ cell tumours 139 Gangliocytoma and mixed gangliocytoma–adenoma 48 Secondary tumours 142 Neurocytoma 49 Paraganglioma 50 3 Tumours of the parathyroid glands 145 Neuroblastoma 51 WHO classifi cation of tumours of the parathyroid glands 146 Tumours of the posterior pituitary 52 TNM staging of tumours of the parathyroid glands 146 Mesenchymal and stromal tumours 55 Parathyroid carcinoma 147 Meningioma 55 Parathyroid adenoma 153 Schwannoma 56 Secondary, mesenchymal and other tumours 159 Chordoma 57 Haemangiopericytoma / Solitary fi brous tumour 58 4 Tumours of the adrenal cortex 161 Haematolymphoid tumours 60 WHO classifi cation of tumours of the adrenal cortex 162 Germ cell tumours 61 TNM classifi
[Show full text]
CANINE INSULINOMA: DIAGNOSIS, TREATMENT, & STAGING Eliza Reiss Grant, DVM, and Kristine E
Peer Reviewed PRACTICAL ONCOLOGY CANINE INSULINOMA: DIAGNOSIS, TREATMENT, & STAGING Eliza Reiss Grant, DVM, and Kristine E. Burgess, DVM, Diplomate ACVIM (Oncology) Tufts University An insulinoma is a malignant pancreatic tumor that DIAGNOSIS inappropriately secretes excessive insulin, resulting in Aside from a histologic confirmation of insulinoma, profound hypoglycemia.1 no currently available diagnostic test provides a de- Pancreatic tumors are classified as: finitive diagnosis of insulinoma. Existing techniques • Exocrine, which includes adenocarcinomas of may help increase suspicion for an insulin-secreting ductular or acinar origin tumor but, with most diagnostic testing, it is im- • Endocrine, which arise from the islets of perative to interpret all results in the context of the Langerhans. coexisting clinical signs. Insulinomas are functional neuroendocrine tumors that originate in the beta cells of the islets Differential Diagnosis of Langerhans.1 A complete work-up, including careful patient history, physical examination, bloodwork, and PRESENTATION diagnostic imaging tests, should be performed to Signalment rule out other causes of hypoglycemia, such as Any breed of dog can be affected, but large sepsis, hepatic failure, adrenal cortical insufficiency, breeds tend to be overrepresented.1 While, in toxin ingestion, and other forms of neoplasia. humans, insulinomas affect females far more frequently than males, there is no apparent sex Laboratory Tests predilection in dogs.1-3 Dogs also commonly Blood Glucose present with a malignant variant, while humans A simple fasting blood glucose level of less than often have a benign adenoma (80%).1 Insulino- 40 mg/dL can suggest hyperinsulinemia, although ma is rare in cats.4 careful monitoring of a fasted dog with suspected insulinoma is strongly recommended due to high Clinical Signs risk for seizure activity.
[Show full text]
Multiple Endocrine Neoplasia Type 1 (MEN1)
Lab Management Guidelines V1.0.2020 Multiple Endocrine Neoplasia Type 1 (MEN1) MOL.TS.285.A v1.0.2020 Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline MEN1 Known Familial Mutation Analysis 81403 MEN1 Deletion/Duplication Analysis 81404 MEN1 Full Gene Sequencing 81405 What is Multiple Endocrine Neoplasia Type 1 Definition Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomal dominant disorder characterized by the development of multiple endocrine and non-endrocrine tumors. Incidence or Prevalence MEN1 has a prevalence of 1/10,000 to 1/100,000 individuals.1 Symptoms The presenting symptom in approximately 90% of individuals with MEN1 is primary hyperparathyroidism (PHPT). Parathyroid tumors cause overproduction of parathyroid hormone which leads to hypercalcemia. The average age of onset is 20-25 years. Parathyroid carcinomas are rare in individuals with MEN1.2,3,4 Pituitary tumors are seen in 30-40% of individuals and are the first clinical manifestation in 10% of familial cases and 25% of simplex cases. Tumors are typically solitary and there is no increased prevalence of pituitary carcinoma in individuals with MEN1.2,5 © 2020 eviCore healthcare. All Rights Reserved. 1 of 8 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines V1.0.2020 Prolactinomas are the most commonly seen pituitary subtype and account for 60% of pituitary adenomas.
[Show full text]
Ganglioneuroblastoma As Vasoactive Intestinal Polypeptide-Secreting10.5005/Jp-Journals-10002-1167 Tumor: Rare Case Report in a Child Case Report
WJOES Ganglioneuroblastoma as Vasoactive Intestinal Polypeptide-secreting10.5005/jp-journals-10002-1167 Tumor: Rare Case Report in a Child CASE REPORT Ganglioneuroblastoma as Vasoactive Intestinal Polypeptide-secreting Tumor: Rare Case Report in a Child 1Basant Kumar, 2Vijai D Upadhyaya, 3Ram Nawal Rao, 4Sheo Kumar ABSTRACT than 60 cases of pediatric VIP-secreting tumors.3 Most Pathologically elevated vasoactive intestinal polypeptide (VIP) of them are either adrenal pheochromocytoma or mixed plasma levels cause secretory diarrhea with excessive loss of pheochromocytoma-ganglioneuroma tumors. Mason water and electrolyte and is characterized by the typical symp- et al,4 first described the secretory nature of neuroblas- toms of hypokalemia and metabolic acidosis. It rarely occurs toma and vasoactive intestinal peptide (VIP) can be in patients with non-pancreatic disease. Despite the clinical severity, diagnosis of a VIP-secreting tumor is often delayed. produced by the mature neurogenic tumors. We herein We herein present a 14-month-old boy having prolonged present a 14 months old boy having prolonged therapy- therapy-resistant secretory diarrhea, persistent hypokalemia resistant secretory diarrhea, persistent hypokalemia with with tissue diagnosis of ganglioneuroblastoma and raised plasma VIP-levels. tissue diagnosis of ganglioneuroblastoma and briefly review the literature. Keywords: Ganglioneuroblastoma, Secretory diarrhea, VIPoma. CASE REPORT How to cite this article: Kumar B, Upadhyaya VD, Rao RN, Kumar S. Ganglioneuroblastoma as Vasoactive Intestinal A 14-month-old boy, weighing 9 kg with advanced symp- Polypeptide-secreting Tumor: Rare Case Report in a Child. World J Endoc Surg 2015;7(2):47-50. toms of persistent secretory diarrhea, hypokalemia and metabolic acidosis was referred to us with radiological Source of support: Nil (computed tomography) diagnosis of retroperitoneal Conflict of interest: None mass.
[Show full text]
Surgical Management of Ampullary Somatostatinoma
JOP. J Pancreas (Online) 2016 Jul 08; 17(4):406-409. CASE SERIES Surgical Management of Ampullary Somatostatinoma Nicholas Phillips, Terence Huey, Joel Lewin, Anthony W Cheng, Nicholas A O’Rourke Hepato-Pancreato-Biliary Unit, Department of General Surgery, Royal Brisbane Hospital, Brisbane, Australia ABSTRACT Introduction Somatostatinomas of the ampulla are rare neuroendocrine tumours with limited studies in the literature. These are often associated with familial genetic predisposition e.g. NF 1 and Von Hippel-Lindau Syndrome. Histology commonly shows classical features such as Psammoma bodies. The classical presentation with inhibitory syndrome is rare, but ampullary mass effects can cause an earlier presentation with potentially better outcomes with earlier intervention and treatment. Case series We report three cases of ampullary the second with recurrent pancreatitis and the third, with elevated CA19-9 levels. Various preoperative localisation techniques were employedsomatostatisnomas: and one had one an sporadic attempted and endoscopic two familial, resection associated yielding with involved neurofibromatosis margins. All type patients 1. The underwent first patient pancreaticoduodenectomy, presented with pruritus, of which one was laparoscopic assisted. The median size of the tumour was 10 mm and one patient had nodal involvement. All 3 patients have remained disease free at most recent follow up ranging from 1.5 to 11 years. Discussion Ampullary somatostatinomas can present early with mass related effects while inhibitory syndrome is rare. Early detection and intervention in ampullary somatostatinoma may contribute to better outcomes than pancreatic somatostatinomas. Long-term survival is achievable through pancreaticoduodenectomy for resectable ampullary somatostatinoma and laparoscopic approach is a feasible and viable option. INTRODUCTION cases of ampullary somatostatinoma that were treated by pancreaticoduodenectomy (PD) with good outcomes.
[Show full text]
Liver, Gallbladder, Bile Ducts, Pancreas
Liver, gallbladder, bile ducts, pancreas Coding issues Otto Visser May 2021 Anatomy Liver, gallbladder and the proximal bile ducts Incidence of liver cancer in Europe in 2018 males females Relative survival of liver cancer (2000 10% 15% 20% 25% 30% 35% 40% 45% 50% 0% 5% Bulgaria Latvia Estonia Czechia Slovakia Malta Denmark Croatia Lithuania N Ireland Slovenia Wales Poland England Norway Scotland Sweden Netherlands Finland Iceland Ireland Austria Portugal EUROPE - Germany 2007) Spain Switzerland France Belgium Italy five year one year Liver: topography • C22.1 = intrahepatic bile ducts • C22.0 = liver, NOS Liver: morphology • Hepatocellular carcinoma=HCC (8170; C22.0) • Intrahepatic cholangiocarcinoma=ICC (8160; C22.1) • Mixed HCC/ICC (8180; TNM: C22.1; ICD-O: C22.0) • Hepatoblastoma (8970; C22.0) • Malignant rhabdoid tumour (8963; (C22.0) • Sarcoma (C22.0) • Angiosarcoma (9120) • Epithelioid haemangioendothelioma (9133) • Embryonal sarcoma (8991)/rhabdomyosarcoma (8900-8920) Morphology*: distribution by sex (NL 2011-17) other other ICC 2% 3% 28% ICC 56% HCC 41% HCC 70% males females * Only pathologically confirmed cases Liver cancer: primary or metastatic? Be aware that other and unspecified morphologies are likely to be metastatic, unless there is evidence of the contrary. For example, primary neuro-endocrine tumours (including small cell carcinoma) of the liver are extremely rare. So, when you have a diagnosis of a carcinoid or small cell carcinoma in the liver, this is probably a metastatic tumour. Anatomy of the bile ducts Gallbladder
[Show full text]
A New Theranostic Paradigm for Advanced Thyroid Cancer
INVITED PERSPECTIVES A New Theranostic Paradigm for Advanced Thyroid Cancer David A. Pattison1,2, Benjamin Solomon3,4, and Rodney J. Hicks1,4 1Centre for Cancer Imaging, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia; 2Endocrinology Service, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia; 3Department of Medical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia; and 4Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, Victoria, Australia Differentiation status and metabolic reprogramming are increas- The successful use of 131I as a systemic treatment of a patient ingly being recognized as determinants of imaging phenotype. In with metastatic thyroid cancer was first reported in 1948 (1). This contrast to well-differentiated tumors, which are reliant on mitochon- agent has subsequently become firmly established as part of the treat- drial oxidative phosphorylation to generate the energy needed for ment of high-risk thyroid cancer and especially for metastatic dis- cellular processes, poorly differentiated cancer cells depend on the ease. There has been an evolution in the quality of assessment of the inefficient mechanism of aerobic glycolysis—the Warburg effect. distribution of radioactive iodine (RAI) within the body, progressing Notably, Hurthle cell (or oncocytic) tumors, both benign and malig- 18 from Geiger–Muller¨ counting to imaging, first with the rectilinear nant, are generally characterized by intense F-FDG avidity represent- scanning and then with the g-camera. The physical characteristics ing inherent constitutive activation of glycolytic pathways (6). Loss of 123I as a diagnostic tracer compared with 131I further improved of the mitochondrial respiratory chain complex I has been shown to imaging by facilitating higher quality SPECT and SPECT/CT.
[Show full text]
Multiple Endocrine Neoplasia Type 1 (MEN1)
Lab Management Guidelines v2.0.2019 Multiple Endocrine Neoplasia Type 1 (MEN1) MOL.TS.285.A v2.0.2019 Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline MEN1 Known Familial Mutation Analysis 81403 MEN1 Deletion/Duplication Analysis 81404 MEN1 Full Gene Sequencing 81405 What is Multiple Endocrine Neoplasia Type 1 Definition Multiple Endocrine Neoplasia Type 1 (MEN1) is an inherited form of tumor predisposition characterized by multiple tumors of the endocrine system. Incidence or Prevalence MEN1 has a prevalence of 1/10,000 to 1/100,000 individuals.1 Symptoms The presenting symptom in 90% of individuals with MEN1 is primary hyperparathyroidism (PHPT). Parathyroid tumors cause overproduction of parathyroid hormone which leads to hypercalcemia. The average age of onset is 20-25 years. Parathyroid carcinomas are rare in individuals with MEN1.2,3,4 Pituitary tumors are seen in 30-40% of individuals and are the first clinical manifestation in 10% of familial cases and 25% of simplex cases. Tumors are typically solitary and there is no increased prevalence of pituitary carcinoma in individuals with MEN1.2,5 © eviCore healthcare. All Rights Reserved. 1 of 9 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines v2.0.2019 Prolactinomas are the most commonly seen pituitary subtype and account for 60% of pituitary adenomas.
[Show full text]
Multiple Endocrine Neoplasia Type 1: a Case Report and Review of the Literature
Cent. Eur. J. Med. • 9(3) • 2014 • 424-430 DOI: 10.2478/s11536-013-0297-8 Central European Journal of Medicine Multiple endocrine neoplasia type 1: a case report and review of the literature Case Report Audrius Šileikis1,2, Edvinas Kildušis*1,2, Ramūnas Janavičius3, Kęstutis Strupas1,2 1 Vilnius University, Faculty of Medicine, 03101, Vilnius, Lithuania 2 Vilnius University Hospital Santariskiu Klinikos, Center of Abdominal Surgery, 08661, Vilnius, Lithuania 3 Vilnius University Hospital Santariskiu Klinikos, Hematology, Oncology and Transfusion Medicine Center,, 08661, Vilnius, Lithuania Received 31 March 2013; Accepted 16 July 2013 Abstract: Multiple endocrine neoplasia syndrome, type 1 (MEN1) is an underdiagnosed autosomal dominant inherited cancer predisposition syndrome with inter- and intrafamilial variability without a known genotype-phenotype correlation. Disease is caused by mutations in the MEN1 gene located on chromosome 11, but other genes (CDKN1B, AIP) and mechanisms might be involved too. We performed retrospective case series study of MEN1 syndrome patient and his family and present our experience of management of genetically confirmed 9 MEN1 syndrome large family members from Lithuania with novel MEN1 gene mutation (MEN1 exon 6 – c. 879delT (p.Pro293Profs*76)) and delineate its clinical phenotype. At present the diagnosis of MEN1 syndrome must be established by direct mutation testing. MEN1 syndrome patients, their relatives and patients suspected of MEN1 are eligible for mutation testing. Patients with MEN1 have a shorter life expectancy than the general population. MEN1 patients and mutation carriers should be subjected to periodic screening in order to detect manifestations in an early stage. Early genetic diagnosis and subsequent periodic screening is associated with less morbidity and mortality at follow-up.
[Show full text]
Rising Incidence of Neuroendocrine Tumors
Rising Incidence of Neuroendocrine Tumors Dasari V, Yao J, et al. JAMA Oncology 2017 S L I D E 1 Overview Pancreatic Neuroendocrine Tumors • Tumors which arise from endocrine cells of the pancreas • 5.6 cases per million – 3% of pancreatic tumors • Median age at diagnosis 60 years • More indolent course compared to adenocarcinoma – 10-year overall survival 40% • Usually sporadic but can be associated with hereditary syndromes – Core genetic pathways altered in sporadic cases • DNA damage repair (MUTYH) Chromatin remodeling (MEN1) • Telomere maintenance (MEN1, DAXX, ATRX) mTOR signaling – Hereditary: 17% of patients with germline mutation Li X, Wang C, et al. Cancer Med 2018 Scarpa A, Grimond S, et al. NatureS L I2017 D E 2 Pathology Classification European American Joint World Health Organization Neuroendocrine Committee on Cancer (WHO) Tumor Society (AJCC) (ENETS) Grade Ki-67 Mitotic rt TNM TNM T1: limit to pancreas, <2 cm T1: limit to pancreas, ≦2 cm T2: limit to pancreas, 2-4 cm T2: >limit to pancreas, 2 cm T3: limit to pancreas, >4 cm, T3: beyond pancreas, no celiac or Low ≤2% <2 invades duodenum, bile duct SMA T4: beyond pancreas, invasion involvement adjacent organs or vessels T4: involves celiac or SMA N0: node negative No: node negative Intermed 3-20% 2-20 N1: node positive N1: node positive M0: no metastases M0: no metastases High >20% >20 M1: metastases M1: metastases S L I D E 3 Classification Based on Functionality • Nonfunctioning tumors – No clinical symptoms (can still produce hormone) – Accounts for 40% of tumors – 60-85%
[Show full text]
New Jersey State Cancer Registry List of Reportable Diseases and Conditions Effective Date March 10, 2011; Revised March 2019
New Jersey State Cancer Registry List of reportable diseases and conditions Effective date March 10, 2011; Revised March 2019 General Rules for Reportability (a) If a diagnosis includes any of the following words, every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report the case to the Department in accordance with the provisions of N.J.A.C. 8:57A. Cancer; Carcinoma; Adenocarcinoma; Carcinoid tumor; Leukemia; Lymphoma; Malignant; and/or Sarcoma (b) Every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report any case having a diagnosis listed at (g) below and which contains any of the following terms in the final diagnosis to the Department in accordance with the provisions of N.J.A.C. 8:57A. Apparent(ly); Appears; Compatible/Compatible with; Consistent with; Favors; Malignant appearing; Most likely; Presumed; Probable; Suspect(ed); Suspicious (for); and/or Typical (of) (c) Basal cell carcinomas and squamous cell carcinomas of the skin are NOT reportable, except when they are diagnosed in the labia, clitoris, vulva, prepuce, penis or scrotum. (d) Carcinoma in situ of the cervix and/or cervical squamous intraepithelial neoplasia III (CIN III) are NOT reportable. (e) Insofar as soft tissue tumors can arise in almost any body site, the primary site of the soft tissue tumor shall also be examined for any questionable neoplasm. NJSCR REPORTABILITY LIST – 2019 1 (f) If any uncertainty regarding the reporting of a particular case exists, the health care facility, physician, dentist, other health care provider or independent clinical laboratory shall contact the Department for guidance at (609) 633‐0500 or view information on the following website http://www.nj.gov/health/ces/njscr.shtml.
[Show full text]
Possible Functional Regression of Insulinoma with Prolonged Octreotide C E H Craig, I W Gallen
623 Postgrad Med J: first published as 10.1136/pmj.78.924.623 on 1 October 2002. Downloaded from CASE REPORT Possible functional regression of insulinoma with prolonged octreotide C E H Craig, I W Gallen ............................................................................................................................. Postgrad Med J 2002;78:623–624 A 75 year old woman was treated for over three years with the somatostatin analogue, octreotide for an insulinoma. She had presented in a hypoglycaemic coma. C-peptide and insulin concentrations were both raised and an area of increased vascularity within the pancreas was shown by angiography. No lesion was found at laparotomy and no resection was performed. After over three years of octreotide treatment it was withdrawn for a week. Her insulin and C-peptide concentrations were greatly reduced at this time and remained so. he somatostatin analogue, octreotide inhibits secretion of a wide variety of peptide hormones including insulin.1 It Thas been successfully used in previous cases of insuli- noma. Other well differentiated endocrine tumours have Figure 1 Coeliac angiogram; area of increased vascularity is reduced in size when treated with drugs that inhibit hormone indicated by arrows. production—for example, bromocriptine in prolactinoma.2 Here we report a case of markedly reduced insulin production from an insulinoma, once octreotide treatment was with- concentration excluded hypothyroidism. Her admission insu- drawn. lin and C-peptide levels were later reported as 438.7 pmol/l (21.5–115.0) and 2810 pmol/l (180–630) respectively. A urinary sulphonylurea screen was negative. As her C-peptide CASE REPORT levels were raised, the presence of exogenous insulin was also http://pmj.bmj.com/ A 75 year old woman presented, unconscious, after a collapse excluded.
[Show full text]