Medical Professionals

DonateNow

Carcinoid E-newsletter signupVisit Our Carcinoid Blog

Search


Medical Professionals



General Information About Carcinoid and
Related Neuroendocrine Tumors

This section contains links to websites with full-text or abstract versions of published papers that focus on general information (reviews) of diagnosis, treatment and current research in the field of carcinoid and neuroendocrine tumors.

Reviews, Research, Peer-Review Articles, Book Resources, and Miscellaneous Information

  • (2/7/10) Targeted Radionuclide Therapy for Neuroendocrine Tumours: Principles and Application

    Maralyn R. Drucea Val Lewingtonb Ashley B. Grossmanb
    Neuroendocrinology. 2010;91(1):1-15. Epub 2009 Jul 7.

    Abstract
    Neuroendocrine tumours comprise a group of neoplasms with variable clinical behaviour. Their growth and spread is often very slow and initially asymptomatic, and thus they are often metastatic at the time of diagnosis and incurable by surgery. An exciting therapeutic strategy for cytoreduction, both for stabilisation of tumour growth and inhibition of hormone production, is the use of targeted radionuclide therapy. Evidence from large-scale, randomised, placebo controlled trials is very difficult to obtain in these rare diseases, but current data appear promising. It is timely to review the principles underlying the use of these therapies, together with the clinical outcomes to date and potential directions for future research.

    PMID: 19590162
  • (2/7/10) Molecular target therapy for gastroenteropancreatic endocrine tumours: Biological rationale and clinical perspectives

    Gabriele Capursoa,1, Nicola Faziob,1, Stefano Festaa, Francesco Panzutoa, Filippo De Braudb, Gianfranco Delle Favea,*

    aDigestive and Liver Disease Unit, S. Andrea Hospital, II Medical School, University “La Sapienza”, Via Di Grottarossa 1035-1039, 00189, Rome, Italy
    bUnit of Clinical Pharmacology and New Drugs, European Institute of Oncology, Milan, Italy

    Critical Reviews in Oncology/Hematology 72 (2009) 110-124

    Abstract
    Gastroenteropancreatic endocrine tumours (GEP ETs) represent a relatively rare and heterogeneous group of neoplasms whose therapy can be challenging. The poorly differentiated, fast-growing cases are treated with chemotherapy. In the slow-growing ones, biotherapy is usually performed. Several categories of targeted therapies have been studied for their treatment in vitro and in vivo. A critical review of molecular alterations suggests a rationale for targeting angiogenesis, and the phosphatidylinositol 3 kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR) pathway. Accordingly, antiangiogenic agents and mTOR inhibitors are presently the most tested agents in phase II and III studies. Bevacizumab, some multitarget inhibitors, and mTOR inhibitors showed promising results in patients with advanced GEP ETs. A limited activity has been reported for imatinib and epidermal growth factor receptor (EGFR) inhibitors. Combinations of molecular targeted therapies with different sites of action, and somatostatin analogues may be relevant to avoid molecular escape pathways. Future trials should include more homogeneous groups of patients and pay more attention to the subgroup with progressive disease.

    PMID: 19249226
  • (2/7/10) The following information was presented at the ASCO Gastrointestinal Cancers Symposium
    held in Orlando, Florida from January 22-24, 2010

    Updated results of the phase III trial of sunitinib (SU) versus placebo (PBO) for treatment of advanced pancreatic neuroendocrine tumors (NET).

    2010 Gastrointestinal Cancers Symposium Abstract 127

    Author(s):

    E. Raymond, P. Niccoli-Sire, Y. Bang, I. Borbath, C. Lombard-Bohas, J. W. Valle, S. Patyna, D. Lu, R. C. Chao, J. Raoul; Beaujon University Hospital, Clichy, France; Service d'Oncologie Medicale, CHU La Timone, Marseille, France; Seoul National University Hospital, Seoul, South Korea; Cliniques Universitaires Saint-Luc, Brussels, Belgium; Hopital Edouard Herriot, Lyon, France; The Christie NHS Foundation Trust, Manchester, United Kingdom; Pfizer Oncology, San Diego, CA; Pfizer Oncology, La Jolla, CA; Centre Eugene Marquis, Rennes, France

    Background: The multitargeted tyrosine kinase receptor inhibitor sunitinib has shown activity against pancreatic NET in the RIP1-Tag2 mouse model and in phase I/II studies.

    Methods:     This phase III, multinational, randomized, double-blind trial (NCT00428597) investigated the efficacy and safety of SU vs PBO in patients (pts) with advanced pancreatic NET. Pts with well-differentiated pancreatic islet cell tumors and documented disease progression within the past 12 months were randomized (1:1) to SU 37.5 mg/day daily or PBO. All pts received best supportive care and somatostatin analogs, if indicated. The primary endpoint was progression-free survival (PFS) with target enrolment of 340 pts.

    Results:     This study was stopped early due to differences in efficacy, as recommended by the independent Data Monitoring Committee. Between Jun 2007 and Apr 2009, 171 pts were randomized to SU (n = 86) or PBO (n = 85); median age was 56 years, 52% were female, 50% had nonfunctional tumors, 95% had metastatic disease, and 71% had received various prior treatments. The most frequently reported adverse events (AEs) with SU were (SU vs PBO, all grades) fatigue/asthenia (60% vs 52%), diarrhea (59% vs 39%), nausea (45% vs 29%) and vomiting (34% vs 31%). Grade 3/4 AEs (SU vs PBO) included neutropenia (12% vs 0%), hypertension (9.6% vs 1.2%), fatigue/asthenia (8.4% vs 12.2%), palmar-plantar erythrodysesthesia (6.0% vs 0%) and abdominal pain (4.8% vs 9.8%). More serious AEs were reported in the PBO arm (26.5% in SU vs 41.5% in PBO). Final analysis of 81 events for PFS included 75 events of disease progression. Median PFS was 11.4 months in the SU arm vs 5.5 months in the PBO arm (p = 0.0001). The objective response rate (ORR) with SU was 9.3% (2 complete and 6 partial responses; 95% CI: 3.2-15.4%). There were 9 and 21 deaths in the SU and PBO arms, respectively.

    Conclusions:     Sunitinib prolonged PFS, increased ORR and OS, and displayed an acceptable safety profile in pts with advanced pancreatic NET.

    • SU (n = 86)

    PBO (n = 85)

    Hazard ratio (95% CI)

    p

    Median PFS, months

    11.4

    5.5

    0.418 (0.263, 0.662)

    0.0001

    ORR, n (%)

    8 (9.3%)

    0

    -

    0.0066

    Median OS

    NR

    NR

    0.404 (0.185, 0.882)

    0.0186

    Abbreviations: SU, sunitinib; PBO, placebo; CI, confidence interval; PFS, progression-free survival; ORR, objective response rate; OS, overall survival; NR, not reached.

    (please note:  sunitinib is not yet FDA-approved for neuroendocrine tumors)


  • (1/22/10) Sunitinib paves the way for targeted therapies in neuroendocrine tumors.

    Raymond E, Faivre S, Hammel P, Ruszniewski P.
    Department of Medical Oncology and Gastroenterology,
    Beaujon University Hospital, Clichy, France,
    eric.raymond@bjn.aphp.fr

    Targeted Oncology (2009) 4:253-254

    ABSTRACT: Sunitinib demonstrating efficacy in pancreatic islet cell carcinomas will pave the way for further trials in other neuroendocrine tumor types such as carcinoid, poorly differentiated neuroendocrine disease, and several other endocrine tumors that are dependent on VEGF/VEGFR for angiogenesis. In addition, other drugs with distinct mechanisms of action, such as mTOR inhibitors, currently investigated in phase III trials, may also supply novel options in those diseases to control tumor growth and metastasis.

    PMID: 19911111 [PubMed – as supplied by publisher]
  • (1/22/10) ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors:Echocardiography

    Ursula Plöckingera Björn Gustafssonb Diana Ivanc Waldemar Szpakd
    Joseph Davare and all other Mallorca Consensus Conference participants

    a Department of Hepatology and Gastroenterology, Campus Virchow-Klinikum, Charité-Universitätsmedizin Berlin, Berlin, Germany;
    b     Medisinsk avd Gastroseksjon, St Olavs Hospital HF, Trondheim, Norway;  
    c     Endocrinology and Diabetology, Klinikum der Philipps-Universität, Marburg, Germany;
    d     Westville Hospital, Amanzimototi, Mayville, South Africa;   
    e Department of Cardiology, Royal Free Hospital, London, UK

    Neuroendocrinology 2009; 90:190-193

    INTRODUCTION: Carcinoid heart disease is observed in 3–4% of all patients with a neuroendocrine tumor and in 40–50% of those with a carcinoid syndrome [1, 2]. Details on well differentiated neuroendocrine jejunal-ileal tumors have already been discussed in the ENETS Consensus Guidelines and the reader is referred to these Guidelines [3]. Here technical questions and quality management for the diagnosis and follow-up of carcinoid heart disease will be discussed. Involvement of the tricuspid leaflets grade 2–3 occurs in 90%, a stenosis of the pulmonary leaflets in 50%, while regurgitation is seen in 81% of the patients during the course of the disease [4, 5] . Carcinoid heart disease is a relatively late manifestation of neuroendocrine tumors; however, it has an important impact on the prognosis of these patients. Thus, early diagnosis and treatment is mandatory in each patient with a carcinoid syndrome. Echocardiography is the gold standard for detection of carcinoid heart disease. This article will concentrate on technical details for echocardiography. The information provided should help those not experienced with this disease to diagnose carcinoid heart disease and provide high-quality information of echocardiographic investigations. The information provided by echocardiography will be the basis for clinical decisions and may well influence the prognosis and outcome of the patient.

    PMID: 19713710 [PubMed – as supplied by publisher]
  • (1/22/10) Chromogranin-A and N-Terminal Pro-Brain Natriuretic Peptide: An Excellent Pair of Biomarkers for Diagnostics in Patients with Neuroendocrine Tumor

    Catharina M. Korse, Babs G. Taal, Cornelis A. de Groot, Robert H. Bakker, and Johannes M.G. Bonfrer

    Journal Clinical Oncology 27:4293-4299

    CONCLUSION: N-terminal pro-brain natriuretic peptide (NT-proBNP) and chromogranin-A (CgA) are very important markers in the diagnosis of CHD in patients with NET. Furthermore, patients with elevated NT-proBNP in addition to elevated CgA levels showed worse overall survival than patients with elevated CgA alone.

    PMID: 19667278 [Pubmed – as supplied by publisher]
  • (12/30/09) Treatment of metastatic carcinoid tumors with radiolabeled biologic molecules

    Bushnell D.
    University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA. david-bushnell@uiowa.edu

    Journal of the National Comprehensive Cancer Network. 2009 Jul;7(7):760-4.

    ABSTRACT: Treatment options in advanced-stage neuroendocrine tumors are limited. A promising new category of therapy was recently introduced for these tumors in which radioactive atoms are attached to molecules that target and bind to neuroendocrine cancer cells. 90Yttrium-DOTA-Phe1-Tyr3-octreotide and radioactive drugs which targets cells by binding to somatostatin receptors. 131Iodine-metaiodobenzylguanidine also targets neuroendocrine tumors using the amine transporter system. Both agents, along with the somatostatin analogue 177Lutetium-DOTATATE, have shown objective response rates in approximately 30% of patients with progressive metastatic disease. Symptomatic improvement is observed in most patients receiving these drugs and evidence of survival benefit is also mounting. Serious side effects are uncommon.

    PMID: 19635228 [PubMed - indexed for MEDLINE]

  • NEW (11/4/09) Long-term results of patients with malignant carcinoid syndrome receiving octreotide LAR.

    Toumpanakis C, Garland J, Marelli L, Srirajaskanthan R, Soh J, Davies P, Buscombe J, Caplin ME.
    Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK.

    Aliment Pharmacology & Therapeutics Journal. 2009 Oct;30(7):733-40. Epub 2009 Jul 2.

    BACKGROUND: Octreotide LAR is an established treatment for malignant carcinoid syndrome. However, studies with large number of patients and long follow-up are lacking. AIM: To present long-terms results with octreotide LAR, assessing duration of clinical and objective response and treatment tolerance, in a large, homogeneous cohort of patients with malignant carcinoid syndrome.
    METHODS: A total of 108 patients with metastatic midgut neuroendocrine tumours were included in this 8-year study. Clinical evaluation was based on a symptom score. Radiological assessment was based on RECIST (Response Evaluation Criteria In Solid Tumours) criteria.
    RESULTS: Of the 108 patients, 24% had a sustained symptomatic response. In the remaining patients, loss of symptomatic response with the initial dose was noted within 3-60 months. In 17% of them, symptoms were controlled by just an increase of octreotide LAR dose, whilst the other patients required additional treatment. Overall, in 45.3% of patients, symptoms were well controlled during the study period with only octreotide LAR, and no additional treatment was required. No significant adverse effects were noted.
    CONCLUSIONS: Octreotide LAR treatment provides a sustained symptomatic response in about half of the patients with malignant carcinoid syndrome and contributes to disease stabilization for a longer period than previously described. PMID: 19573169 [PubMed - in process]

    http://www3.interscience.wiley.com/journal/122484106/abstract
  • NEW (11/2/09) Biochemical testing for neuroendocrine tumors

    Vinik AI, Silva MP, Woltering G, Go VL, Warner R, Caplin M.
    Eastern Virginia Medical School, Strelitz Diabetes Research Center, Norfolk, VA 23510, USA. vinikai@evms.edu

    Pancreas. 2009 Nov;38(8):876-89.

    ABSTRACT: In this review, we focus on the use of biochemical markers for the diagnosis of neuroendocrine tumors and exclusion of conditions that masquerade as neuroendocrine tumors. In addition, we outline the use of biochemical markers for follow-up, response to intervention, and determination of prognosis. Previous publications have focused only on markers specific to certain tumor types, but the uniqueness of this chapter is that it presents a new approach ranging from biochemical markers that relate to symptoms to the use of markers that facilitate decision making with regard to optimizing the choices of therapy from the complex arrays of intervention, The sequence of presentation in this chapter is first to provide the usual view, that is, biochemical markers of each tumor type and thereafter the diagnosis of the underlying condition or exclusion thereof and finally the algorithm for their use from the clinical presentation to the suspected diagnosis and the biochemical markers to monitor progression and therapeutic choice. There is also a specific description of the properties of the most important biochemical markers and 2 complications, bone metastasis and carcinoid heart disease, from the biochemical point of view.
    PMID: 19855234 [PubMed - in process]

    http://journals.lww.com/pancreasjournal/pages/currenttoc.aspx

  • NEW (11/1/09) Placebo-Controlled, Double-Blind, Prospective, Randomized Study on the Effect of Octreotide LAR in the Control of Tumor Growth in Patients With Metastatic Neuroendocrine Midgut Tumors: A Report From the PROMID Study Group

    Anja Rinke, Hans-Helge Müller, Carmen Schade-Brittinger, Klaus-Jochen Klose, Peter Barth, Matthias Wied, Christina Mayer, Behnaz Aminossadati, Ulrich-Frank Pape, Michael Bläker, Jan Harder, Christian Arnold, Thomas Gress, and Rudolf Arnold
    Journal of Clinical Oncology. 2009 Oct 1;27(28):4656-63.

    Conclusion: Octreotide LAR significantly lengthens time to tumor progression compared with placebo in patients with functionally active and inactive metastatic midgut NETs.
  • NEW   Dr. Demeure's Power Point Presentation (8.5MB PDF) from the AAES meeting in
    Madison, WI, May 2009
    Comprehensive description of neuroendocrine tumors; Symptoms, Diagnosis and treatment.
    Presented here with permission from Dr. Demeure.
    Michael J. Demeure, MD, MBA
    Senior Investigator
    Endocrine & Cancer Surgery
    Scottsdale, AZ
    Tel:480-323-1570
  • NEW Original Article
     Prolonged survival after hepatic artery embolization in patients with midgut carcinoid syndrome.
    by Swärd C, Johanson V, Nieveen van Dijkum E, Jansson S, Nilsson O, Wängberg B, Ahlman H, Kölby L on May 2009 in British Journal of Surgery

    Conclusion:
    Hepatic Arery Embolization is safe, provides good control of hormonal symptoms, and prolongs survival in biochemically responsive patients. It is a valuable palliative option for patients with midgut carcinoid syndrome due to liver metastases and can be repeated in patients with a favourable response to the first procedure. Copyright (c) 2009 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.PMID: 19358175 [PubMed - as supplied by publisher].........read full text

  • NCI Carcinoid/NET Summit 2007; Main Conclusions

    Priorities for Improving the Management of
    Gastroenteropancreatic Neuroendocrine Tumors
    Irvin M . Modlin , Steven F . Moss , Daniel C . Chung , Robert T . Jensen , Elizabeth Snyderwine
    Published by: J Natl Cancer Inst 2008;100: 1282 – 1289 Vol. 100, Issue 18 | September 17, 2008

    ABSTRACT:A National Cancer Institute summit meeting on gastroenteropancreatic neuroendocrine and carcinoid tumors was held in September 2007 to present the currently accepted standards of care for patients with these tumors and to identify areas requiring investigation and development. These tumors are clinically and pathologically heterogeneous, present commonly with obscure symptoms that lead to delays in diagnosis of years, and have an incidence in the United States of 2.5 to 5 cases per 100 000. The 5-year survival rates range between 15% and 95%, depending on the site and extent of disease. This report delineates the main conclusions of the meeting, including the best practice diagnosis and treatment strategies for gastropancreatic neuroendocrine tumors, and the identification of clinical and scientific areas that are most in need of attention. The most pressing needs were public and physician education,
    identification of molecular markers for early diagnosis and therapeutic monitoring, improved imaging modalities and molecular prognostication, development of a standardized pathological classification system, and creation of regional centers of expertise with tumor and laboratory data banks. In addition, adequately validated neuroendocrine tumor models and cell lines should be established to investigate the molecular mechanisms involved in the control of their growth and secretion, and to facilitate the development of specific therapies that should be examined in well-designed multicenter studies of defined patient groups.
  • Clinical Value of monitoring Plasma Octreotide Levels During Chronic OctreotideLong-Acting Repeateble Threapy in Carcinoid Patients

    Woltering EA, Salvo VA, O'Dorisio TM, Lyons J 3rd, Li G, Zhou Y, Seward JR, Go VL, Vinik AI, Mamikunian P, Mamikunian G.
    Department of Surgery, Louisiana State University Health Sciences Center, New Orleans, LA, USA. ewolte@lsuhsc.edu
    Pancreas. 2008 Jul;37(1):94-100.
    Abstract

    CONCLUSIONS: Current plasma octreotide values are significantly lower than previously reported for 30-, 60-, and 120-mg/mo LAR doses. Serial plasma octreotide value measurements should be used to determine if increasing symptoms or tumor growth are associated with suboptimal drug dosing.
  • Improving Worldwide Cancer Care and Prevention Awareness
    Oncology by OncologyStat
     Elsevier’s Oncologystat.com recently upgraded its website.
    OncologySTAT's mission is to improve worldwide cancer care and prevention by providing healthcare professionals with immediate integrated access to the most authoritative evidence-based information available. OncologySTAT’s commitment to international health ensures that news, research, education, and analysis from all regions of the world are covered in a publisher, society, and sponsor-neutral online environment.

    TIP: for example, type"carcinoid tumors all sites" (or other cancers) in the advanced search window and you will receive a comprehensive list of up-to-date peer-review articles of carcinoid tumors from over 100 Elsevier cancer-related journals, including The Lancet Oncology, The Breast, Lung Cancer, The American Journal of Medicine, Cancer Letters, etc. Many articles are full text.

  • Population-based study of islet cell carcinoma
    Online Full Text
    Yao JC, Eisner MP, Leary C, Dagohoy C, Phan A, Rashid A, Hassan M, Evans DB. Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA. jyao@mdanderson.org
    Ann Surg Oncol. 2007 Dec;14(12):3492-500. Epub 2007 Sep 26.

    CONCLUSION: Patients with malignant pancreatic neuroendocrine tumors commonly present with advanced disease. Although, curative resection is not frequent, survival benefit may be obtainable with aggressive surgical management even in the face of metastatic disease.

    Note: Acknowledgments Supported by gifts from Raymond Sackler, the J. Stuart Foundation, and the Carcinoid Cancer Foundation.

  • Chemoembolization and Bland Embolization of
    Neuroendocrine Tumor Metastases to the Liver     (Full text)
    J Vasc Interv Radiol. 2007 Jul;18(7):847-55
    Ruutiainen AT, Soulen MC, Tuite CM, Clark TW, Mondschein JI, Stavropoulos SW, Trerotola SO.
    Division of Interventional Radiology, University of Pennsylvania, 1 Silverstein, Philadelphia, PA 19104, USA.
    (Abstract)
    CONCLUSIONS: Chemoembolization was not associated with a higher degree of toxicity than bland embolization. Chemoembolization demonstrated trends toward improvement in TTP, symptom control, and survival. Based on these results, a multicenter prospective randomized trial is warrante
  • Valproic Acid activates Notch-1 Signaling and Regulates the Neuroendocrine Phenotype in Carcinoid Cancer Cells
    Oncologist.     2007 Aug;12(8):942-51
    Greenblatt DY, Vaccaro AM, Jaskula-Sztul R, Ning L, Haymart M, Kunnimalaiyaan M, Chen H

    F.A.C.S., H4/750 Clinical Science Center, 600 Highland Avenue, Madison, Wisconsin 53792, USA.

    Carcinoid tumors are neuroendocrine malignancies that frequently metastasize and secrete hormones that cause debilitating symptoms in patients. In this study we report the effects of valproic acid (VPA), a drug long used for the treatment of epilepsy, on the growth and neuroendocrine phenotype of human carcinoid cancer cells. VPA treatment of gastrointestinal and pulmonary carcinoid cells resulted in a dose-dependent inhibition of cancer cell growth. Western blot analysis revealed degradation of cyclin D1 and an increase in cyclin-dependent kinases p21 and p27 with VPA treatment. Flow cytometry confirmed that the mechanism of VPA-induced growth inhibition is G(1) phase cell cycle arrest. Furthermore, VPA suppressed expression of the neuroendocrine tumor marker chromogranin A. In addition to these effects, VPA also increased levels of full-length Notch-1 and the active Notch-1 intracellular domain. Luciferase reporter assays incorporating the centromere-binding factor 1 (CBF-1) binding site and the achaete-scute complex-like 1 (ASCL-1) promoter confirmed the functional activity of VPA-induced Notch-1. Transfection of Notch-1 small-interfering RNA into carcinoid tumor cells blocked the effects of VPA on Notch-1 activation, ASCL-1 suppression, p21 induction, and cell growth inhibition. VPA also suppressed growth of carcinoid tumors in vivo in a mouse tumor xenograft experiment. These findings confirm the important role of Notch-1 in regulating the growth and neuroendocrine phenotype of carcinoid tumor cells. On the basis of this study, a clinical trial of VPA for patients with advanced carcinoid cancer will be conducted.

  • Tumor Suppressor Role of Notch-1 Signaling in Neuroendocrine Tumors
    Muthusamy Kunnimalayaan, Herbert Chen
    Endocrine Surgery Research Laboratories, Department of Surgery, The University of Wisconsin, and the University of Wisconsin Comprehensive Cancer Center, Madison, Wisconsin, USA
    The Oncologist 2007;12:535–542

    ABSTRACT
    A growing body of literature is demonstrating that Notch signaling is a more complex process than originally thought. Contradictory findings of notch-1 acting as an oncogene or a tumor suppressor revealed that its role is very specific to the cellular context. In this review we focus on the tumor suppressor role of Notch-1 signaling in neuroendocrine tumors (NETs) such as carcinoid and medullary thyroid cancers. NETs secrete various bioactive hormones that can cause debilitating symptoms. Surgery is the only potential curative treatment for the patients with NETs. Notch-1 signaling is absent in these tumors and activation of Notch-1 significantly
    reduces tumor growth in vitro. Therefore, identification
    of compound(s) that activate the Notch-1 pathway in NETs could be a potential strategy to treat patients with NETs.
  • ENETS 2007 Consensus Guidelines for the Management pf Patients with Digestive Neuroendocrine tumors. Part1 Stomach, Duodenum and Pancreas
  • Liver metastases of neuroendocrine tumors; early reduction of tumour load lead to improved life expectancy
    .(for full text)
    Liesbeth M Veenendaal1, Inne HM Borel Rinkes1, Cornelis JM Lips2 and Richard van Hillegersberg, Netherlands.
    Published: 26 June 2006 World Journal of Surgical Oncology 2006, 4:35
    Review

    Abstract : Background: Neuroendocrine tumours frequently metastasize to the liver. Although generally slowly progressing, hepatic metastases are the major cause of carcinoid syndrome and ultimately lead to liver dysfunction, cardiac insufficiency and finally death.
    Conclusion: Treatment for patients with neuroendocrine hepatic metastases must be tailored for each individual patient. When local ablative therapies are used early in the course of the disease, the occurrence of carcinoid syndrome with end stage hepatic disease can be postponed or prevented.
  • Goblet cell carcinoid of the appendix
    Payam S Pahlavan1  and Rani Kanthan2 
    1Department of Physiology and Pathophysiology, University of Heidelberg, Heidelberg, Germany
    2Department of Pathology, University of Saskatchewan, Saskatoon, Canada
    World Journal of Surgical Oncology 2005, 3:36
  • Over expression of the Notch1 intracellular domain (NICD) inhibits cellular proliferation and alters the neuroendocrine phenotype of medullary thyroid cancer cells
    Muthusamy Kunnimalaiyaan, Abram M. Vaccaro, Mary A. Ndiaye, and Herbert Chen
    Surgery, University of Wisconsin, Madison, WI 53792
    Papers In Press, published online ahead of print November 7, 2006
    J. Biol. Chem, 10.1074/jbc.M603578200
    Accepted on November 7, 2006
    Full Text

    Other papers published by Muthusamy Kunnimalaiyaan in Pub Med
  • Human Tumor Assay
    Editor Larry Weisenthal, MD, Phd

    What is Cell culture drug resistance testing (CCDRT)?
    Also known as "chemotherapy sensitivity and resistance assays" or ("CSRAs")
    This journal describes the use of CCDR and its use to identify the best forms of chemotherapy for cancer patients on an individual basis.
  • Information on Carcinoid and Related Neuroendocrine Tumors (HTML page)
    This website, created by Endotext.org. provides comprehensive, authoritative, and updated full text information on endocrine disease directed to physicians around the world caring for patients with these problems. All material may be freely downloaded for personal use and is a textbook format.

    Topics covered on these webpages included:
    • Introduction-Development and Anatomy, by Aaron Vinik, M.D. , Ph.D.
    • Carcinoid Tumors, by Aaron Vinik M.D.
    • Gastrinoma Syndrome, by Roger R Perry, M.D. , FACS
    • Insulinomas, by Roger R Perry M.D.
    • MEN I and MEN I, by Roger R Perry M.D.
    • VIPomas, by Aaron Vinik M.D.
    • Glucagonoma, by Aaron Vinik M.D.
    • Somatostatinoma, by Aaron Vinik M.D.
    • Pancreatic Polypeptide-oma, by Aaron Vinik M.D.
    • Neurotensinoma, by Aaron Vinik M.D.
    • Ghrelinoma, by Aaron Vinik M.D.
    • Management of Neuroendocrine Tumors of the GI Tract, by Aaron Vinik M.D.
    • Chemotherapy for Islet Cell Carcinomas, by Aaron Vinik M.D.
  • Gastroenteropancreatic Neuroendocrine Tumor Disease: Molecular and Cell Biological Aspects
    This link is to an abstract. Full text of this volume is available to subscribers of the Annals of the New York Academy of Sciences or by per-article payment. Nonmembers may browse the table of content and abstracts by following the link above.
    Volume 1014 published Apr 2004
    Edited by Bertram Wiedenmann; Gerhard M. Christofori; Michael Hoecker
  • Diagnosis and Management of Neuroendocrine Tumors
    by Rudolph Arnold, M.D., FRCP
    8th United European Gastroenterology Week
    Reference provided by Medscape
  • Considerations Concerning a Tailored, Individualized Therapeutic Management of Patients with (neuro)Endocrine Tumors of the Gastrointestinal Tract and Pancreas (Full text PDF)
    W W de Herder, E P Krenning, C H J van Eijck 2 and S W J Lamberts
    Endocrine-Related Cancer (2004) 11 19–34
  • Carcinoid Tumors: Molecular genetics, tumor biology, and update of diagnosis and treatment
    This link is to an abstract. Full text of this volume is available to subscribers of the Current Opinion on Oncology or by per-article payment.
    Article by Oberg Kjell M.D.
    Department of Medical Sciences, Uppsala University Hospital,
    Uppsala, Sweden.
    Current Opinion Oncology 2002 Jan;14(1):38-45

    "Chromogranin A is an important general tumor marker for all types of carcinoid tumors. Somatostatin receptor scintigraphy is a cornerstone in staging and localization of carcinoid tumors, but newer techniques such as positron emission tomography will challenge its position in the future. Although surgical cure is not obtainable, a more aggressive surgery has emerged during the last decade. Debulking and other cytoreductive procedures are quite common today. Somatostatin analogues have been the treatment of choice in symptomatic patients with carcinoid tumors, but more recent studies have indicated a cytostatic effect of somatostatin analogues. Tumor-targeted radioactive treatment based on somatostatin analogues is now under clinical evaluation. Preliminary data indicate interesting clinical potentials"
  • Gastrointestinal Carcinoid Tumors (PDQ®): Treatment for Health Professionals (HTML page)
    From the National Cancer Institute

Incidence/Statistics

  • A 5-Decade Analysis of 13,715 Carcinoid Tumors (Full text PDF)
    By Irving M. Modlin, M.D., Kevin D. Lys, M.D., Mark Kidd, Ph.D.
    Cancer 2003 Feb 15;97(4):934-59

    CONCLUSIONS: Carcinoids appear to have increased in overall incidence over the past 30 years; for some sites, this trend has been evident for nearly half a century. Recent marked increases in gastric and rectal carcinoids and a concomitant decrease in appendiceal carcinoid incidence may be due in part to varying rules of registration among the compiled databases examined in this report or to improvements in diagnostic technology; increased awareness of and about carcinoid tumors also may play a significant role. In 12.9% of all patients with carcinoid, distant metastases already were evident at the time of diagnosis; the overall 5-year survival rate for all carcinoid tumors, regardless of site, was 67.2%. These findings bring into question the widely promulgated relative benignity of carcinoid disease. Certain carcinoid tumors, such as those of the rectum, appear to be over-represented among the black and Asian populations within the United States, suggesting the role of genetics in the development of this intriguing disease.
  • Updated Population-Based Review of Carcinoid Tumors (Full text PDF)
    By Maggard MA, O'Connell JB, Ko CY.
    Ann Surg. 2004 Jul;240(1):117-22

Carcinoid Heart Disease

  • Early and Late Results of Valvular Surgery for Carcinoid Heart Disease
    Javier G. Castillo, Farzan Filsoufi, Parwis B. Rahmanian, Anelechi Anyanwu, Jerome S. Zacks, Richard R.P. Warner, David H. Adam
    Journal of the American College of Cardiology
    Volume 51, Issue 15, 15 April 2008, Pages 1507-1509
    Pages 1507-1509
    Full Text
  • Carcinoid heart disease: The role of urinary 5-hydroxyindoleacetic acid excretion and plasma levels of atrial natriuretic peptide, transforming growth factor-beta and fibroblast growth factor (Full text PDF)
    Zuetenhorst JM, Bonfrer JM, Korse CM, Bakker R, Van Tinteren H, Taal BG.
    Cancer 2003;97:1609-1615
  • Carcinoid heart disease: presentation, diagnosis, and management
    Fox and Khattar
    Heart, 90 (10), p. 1224
  • Carcinoid Heart Disease
    By Zuetenhorst JM, Taal BG.
    N Engl J Med. 2003 Jun 5;348(23):2359-61

    CONCLUSIONS: High levels of 5-HIAA excretion and ANP were found to be associated with CHD.
    " ...there was an even stronger relationship between carcinoid heart disease and the serotonin load over time."
    " Even in the absence of severe symptoms of the carcinoid syndrome, it is highly recommended that serotonin levels be reduced." "Which can be realized by combination of different treatment modalities such as octreotide analogues,......" and other treatment modalities.
  • Carcinoid heart disease : presentation, diagnoaias, and managemente
    This link is to an abstract. Full text is available at www.heartjnl.com to subscribers of Heart or by per-article payment
    Fox DJ, Khattar RS.
    Heart. 2004 Oct;90(10):1224-8

    Review:
    Tricuspid and pulmonary valve regurgitation usually occurs as a secondary phenomenon caused by dilatation of the valve ring secondary to right ventricular failure or pulmonary hypertension, respectively. Primary diseases of the tricuspid or pulmonary valves are uncommon, but the more likely causes might include congenital abnormalities, rheumatic heart disease, or infective endocarditis. Carcinoid heart disease is a rare, but interesting and important cause of intrinsic tricuspid and pulmonary valve disease leading to significant morbidity and mortality caused by right heart failure. When treated medically, and in appropriate cases surgically, significant benefits in overall quality of life and long term survival can be achieved. We review the current literature regarding the pathophysiological basis of the disease, the cardiovascular complications, and the currently available treatment strategie.

Clinical trials

  • University of California, San Francisco (UCSF) Comprehensive Cancer Center - Clinical Trial
    Chemotherapy drugs like 5-fluorouracil, leucovorin, and oxaliplatin (FOLFOX) work in different ways to cause tumor cells to stop growing or die. Bevacizumab is a monoclonal antibody that may inhibit cancer growth by blocking blood flow to tumors. Adding bevacizumab to combination chemotherapy may be a better way to block tumor growth than giving either type of therapy alone. The FOLFOX plus bevacizumab combination is being studied in patients with neuroendocrine tumors because FOLFOX appears to inhibit the growth of a variety of different tumor types but has not yet been tested in this disease. In addition, neuroendocrine tumors appear to depend on blood vessels for growth suggesting that they may respond to a treatment like bevacizumab. This clinical trial is for patients who have not responded to other treatments to see if the FOLFOX/bevacizumab combination is safe and if it can inhibit the growth of metastatic neuroendocrine tumors.

    LOCATION AND CONTACT INFORMATION
    Mary O’Rourke, R.N.
    Clinical Trials Recruitment Nurse
    UCSF Comprehensive Cancer Center
    Clinical Research Support Services
    1600 Divisadero Street
    San Francisco, CA 94115
    Tel: (415) 353-9612
    Fax: (415) 353-9738
    Email:    morourke@cc.ucsf.edu
  • Results from Clinical Trials of Systemic treatment with Y90, LU-177 and 111-In
    Overview of Results of Peptide Receptor Radionuclide Therapy with 3 Radiolabeled Somatostatin Analogs (Full Text)
    Kwekkeboom DJ, Mueller-Brand J, Paganelli G, Anthony LB, Pauwels S, Kvols LK, O'dorisio TM, Valkema R, Bodei L, Chinol M, Maecke HR, Krenning EP.
    J Nucl Med. 2005 Jan;46 Suppl 1:62S-6S
    .
    Conclusion:The results obtained with [(90)Y-DOTA(0),Tyr(3)]octreotide and [(177)Lu-DOTA(0),Tyr(3)]octreotate are very encouraging in terms of tumor regression. Also, if kidney protective agents are used, the side effects of this therapy are few and mild, and the duration of the therapy response for both radiopharmaceuticals is more than 2 y. These data compare favorably with those for the limited number of alternative treatment approaches.

Other Topics of Interest

Pediatric Carcinoid

Bronchial/Lung Carcinoid

  • NEW  Bronchopulmonary Carcinoid: Phenotype and Long-term Outcome in
    a Single-Institution Series of Italian Patients (Full Text PDF)
    Massimo Rugge, Matteo Fassan, Roberto Clemente4, Giovanna Rizzardi,
    Luciano Giacomelli, Gianmaria Pennelli, Claudia Mescoli1, Daniela Segat, and Federico Rea
    Clinical Cancer Research 14, 149-154, January 1,
    2008. doi: 10.1158/1078-0432.CCR-07-1631
  • Bronchial Carcinoid
    By Dan Granberg M.D. : Bronchial Carcinoids.
    Uppsala, 2001. - 75p.
    Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine,
    ISSN 0282-7476 ; 1006
    ISBN 91-554-4957-3
  • Additional references regarding treatment of patients with bronchial carcinoids
    Provided by
    Dan Granberg, MD, PhD, Consultant, Assistant Head of Department
    Department of Endocrine Oncology
    University Hospital
    SE-751 85 Uppsala
    Sweden

    Lung and t    hymic neuroendocrine tumors.
    Granberg D, Skogseid B.
     In: Doherty G, Skogseid B, eds. Surgical Endocrinology. Philadelphia. Lippincott Williams & Wilkins. 2001:413-430

    Granberg D, Eriksson B, Wilander E, Grimfjärd P, Fjällskog M-L, Öberg K, Skogseid B. Experience in treatment of metastatic pulmonary carcinoid tumors. Ann Oncol 2001;12(10):1383-1391
    2008-04-01 kl. 14.18 skrev Monica Warner:

    Temozolomide as monotherapy is effective in treatment of advanced malignant neuroendocrine tumors.
    Ekeblad S, Eriksson B, Sundin A, Welin S, Granberg D, Kindmark H, Dunder K, Kozlovacki G, Örlefors H, Sigurd M, Öberg K, Janson ET, Skogseid B
    Clin Cancer Res 2007;13(10):2986-2991

    Outcome of patients with pulmonary carcinoid tumors receiving chemotherapy or chemoradiotherapy. Lung Cancer 2004;44:213-20.

    Peptide receptor radionuclide therapy with (177)Lu-octreotate in patients with foregut carcinoid tumours of bronchial, gastric and thymic origin
    Wirth LJ, Carter MR, Jänne PA, Johnson BE.
    .van Essen, M et al. Eur J Nucl Med Mol Imaging 2007;34(8):1219-1227

    Response of atypical pulmonary carcinid tumors to chemotherapy. Aretrospective study of 37 patients.
    Guigay J.
    Poster presented at the12th World Conference on Lung Cancer, Seoul, Korea, September 2007.

    Lung and Thymic Neuroendocrine Tumors.
    Granberg D, Öberg K.
    In: Hay I, Turner H, Wass J, eds. Clinical Endocrine Oncology, 2nd edition. Oxford. Blackwell Publishing Ltd. In press, to be published on April 15th.
  • A list of proceedings from the UK-NET/ENET meeting in London, May 2003
    This page also includes links to full text versions of the presentations.

Neuroendocrine Societies

  • NANETS (North American NeuroEndocrine Tumor Society)
    ABOUT NANETS :
    NANETS was established in September 2006. The purpose of this professional organization is to improve neuroendocrine tumor (NET) disease management through increased research and educational opportunities
    Read more.......
  • ENETS (European Neuroendocrine Tumor Network Society
    The European Neuroendocrine Tumor Society is an international professional association composed of physicians and researchers, whose main area of study is related to neuroendocrine tumors. Society members, currently numbering around 200, bring a variety of expertise to ENETS, such as oncology, pathology, endocrinology, surgery and gastroenterology. ENETS is supported via membership dues, as well as through educational grants from the pharmaceutical community. It is administratively-based in Berlin, Germany.
    European Neuroendocrine Tumor Network (ENETS)
    Budapest, Hungary, March 25 – 27, 2004.(PHOTOS)

Genetics

 

Last Modified: Monday, 08-Feb-2010 03:59:02 EST

Website copyright and information
Disclaimer