NEUROENDOCRIN/CARCINOID CANCER

Carcinoid A rare form of "slow-growing" neuroendocrine cancer

Susan Anderson - An advocate for Carcinoid and Neuroendocrine Tumor Awareness

Many of these are NEW links although same topic!
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HOW TO DIAGNOSE AND MONITOR CARCINOID (NEUROENDOCRINE TUMORS)

 WHICH TESTS AND HOW OFTEN?

Permission granted from: Dr. Lowell Anthony, Dr. Richard R.P. Warner and Dr. Eugene  A. Woltering.

Review the information that follows, print what you need, AND do additional research on each of the tests, if you desire.

PLEASE do not copy any of this information to post on another web page without getting specific permission from the doctors listed above.

Please remember each patient's Carcinoid is different.  We are all distinct individuals and our cancer must be treated on an individual basis.  There are many factors that influence how to diagnosis and monitor Carcinoid, a few are: where our Carcinoid tumor(s) is located, our health before diagnosis, other health problems we may be experiencing; and age. Thus how our bodies and minds react to Carcinoid vary a great deal.   Due to the differences in each case a protocol for how to monitor Carcinoid needs to be “tailor-made” for each patient – there is NO one protocol for diagnostic and then surveillance that fits all persons.

How to monitor Carcinoid is not as simple as just testing for blood sugar levels if you suspect Diabetes and to follow with blood sugar levels to make sure it is controlled.  Only a physician knowledgeable and experienced with neuroendocrine tumors/Carcinoid can make the decision about what to test for initially, how to follow up, and then how to interpret changes once treatment(s) are started.

I have prepared a summary from information provided by Drs. Anthony, Warner and Woltering.  After my summary there is additional information from each of these doctors based on their own experience.  These are some of the few physicians in the USA with decades of interest and experience with neuroendocrine tumors.

Compiled by Susan L. Anderson   http://www.carcinoidinfo.info

Please scroll down for new Guidelines as of 7 Feb. 2011

SUMMARY - In General

INITIAL WORK-UP – dependent on the type and location of tumor(s): every patient does NOT need all of these tests.  Only a physician knowledgeable and experienced with Carcinoid can determine what is right for each patient.

• At the time of initial diagnosis, biomarkers should be measured dependent upon site of the primary tumor:
  
  There should be tumor stains for Chromogranin-A and Ki-67 (also known as MIB-1).
   

• Pancreatic primary:  Chromogranin-A, Pancreatinc Polypeptide, Substance P, and specific markers depending on clinical signs and symptoms 5-HIAA, Serotonin,       Gastrin, Glucagon, Insulin, VIP, Somatostatin.
   

• Lung primary:  Chromogranin-A, Neuron Specific Enolase (NSE), 5-HIAA, Serotonin, Substance P, ACTH
   

• Gut primary:  Chromogranin-A, Substance P, 5-HIAA, Serotonin, Urinary Methyl-Histamine (gastric primaries only)
   

• Rectal primary: alpha and beta subunits of HCG.

Unknown primary:  urine 5-HIAA, Chromogranin-A, Serotonin, Neuron Specific Enolase (NSE), Substance P, Neurokinin A and alpha and beta subunit of HCG (human chorionic gonadotropins).

More from each individual doctor further down in this article.

FOLLOW-UP every 3 to 4 months or 6 months, each patient is different:

Review of clinical symptoms: flushing, diarrhea, fatigue, weight loss and others.

What markers to test for depends on each individual patient, which markers were elevated initially, and where the Carcinoid is/was located.

More from each individual doctor further down in this article.

IMAGING:  Every 6 to 12 months, each patient is different:

• CT with contrast or MRI with contrast.
   
• OctreoScan initially (to have as a baseline) and then later ONLY if it influences therapy.
   
• Echocardiogram: (to have as a baseline) and then annually for early detection of  Carcinoid heart disease.  (Only if serotonin and/or urine 5-HIAA increased.).

Imaging:  http://www.medicalassistantdegree.com/resources/medical-imaging-resources/

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From:  Dr. Eugene Woltering, July 30, 2002

• Initial tumor-stain for chromogranin-A and Ki-67.
   
• Initial work up---CT of area - Octreoscan - blood for chromogranin A -sub P - 5HIAA (i.e.) a 24 hours urine-consider if weird symptoms-and I do mean weird----consider sending off a tube of blood to the Ohio State University Core   Peptide Lab at 614-293-8423 ask for Mr. Brent  Howe he will give your doctor the instructions on how to get blood collected spun and frozen/ sent to the OSU.  Ask them to do a "peptide profile".
   
• Follow up --- I recommend the chromogranin-A (sub -P if initially elevated)  every three months and the 5HIAA urine every six months and I really recommend that you graph the results to look at the trend.  The trend is much more important than the specific value.
   
• I recommend the Octreoscan and the CT scan once a year but I space them six month apart so you do some form of scan every six months
   
• What I don't recommend - Serotonin in blood or urine unless you know your lab and you know that reference lab - also I don't recommend PET scans unless you are going to see Dr. Oberg's group in Sweden who do very special testing with radioactive compounds not generally available.

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From:  Dr. Richard R. P. Warner, August 26 & September 7, 2002

1. Chemical markers are important to measure and follow since they change before imaging tests do and before physical conditions (symptoms) do.  They are useful early indicators of the tumor status.

2. The tempo of the disease varies from one patient to the next and hence the frequency the tests should be done may vary from yearly to every 3 months.  The average is twice a year.

3. Chromogranin A (CgA) is the most stable and dependable marker in 90% of cases. In many cases it can be supplemented by other markers which should have been tested originally and those found abnormal can also be followed subsequently.

    In carcinoid we initially include: Urine 5HIAA, Blood serotonin, Neuron Specific Enolase, Pancreastatin, Substance P, Pancreatic Polypeptide and Atrial Naturetic hormone (ANH) (fasting).  The latter (ANH) helps indicate development of carcinoid heart disease in patients with functioning tumors.

     In other neuroendocrine tumors, depending on type we measure Gastrin, VIP, Calcitonin, CEA, Insulin, Glucagon, Alpha/Beta subunits of HCG and ACTH.

     In all cases we check blood CgA which is most often positive in most tumors regardless of presence or absence of any specific endocrine function.  When ascites is present we measure ascitic fluid CgA also.

4.  Appropriate imaging tests such as CT-scan with contrast, MRI with contrast and OctreoScan are also included in monitoring with frequency customized for each case.

5.  Included in the initial work up is also tumor-stain for Chromogranin A and Ki-67 (MIB-1) and in aggressive tumor we also measure for P-53 and C-kit..

6.  Echocardiogram initially and yearly thereafter if ANH(Atrial Naturetic Hormone also sometimes known as ANF=Atrial Naturetic Factor) is increased above normal.

Richard R.P.Warner M.D.
Medical Director
   The Carcinoid Cancer Foundation, Inc.
 Clinical Professor of Medicine
   The Mount Sinai School of Medicine, New York City

The Carcinoid Cancer Foundation, Inc.
333 Mamaroneck Avenue #492
White Plains, NY 10605
Tel: 888-722-3132   Tuesday - Thursday (for general questions only)
Fax: 914-683-5919
Office Tel: 212-722-2100

URL: http://www.carcinoid.org  Carcinoid Cancer Foundation check for tests: in the FAQs section (frequently asked questions):

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Since attending the first Carcinoid Seminar in Sarasota, FL, in March 1999, I have included the following lists from Dr. Anthony’s talk at that Seminar  on my web page.  He has now expanded this information, see below:

How to Monitor Carcinoid - from Dr. Lowell Anthony's lectures at the first Carcinoid Seminar in Sarasota, FL, March 1999,  and other carcinoid "experts"

1.  Clinical symptoms:  flushing, diarrhea, tiredness, weight loss, other; every 3 - 4 months

2.  Biochemical markers:  24-hour 5-HIAA; plasma chromogranin A; substance P; others, every 3 - 4 months

3.  Imaging:  CT/MRI; every 6-12 months

4.  OctreoScan:  baseline and with clinical / radiographic changes ONLY IF it influences therapy.

5.  Echocardiogram:  baseline and annually:  Early detect carcinoid heart disease.

 From: Dr. Lowell Anthony, August 29, 2002 & September 3, 2002

Expanded #2, August 2002

• At the time of initial diagnosis, biomarkers measured dependent upon site of primary as follows:
   
• Pancreatic primary:  nonspecific markers (chromogranin A, pancreatic polypeptide,
  substance P) and specific markers depending on clinical signs and symptoms (5-HIAA /serotonin, gastrin, glucagon, insulin, VIP, somatostatin).
   
• Lung primary:  chromogranin A, neuron specific enolase (NSE), 5-HIAA / serotonin, substance P, ACTH.
   
• Gut primary:  chromogranin A, substance P, 5-HIAA / serotonin, substance P, urinary methyl-histamine (gastric primaries only).
   
• Rectal primaries:  usually have NO elevated markers.
   
• Unknown primary:  chromogranin A, 5-HIAA / serotonin, NSE, substance P.
   
• For longitudinal follow-up:  5-HIAA or CGA every 3-4 months or, if WNL at presentation, any other elevated biomarker.
   
• Substance P is a more "labile" neuropeptide meaning that it is more likely to be affected by conditions of sampling (temperature, processing time, etc.).  In my own experience, I rarely use this marker to serially follow someone's disease since it is more likely to give confusing information (going down when the CGA or 5-HIAA is rising or vice versa) rather than the meaningful data we need.  The general principle I follow is to identify the best marker at the time of diagnosis and stick to it, as best as possible, over time.  If substance P is the only elevated marker, then I would consider following it.  Rarely, is it an isolated elevated marker.

Dr. Lowell Anthony, M.D.  (Fall 2011)
Dr. Anthony recently became a Professor of Medicine at the University of Kentucky, Lexington, 
after many years serving as Director of Gastrointestinal and Neuroendocrine Oncology at LSU
Health Sciences Center in New Orleans, Louisiana.

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From web page of Dr. Kjell Oberg in Sweden

In this section you can see the different tests and examinations that we use to evaluate each patients disease. All patients do not have to do all tests and for each individual patient an individual work-up schedule is designed.
 

DIAGNOSTIC PROCEDURE FOR:

• MIDGUT CARCINOID TUMORS
   
• Ordinary blood parameters including haemathology tests, liver and kidney function tests and electrolytes
   
• U-5HIAA - mean of two 24-hour urine collections during dietary restrictions
   
• p-chromogranin A
   
• p-neuropeptide K
   
• Histopathological examination of surgically removed tumor specimen or from a 1.2 mm needle biopsy from a metastasis stained with Grimelius and Masson sliver stainings and  immunohistochemical investigation for chromogranin A and serotonin.
   
• Tumor biology program including Ki67 (cellproliferation), celladhesion CD44, angiogenesis with bFGF and VEGF and somatostatin receptor subtype expression.
   
• Abdominal ultrasonography
   
• Magnetic Resonance Tomography
   
• Abdominal computerized tomography
   
• Somatostatin receptor scintigraphy (OctreoScan)
   
• Positron Emission Tomography (PET) with specific tracers including 11C-5-HTP
   
• Chest X-ray

 ENDOCRINE PANCREATIC TUMOR

• Ordinary blood parameters including hemathology tests, liver and kidney function tests and electrolytes.
   
• Specific hormones including gastrin, insulin, proinsulin, calcitonin, VIP, glucagon, ACTH, cortisol, PP.
   
• p-chromogranin A
   
• hCG-alpha and-beta
   

• Histopathological examination on surgically removed tumor specimen or from a 1.2 mm needle biopsy from a metastasis stained with Grimelius and Masson sliver
  stainings and immunohistochemical investigation for   chromogranin A and specific hormones.
   

• Tumor biology program including Ki67 (cellproliferation), celladhesion CD44, angiogenesis with bFGF and VEGF and somatostatin receptor subtype expression.
   
• Abdominal ultrasonography
   
• Magnetic Resonance Tomography
   
• Abdominal computerized tomography
   
• Somatostatin receptor scintigraphy (OctreoScan)
   
• Positron Emission Tomography (PET) with specific tracers including 11C-5-HTP
   
• Endoscopic ultrasonography
   
• Chest X-ray
  
   MULTIPLE ENDOCRINE NEOPLASIA TYPE 1
   
• Ordinary blood parameters including hemathology tests, liver and kidney function tests and electrolytes.
   
• S-Ca levels, urinary calcium and PTH
   
• Meal stimulation test with measurements of PP and gastrin
   
• Specific hormones including gastrin, insulin, proinsulin, calcitonin, VIP, glucagon, ACTH, cortisol, PP, prolactin, GH.
   
• p-chromogranin A
   
• hCG-alpha and-beta
   

• Histopathological examination on surgically removed tumor specimen or from a 1.2 mm needle biopsy from a metastasis stained with Grimelius and Masson sliver stainings and immunohistochemical investigation for chromogranin A and specific hormones.
   

• Tumor biology program including Ki67 (cellproliferation), celladhesion CD44, angiogenesis with bFGF and VEGF and somatostatin receptor subtype expression.
   

• Abdominal ultrasonography
   
• Magnetic Resonance Tomography
   
• Abdominal computerized tomography
   
• Somatostatin receptor scintigraphy (OctreoScan)
   
• Positron Emission Tomography (PET) with specific tracers including 11C-5-HTP
   
• Endoscopic ultrasonography
• Chest X-ray
   

Professor Kjell Öberg
Dean of The Medical Faculty,
Uppsala University.
751 85 Uppsala
Sweden
Telefon/Phone: +46 18 611 49 17
Fax:+46 18 510133
Akademiska sjukhuset Phone : +46 18 53 36 98
 E-mail: Kjell.Oberg@medicin.uu.se

President of ENET

ENET (The European Neuroendocrine Network) - A European project between different centers working with these tumors with the aim to set up guidelines  for tumor biology program,   radiological work-up, and randomized trials of both new and established therapies.

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OF SPECIAL INTEREST FOR THE PHYSICIAN:
 

Information for your medical doctor and other medical professionals from CCF

Medical Reviews

General Information About Carcinoid and Related Neuroendocrine Tumors -- WITH A BIG THANK YOU TO THE Carcinoid Cancer Foundation (CCF).  General Information About Carcinoid and Related Neuroendocrine Tumors.  This section contains links to websites and full-text and 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.

Thank you to the Carcinoid Cancer Foundation, Inc. (CCF), for giving me permission to link directly to this important consensus report below.

Consensus Statements and Guidelines

 "This consensus report gives a detailed description of the use of somatostatin analogs in the management of neuroendocrine tumors of the gastroenteropancreatic system. As background information we have outlined critical aspects of the pathology, the use of tumor markers, a definition of functional and non-functional digestive neuroendocrine tumors, different imaging modalities, surgical considerations, liver embolization and the use of cytotoxic drugs as well as interferon. Included in the report is an overview of somatostatin, somatostatin analogs and its receptor expression in different neuroendocrine tumors. It will also define the binding affinities of different somatostatin analogs to the five different subtypes of somatostatin receptor. We compare the efficacy of octreotide and lanreotide in reducing diarrhea and flushing. Side-effects are described and we provide practical information on somatostatin analog treatment."

 NATIONAL COMPREHENSIVE CANCER NETWORK  http://www.nccn.org

The National Comprehensive Cancer Network (NCCN) is a not for profit, tax-exempt corporation that is an alliance of the world's leading cancer centers.  The NCCN Practice Guidelines in Oncology presents statement of consensus from some physicians dealing with neuroendocrine tumors regarding their view of currently accepted approaches to treatment and surveillance.

Under the heading "Guidelines for Treatment of Cancer by Site" these guidelines include a section on Neuroendocrine Tumors (Version 1.2001) (including Carcinoid ), which contain algorithms of Initial work up, Clinical Presentation , Treatment and Surveillance.

• 
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  Since 1996 --- when I first located others with Carcinoid --- I have answered every email sent to me, although some replies were delayed longer than I liked. I am NO longer able to reply to all e-mails due to the volume and other things going on in my life (all good).  I do love hearing from others, but an unable to reply to all individually. 
  This web site was 20 years old on April 27, 2017.

  Since my husband "retired" I am away from my computer for days, and sometimes, weeks at a time.  Yes, there is wireless internet and we have tried that a number of places.  But, if I deal with email when we are away from home then it is not a "restful vacation" for me.

  PLEASE use the SEARCH capability at the top my pages.  You may search for a word, a phrase, a drug, a treatment, a book title or anything you can think of. You may search this site only, or search the complete World Wide Web.

  To speak with a person please know you may call the “telephone information and support line” of the Carcinoid Cancer Foundation, Inc. (CCF), Tuesday through Thursday  10 a.m. to 4 p.m. Eastern Time at  1-888-722-3132 (free) or 1-914-693-1001.   “The information and support line” is staffed by medical professionals.   Mondays and Fridays are research days, if you call then and do not reach a person do leave a clear message.  The Carcinoid Cancer Foundation’s (CCF) superb web site is at http://www.carcinoid.org

  + + + + + + + + + +

  CCF -  Serving the carcinoid/NETs  patient and medical community for 
  more than forty years (chartered in 1968)
     “Meets Extensive Standards of America’s Most Experienced Charity Evaluator”
  Better Business Bureau

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  NEW - 7 February 2011 - NEW

  NET Guidelines from NANETS (North American NeuroEndocrine Tumor Society)

   http://nanets.net/research/articles/nanets-guidelines

   Carcinoid Cancer Foundation (CCF)

  The culmination of years of dedicated efforts by a host of worldwide experts, the NANETS (North American NeuroEndocrine Tumor Society) consensus guidelines present an up-to-date synthesis of the best available knowledge on the classification, diagnosis, and treatment of NETs. Please share these guidelines with any physician or healthcare professional who might benefit from this important resource.

  

  NANETS - NANETS Guidelines

  nanets.net

   

  • Video Presentations
  • New ICD-9-CM codes for NETs

  

  NANETS Guidelines

  ·        The North American Neuroendocrine Tumor Society (NANETS) Guidelines: Mission, Goals, and Process.
  Authors: Kvols, Larry K. MD *; Brendtro, Kari L. +
  DOI: DOI: 10.1097/MPA.0b013e3181eb7451
   

  ·        The Pathologic Classification of Neuroendocrine Tumors: A Review of Nomenclature, Grading, and Staging Systems.
  Authors: Klimstra, David S. MD *; Modlin, Irvin R. MD, PhD +; Coppola, Domenico MD ++; Lloyd, Ricardo V. MD, PhD [S]; Suster, Saul MD [//]
  DOI: DOI: 10.1097/MPA.0b013e3181ec124e
   

  ·        NANETS Consensus Guidelines for the Diagnosis of Neuroendocrine Tumor.
  Authors: Vinik, Aaron I. MD, PhD *; Woltering, Eugene A. MD +; Warner, Richard R. P. MD ++; Caplin, Martyn MD [S]; O'Dorisio, Thomas M. MD [//]; Wiseman, Gregory A. MD [P]; Coppola, Domenico MD #; Go, Vay Liang W. MD **
  DOI: DOI: 10.1097/MPA.0b013e3181ebaffd
   

  ·        NANETS Treatment Guidelines: Well-Differentiated Neuroendocrine Tumors of the Stomach and Pancreas.
  Authors: Kulke, Matthew H. MD *; Anthony, Lowell B. MD +; Bushnell, David L. MD ++; de Herder, Wouter W. MD, PhD [S]; Goldsmith, Stanley J. MD [//]; Klimstra, David S. MD [P]; Marx, Stephen J. MD #; Pasieka, Janice L. MD **; Pommier, Rodney F. MD ++; Yao, James C. MD ++++; Jensen, Robert T. MD [S][S]
  DOI: DOI: 10.1097/MPA.0b013e3181ebb168
   

  ·        The NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors: Well-Differentiated Neuroendocrine Tumors of the Jejunum, Ileum, Appendix, and Cecum.
  Authors: Boudreaux, J. Philip MD *; Klimstra, David S. MD +; Hassan, Manal M. MD, PhD ++; Woltering, Eugene A. MD *; Jensen, Robert T. MD [S]; Goldsmith, Stanley J. MD [//]; Nutting, Charles DO [P]; Bushnell, David L. MD #; Caplin, Martyn E. MD **; Yao, James C. MD ++
  DOI: DOI: 10.1097/MPA.0b013e3181ebb2a5
   

  ·        The NANETS Consensus Guidelines for the Diagnosis and Management of Gastrointestinal Neuroendocrine Tumors (NETs): Well-Differentiated NETs of the Distal Colon and Rectum.
  Authors: Anthony, Lowell B. MD *; Strosberg, Jonathan R. MD +; Klimstra, David S. MD ++; Maples, William J. MD [S]; O'Dorisio, Thomas M. MD [//]; Warner, Richard R.P. MD [P]; Wiseman, Gregory A. MD #; Benson, Al B. III MD **; Pommier, Rodney F. MD ++
  DOI: DOI: 10.1097/MPA.0b013e3181ec1261
   

  ·        The North American Neuroendocrine Tumor Society Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors: Pheochromocytoma, Paraganglioma, and Medullary Thyroid Cancer.
  Authors: Chen, Herbert MD *; Sippel, Rebecca S. MD *; O'Dorisio, M. Sue MD, PhD +; Vinik, Aaron I. MD, PhD ++; Lloyd, Ricardo V. MD, PhD [S]; Pacak, Karel MD, PhD, DSc [//]
  DOI: DOI: 10.1097/MPA.0b013e3181ebb4f0
   

  ·        NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors: Well-Differentiated Neuroendocrine Tumors of the Thorax (Includes Lung and Thymus).
  Authors: Phan, Alexandria T. MD *; Oberg, Kjell MD, PhD +; Choi, Junsung MD ++; Harrison, Lynn H. Jr MD [S]; Hassan, Manal M. MD, PhD [//]; Strosberg, Jonathan R. MD [P]; Krenning, Eric P. MD, PhD #; Kocha, Walter MD **; Woltering, Eugene A. MD ++; Maples, William J. MD ++++
  DOI: DOI: 10.1097/MPA.0b013e3181ec1380
  
  The NANETS Consensus Guidelines for the Diagnosis and Management of Poorly Differentiated (High-Grade) Extrapulmonary Neuroendocrine Carcinomas.
  Authors: Strosberg, Jonathan R. MD *; Coppola, Domenico MD +; Klimstra, David S. MD ++; Phan, Alexandria T. MD [S]; Kulke, Matthew H. MD [//]; Wiseman, Gregory A. MD [P]; Kvols, Larry K. MD *
  DOI: DOI: 10.1097/MPA.0b013e3181ebb56f

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• DISCLAIMER:  I am a patient and NOT a medical doctor or health care professional.  I share information and links to information that has been helpful to me and that I believe to be correct and good, but I cannot guarantee the accuracy of this information, except for MY stories.  I urge you not to rely only on this information but I believe you should discuss your situation and information with your medical doctors and/or other medical professionals.  "Sunny" Susan Anderson
   

• Copyright © 1997-2017 Susan L. Anderson. All Rights Reserved.

Susan L. Anderson, Mesa, AZ, USA
Email:  SunnySusan@Cox.Net
 

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