Living with an advanced neuroendocrine tumor

CELEBRATING
YEA R S
Committed to improving
knowledge and care in NETs
For me, my health comes first.
Everything else comes second.
That was a decision I had to make…
Living with an advanced
neuroendocrine tumor
Take control of your disease
Brought to you by
Living with an advanced neuroendocrine tumor (NET)
Introduction
This booklet was written for you. If you are reading this, you either have been diagnosed with an advanced
NET or you are caring for a person diagnosed with an advanced NET.
An emotional journey
Being diagnosed with a NET means you have a type of cancer that is not as well known as other types of
cancer. When you first hear a diagnosis like this, it’s normal to feel overwhelmed, confused, afraid, and even
angry. Your family and friends may feel the same way.
However, it’s important to remember that detailed disease information, experienced healthcare professionals,
treatment options, and personal support are all available. It’s up to you to take advantage of them.
You try to get yourself in an
emotional place that won’t wear
you down. There’s no handbook
for this kind of journey.
2
You can take control of your disease
Just because you’ve been diagnosed with a NET doesn’t mean you can’t continue to live the life that you’ve
known. There is much you can do to manage your disease.
A good place to start is by realizing that you are not alone. Many people who have NETs continue to lead
active, fulfilling lives.
It is true that advanced NETs present challenges, both for you and for your healthcare team. But by learning
about your disease and talking with doctors and other patients, you can be better prepared to manage the
challenges you may face.
This booklet describes what it means to have an advanced NET. Divided into 3 parts, the booklet will
help you
• Learn about your disease
• Understand your care plan and other steps that can help you live with your disease
• Find support from other people who are affected by NETs
This booklet also includes a glossary. It explains many of the medical terms that you may read about or hear
when speaking to your doctor. Some of these terms are indicated like this within this booklet. There is also a
resource section with websites you can visit to obtain additional information, including connections to support
groups around the world.
Use Living With an Advanced NET as a starting point. Learn as much as you can, ask questions, and work
with your doctors and other healthcare professionals to ensure the best plan of care for your needs.
3
4
Table of contents
Understanding your disease............................................................................................... 6
The neuroendocrine system..................................................................................................................... 7
NETs—A type of neuroendocrine cancer................................................................................................. 8
Different names for NETs......................................................................................................................... 8
Types of NETs.......................................................................................................................................... 8
Classification of NETs ........................................................................................................................... 10
Advanced NETs and metastases ........................................................................................................... 12
How does a tumor metastasize?............................................................................................................ 12
Managing your disease..................................................................................................... 14
Management starts with an accurate diagnosis..................................................................................... 14
Tests your doctor may order.................................................................................................................. 15
Deciding on the right care plan.............................................................................................................. 17
Possible courses of action..................................................................................................................... 18
Why multidisciplinary care is important................................................................................................. 20
Living with your disease.................................................................................................... 22
Make time for yourself .......................................................................................................................... 22
Building your network of support........................................................................................................... 25
Communicating with caregivers and loved ones.................................................................................... 26
Practical issues..................................................................................................................................... 27
Resources to help you (websites)..................................................................................... 30
Frequently asked questions.............................................................................................. 34
Glossary of terms.............................................................................................................. 36
5
Understanding your disease
When people are diagnosed with a NET, it is usually months or years since they first started experiencing
symptoms. Now they can finally focus on their disease. However, they may feel overwhelmed by all the
symptoms, tests, treatments, and statistics. Sometimes people with NETs feel alone because they have a
less common type of cancer.
You may have had a discussion with your doctor about your disease when you were first diagnosed. During
this conversation, details may have seemed clear, but by now you may have forgotten them or thought of new
questions. This booklet is designed to help you learn more about your disease so you can be informed and
take control.
Become your own health advocate
Being diagnosed with a NET is a turning point. It’s a chance to take control of the next chapter in your life —
by becoming your own health advocate, learning more about your disease, and making a plan to live the way
you want. The first step? Understanding what a NET is and what type of NET you have.
Diagnosis was a shock,
but at the same time I was
relieved. Now I had proof
that my complaints were
not just imaginary.
6
The neuroendocrine system
The neuroendocrine system is made up of 2 systems — the nervous system and the endocrine system —
that work together to control various body functions. Neuroendocrine cells act as messengers, releasing
hormones that allow the brain to communicate with certain parts of your body. For example, when your
body needs to digest food, neuroendocrine cells release a hormone called serotonin. Serotonin activates the
muscles in the digestive tract. Other important hormones you may have heard about are insulin, adrenaline,
estrogen, and testosterone.
Parathyroid
glands
Hypothalamus
Major glands of the endocrine system
Pituitary
gland
Neuroendocrine cells are located throughout the body,
including in glands, such as the adrenal glands, pancreas,
thyroid, pituitary, ovaries, and testes (see figure). These
glands help control important body functions, such as
growth, metabolism, reproduction, and response to stress
or injury.
Thyroid
gland
Adrenal
glands
Pancreas
Ovaries
(female)
Reprinted from The Merck Manual Online Medical Library.
Testes
(male)
Normal cell
division
Abnormal cell division turns to cancer
Normally, endocrine cells — like other cells that
make up the tissue and organs in your body —
grow, divide, die, and are replaced by new cells in
a continuous cycle. If this process breaks down,
some cells can grow uncontrollably and may form
cancerous tumors (masses of tissue) or cancer
(see figure).
Cell suicide
or apoptosis
Cell damage–
no repair
Cancer cell
division
Source: National Cancer Institute. Understanding Cancer Series: Cancer.
www.cancer.gov.
First
mutation
Second
mutation
Third
mutation
Fourth or
later mutation
Uncontrolled growth
7
Understanding your disease
NETs — A type of cancer
Neuroendocrine tumors, or NETs, are a type of cancer affecting neuroendocrine cells. These damaged, or cancerous,
cells look different from the cells in surrounding tissue. Like other types of cancer cells, NET cells may also spread, or
metastasize, to other parts of the body — most often to the liver, lymph nodes, bone, and/or lungs.
The term “advanced NET” is usually used to describe a NET that has grown or spread to other parts of the
body. Many of the symptoms and health issues that people with NETs may experience occur when their NETs
become advanced.
Different names for NETs
Although your disease may have spread to other parts of your body, your original NET (also referred to as a
“primary tumor”) may be quite small. Unlike other types of cancer, such as breast or lung cancer, primary NETs
can appear in many different parts of the body where neuroendocrine cells are found.
In the past, doctors did not realize that all these types of cancer were related. As a result, doctors used different
names and descriptions over the years. The term “neuroendocrine tumor” may not be the exact term your
doctor uses. Other common terms are listed in the table below.
In this booklet, we will only use the term neuroendocrine tumor, or NET, to refer to the whole family of tumors.
Be sure to ask your doctor what terms are used in the office you visit.
Types of NETs
Because NETs can form in so many different places in the body, they are usually named based on where they
first appear. The place where a NET appears is called the primary site or site of origin.
The most common types of NETs are found in the digestive tract, or gastrointestinal tract. This includes the
small and large intestines, appendix, colon, rectum, and stomach. These NETs are sometimes grouped together
with NETs that form in the pancreas and are called gastroenteropancreatic NETs, or GEP-NETs. NETs can also
originate in your lungs, ovaries, testes, thymus, and adrenal glands.
Terms for NETs
• Neuroendocrine tumor
• Carcinoid tumor (typical or atypical)
• Neuroendocrine neoplasm
• Gastroenteropancreatic NET
(GEP-NET)
• Neuroendocrine carcinoma
Types of NETs
• Pancreatic NET; the following are terms
for specific types of pancreatic NETs
–– Islet cell tumor –– Glucagonoma
–– Insulinoma
–– Gastrinoma
• Merkel cell carcinoma (a type of neuroendocrine skin cancer)
• Lung NET
• Goblet cell (a type of NET that starts in the appendix)
• Pheochromocytoma (NET of the adrenal glands)
8
NETs are different from other types of cancer because they can produce hormones. Your doctor may talk
about your NET in terms of whether or not it produces, or secretes, hormones.
Functional NETs produce large amounts of hormones that may cause harmful syndromes. A syndrome is
a set of symptoms that occur together. The syndrome caused by a functional NET depends on the excess
hormones that are secreted.
Nonfunctional NETs do not produce large amounts of hormones. Like other cancers, symptoms or other
manifestations of nonfunctional NETs are usually caused by the tumor’s growth or mass. These symptoms
can include pain, intestinal blockage, or bleeding.
An overview of the most common NET syndromes and their symptoms is shown below. Keep in mind that not
everyone has all the symptoms associated with each syndrome. Be sure to ask your doctor to explain any of
the terms or symptoms you don’t understand when talking about your NET.
Overview of common syndromes and symptoms associated with functional NETs
Primary tumor site
Possible associated syndrome
Possible related symptoms*
Digestive system
Carcinoid syndrome
Flushing, diarrhea, wheezing, abdominal pain,
skin rash, heart disease, weight loss, rectal
bleeding, pain, constipation
Lung
Carcinoid syndrome is rare but may occur
in some patients with metastases
Weight loss, loss of appetite, fever, cough,
shortness of breath, chest pain/back pain
• Ulcers, diarrhea (possibly severe and
• Zollinger-Ellison syndrome (ZES)
watery), abdominal pain
• Severe, watery diarrhea; hypochlorhydria
(low hydrochloric acid levels)
• Verner-Morrison syndrome
Pancreas
• Hypoglycemia, weight gain
• Hypoglycemia syndrome
• Excessive torso fat; large, round face;
(Whipple’s triad)
muscle fatigue
• Other symptoms may include: weight loss,
• Cushing syndrome
rash, diabetes, hyperglycemia, gallstones,
anemia, facial flushing
Thymus
Cushing syndrome
Chest pain, weight loss, shortness of breath
Thyroid
Occasional carcinoid syndrome;
rare Cushing syndrome
Thyroid mass, occasional diarrhea,
facial flushing
Appendix
Syndromes are rare
Nonspecific symptoms, sudden or
severe signs of appendicitis
*Symptoms may vary among patients.
9
Understanding your disease
Classification of NETs
Like other cancers, NETs are classified
using 3 terms that describe important
aspects of the tumor: stage,
differentiation, and grade.
Stage: The extent of disease
Stage is a measure of how far a tumor
has spread. When determining the stage
of a patient’s cancer, doctors look for
3 factors, referred to as TNM: T (Tumor),
the location and size of the primary
tumor; N (Node), whether the tumor
has spread to the lymph nodes; and
M (Metastasis), whether the cancer
has metastasized (spread) to other parts of the body. Health organizations around the world use slightly
different classification systems for NETs. For this booklet, we will use the World Health Organization (WHO)
classification. The WHO classification uses 4 stages (I through IV), with a higher number indicating more
widespread disease.
The following example shows the stage descriptions* for a NET of the small intestine:
Stage Itumor is less than 1 cm in size and has not spread to the lymph nodes or other
parts of the body.
Stage II tumor is greater than 1 cm in size and has started to spread beyond the original
location, but has not spread to the lymph nodes or other parts of the body.
Stage IIIis any size tumor that has spread to nearby areas of the body and also to at least
one lymph node.
Stage IV is any size tumor that has spread to one or more lymph nodes and has also
spread to other, more distant areas of the body (such as the liver).
* The stage descriptions are from the World Health Organization (WHO) Classification of Tumours of the Digestive System.
Please note that the stage descriptions may also vary depending on the type of NET you have. For example,
stages for a NET of the lung can differ from stages for a NET of the colon.
10
Differentiation: What tumor cells look like
Doctors also describe a NET in terms of how it looks under a microscope. This includes how different the
NET cells look compared with the healthy cells around them. This is called “differentiation.”
A NET can be described as being “well-differentiated,” meaning it looks similar to surrounding tissue.
Well-differentiated NETs are considered to be either grade 1 or grade 2 (see table below). Your doctor may
also call these NETs “low grade” or “intermediate grade.” A “poorly differentiated” tumor means that the
tumor cells look very different from the surrounding tissue. These tumors are usually more dangerous and are
categorized as grade 3, or “high grade,” neuroendocrine carcinomas.
Grade: How aggressive the tumor is
The grade of a NET is an estimation of how fast the cancer cells are dividing to form new cells. The grade of a
NET is related to its differentiation. There are several systems for grading NETs, including a new WHO system
introduced in 2010, as shown below.
Grade
Differentiation
1. NET grade 1
Well-differentiated tumor with a low number
of cells actively dividing
2. NET grade 2
Well-differentiated tumor, but with a higher
number of cells actively dividing
3. Neuroendocrine carcinoma
(NEC) grade 3
Poorly differentiated, malignant carcinoma
(most aggressive form)
By using information about stage, differentiation, and grade together, your doctor can decide how best to
manage your NET. Disease classification information can also help you get a sense of how serious your NET is.
Although your doctor may not talk about grade and differentiation with you, he or she may talk about
how serious or aggressive your NET is, which refers to how quickly or how much it may grow or spread.
Ask questions if you are unsure about something or want to better understand your disease.
11
Understanding your disease
Advanced NETs and metastases
NETs are commonly advanced by the time they are diagnosed. Studies show that by the time patients receive
a diagnosis, about 50% have NETs that have spread, or metastasized, to other parts of the body.
Three important reasons explain why advanced NETs are common:
• Some NETs start out so small that they are hard
to find until they have grown or spread
• Nonfunctional NETs do not cause any signs or
symptoms, or may cause symptoms only after
they have grown or spread
• In other cases, NETs cause symptoms that
are similar to those caused by more common
conditions, like irritable bowel syndrome (IBS),
making them harder to diagnose
Cancer that spreads to other organs or to lymph nodes throughout the body is called metastatic disease.
Doctors sometimes also call this regional disease (spreading to nearby organs) or distant disease (spreading
to more distant organs).
How does a tumor metastasize?
Cancer cells can break away from a primary tumor and spread through the body using the bloodstream or
lymph nodes (which produce, store, and carry the cells that fight infections). These cells settle in other parts
of the body and continue to grow and divide there. But under a microscope, they still look like cells from the
site of origin. This is how doctors identify metastases.
Although cancer cells can spread to almost any part of the body, NETs usually spread to the liver, lymph
nodes, and, rarely, to bone.
12
Lymph node metastases
Metastasis of NETs to lymph nodes is called lymph node involvement or regional disease. How often this
happens depends on the type of NET. For example, among NETs of the digestive system, metastases to
regional lymph nodes are found in 20% to 50% of cases. In one type of pancreatic NET called a “gastrinoma,”
lymph node metastases are present 70% to 80% of the time.
Liver metastases
The main function of the liver is to absorb nutrients from the blood and to make bile, a fluid that helps digest
fats. The liver breaks down and stores many of the nutrients absorbed from the intestine and helps remove
toxic wastes from the body.
Cells that break away from the primary tumor are commonly carried through the bloodstream and eventually
are deposited in the liver. In fact, liver metastases develop in approximately two-thirds of patients who have
NETs in their gastrointestinal tract.
Bone metastases
NETs do have the potential to metastasize to bone, but this happens rarely. For example, only 12% of patients
with gastrinomas have bone metastases at diagnosis.
Although disease that has spread can present challenges both for you and for your healthcare team, it is
important to stay positive. NETs can be very different in terms of how serious they are or the symptoms they
cause. Staying informed about the specific characteristics of your NET may enable you to
• Provide your healthcare team with useful information about any changes you may be experiencing
• Ask your healthcare team the most meaningful questions
• Better understand the answers and information your healthcare team gives you
• Feel more confident about your care plan and the role you can play in your care
13
Managing your disease
I fully rely on my oncologist. We have a wonderful,
open relationship, and I fully trust her.
Management starts with an accurate diagnosis
Many people with advanced NETs have been previously diagnosed with disorders other than a NET —
sometimes for years. Once a doctor suspects a NET, however, there are several types of tests that can be
ordered to help confirm a diagnosis. In addition to these tests, the doctor may request a biopsy, or tissue
sample, of the tumor.
Biopsy samples are sent to a laboratory for examination by a pathologist, one of the many specialists who
may be part of your healthcare team. (See “Why multidisciplinary care is important,” page 20) The pathologist
examines NET tissue under a microscope to help determine its grade, stage, and differentiation. (See
“Classification of NETs,” page 10)
A comprehensive pathology report, along with a physical examination and other tests your doctor may order,
provides the information your doctor needs to confirm the diagnosis and develop a customized care plan for you.
[My doctor] said there are going
to be times when you’ll think
you just want to give up. But
you just need to stick with it.
14
Tests your doctor may order
With advanced NETs, many types of tests are used to monitor your disease. Not all tests are appropriate for
all patients. Your doctor will determine which tests are right for you.
Imaging tests
The table below gives an overview of some commonly used tests for monitoring advanced NETs. Imaging
tests use special equipment to show pictures of internal organs or tissues.
Imaging tests
Bronchoscopy
A bronchoscopy is performed using a thin, long, flexible tube with a camera on the tip to take pictures of the lungs
and airways. Prior to the procedure you should not eat or drink for 12 hours. When it is time for the surgery, a local
anesthetic will be sprayed in your nose and mouth to numb your throat. The tube is then inserted through your
nose or mouth and down your throat. The procedure usually takes 30 to 60 minutes to complete.
Computed
tomography (CT)
CT uses x-rays to take pictures of the body from different angles. These pictures are sent to a computer and
combined into images that show the shape, size, and position of internal organs and tissues. The images can then
be used to determine the location and size of tumors. Before your CT scan, you may receive an injection or drink
a fluid containing dye that shows up on the images. After your first scan, your doctor may order a follow-up scan
occasionally to evaluate your tumor’s growth and/or stability. Apart from the injection of the dye, CT is a painless
procedure that can be performed in an outpatient clinic.
Echocardiogram
An echocardiogram is an ultrasound of the heart. The animated images taken can be of the muscles, valves, and
other heart functions. No preparations are necessary prior to the approximately 45-minute procedure.
Gastrointestinal
(GI) probe
A GI endoscopy, or endoscopic ultrasound, is a highly sensitive ultrasound used to help identify the cause of
unexplained GI symptoms. An endoscopy can also be used as a screening test to check for cancer or precancerous
growths. Endoscopic procedures can also be used to collect tissue samples, called biopsies, for evaluation.
Metaiodobenzyl­
guanidine
(MIBG) scan
MIBG is useful for the localization of some NETs. Before the scan can take place, you are injected with a “tracer,”
similar to Octreoscan™, with a small amount of radioactivity that attaches to the tumors cells. Twenty-four to
72 hours after the injection, you will be placed under the scanner, which will take numerous photos as it moves
from your head to your toes.
Magnetic
resonance
imaging (MRI)
MRI is similar to CT except that it uses strong magnets instead of radiation to make the images of your body. MRI
equipment can take views from many angles, and then create detailed pictures of soft tissue, allowing your doctor
to distinguish normal, healthy tissue from tumor tissue. MRI is usually used to monitor tumor growth or spread.
Octreoscan
somatostatin
receptor
scintigraphy (SRS)
Octreoscan is a whole-body imaging technique used to identify a certain type of primary NET that expresses
somatostatin receptors. Before the imaging can take place, you will receive an injection of a tracer chemical
24 hours in advance. This tracer is able to bind to NET cells that have receptors for a hormone called somatostatin.
These cells then appear on the scan. Octreoscan is highly effective in identifying NETs. This imaging technique
can also be used to monitor tumor growth, spread, and response during treatment.
Positron emission
tomography (PET)
PET uses an injectable form of glucose (sugar) as a chemical tracer. The scan calculates the rate at which the
tumor is using the glucose. Usually, more aggressive tumors use more glucose than normal, while less aggressive
tumors use less glucose. Your doctor may order a PET scan to look for new metastases or to help measure the
stage of advanced disease.
Octreoscan is a trademark of Covidien AG or one of its affiliates.
15
Managing your disease
Laboratory tests
Laboratory tests involve taking a sample of your blood or urine to see how much of a certain enzyme or
protein is circulating in your body. Many laboratory tests are available for NETs, some of which depend on the
type of NET and the hormone it secretes. The 2 most common tests are CgA (used for most NETs) and 5-HIAA
(used for NETs that secrete serotonin, which is often associated with carcinoid syndrome).
Laboratory tests
CgA is a protein secreted by neuroendocrine cells that can be
detected in blood. When cancerous neuroendocrine cells divide and
grow uncontrollably, CgA levels increase above normal ranges.
Chromogranin A (CgA)
5-Hydroxyindoleacetic acid (5-HIAA)
CgA testing can be used to help confirm a diagnosis, detect the
return of a tumor, or measure the amount of cancer in your body.
CgA testing may be repeated on a regular basis (as determined by
your doctor) to help measure changes in a tumor, by comparing
changes in your CgA levels with original test results.
5-HIAA is a substance that occurs naturally in your body. Usually
only small amounts are present in urine. NETs that secrete a
chemical called serotonin may cause 5-HIAA levels to rise. 5-HIAA
is measured in a 24-hour urine specimen. It is important to avoid
specific serotonin-rich foods (eg, bananas, tomatoes, avocados,
plantain, plums, pineapples, eggplant, and walnuts) that can
increase urinary 5-HIAA levels prior to specimen collection.
Other tests your doctor may order include urea, electrolytes, pituitary hormone, parathyroid and thyroid
function, and liver function.
Note: Some types of foods, medicines, and medical conditions may affect your test results.
Levels of CgA in your blood, for example, may be increased if you don’t have a NET but have other conditions,
such as inflammation of your stomach lining (called atrophic gastritis), liver or kidney failure, or some types
of heart disease, such as hypertension. CgA levels may also be elevated if you are receiving a proton pump
inhibitor. Your doctor will ask you to stop taking these medicines for a certain period of time before your
CgA test.
Also, foods and medicines containing high levels of serotonin can increase the amount of 5-HIAA in your
urine and could cause incorrect test results. Avoid these foods and medicines before a 5-HIAA test: bananas,
avocados, plums, eggplants, tomatoes, plantains, pineapples, walnuts; cough medicine with guaifenesin,
pain relievers with acetaminophen, skin-care products with salicylates, and medicines or dietary supplements
with l-dopa.
16
The disease is just one of the hard
things life puts in front of us…the
treatments I’m taking are the tools
I need to get back to my daily routine…
Deciding on the right care plan
Because of the different types of NETs, the hormones they may secrete, and the ways NETs can spread,
options for managing each patient’s NET are highly personalized. You and your healthcare team will decide
on a care plan, which may include surgery, medical therapy, or other procedures. It’s also important that
your overall plan covers not only your physical needs, but your psychological and emotional needs, as well.
(For tips on living with your disease, see page 22)
When putting together a care plan, you and your doctor will probably take into account a number
of factors, including
• The size and location of your NET
• Whether your NET is functional or nonfunctional (see page 8)
• How aggressive your NET is (its grade and stage; see page 10)
• Any symptoms you may be experiencing
• Your quality of life, overall health, and personal considerations
17
Possible courses of action
Below are management options for patients with advanced NETs. This information may be helpful when you
discuss your care plan with your doctors.
Surgery
If you have a less-aggressive, grade
1 or grade 2 NET that has not spread,
your doctor may recommend surgery
to fully remove the primary, or original,
tumor. Even if the disease has spread to
other parts of your body, surgery can be
performed to remove these new tumors
or to reduce their size.
Medical therapy
Surgery is the only curative treatment
currently available for NETs. The first
treatment option usually involves
surgical removal of the primary tumor
and associated disease in the lymph nodes, if appropriate. It is important for patients to continue seeing
doctors to ensure that all tumors were removed during surgery and no new ones have formed. Although
surgery may be curative for certain types of NETs, some grade 1 and grade 2 NETs cannot be surgically
removed. This is the case, for instance, if the tumor has grown too big to operate on, if the tumor is in a
location where it is difficult to operate, or if the surgery might be too risky. In such cases, medical therapies
or drugs may be appropriate.
18
Hepatic (liver) artery embolization, chemoembolization, or radioembolization
Hepatic artery embolization is a procedure that cuts off the blood supply to a tumor within the liver by
injecting materials that block the tumor’s blood supply. During chemoembolization, chemotherapy drugs
may be injected directly into the tumor. Similar to chemoembolization, radioembolization allows a more direct
delivery to the tumor, but using radiation rather than chemotherapy drugs.
Radiofrequency ablation (RFA)
In this technique, radio waves are used to heat and destroy cancer cells, particularly in the liver.
Radiotherapy (radiation)
Radiotherapy works by killing cancer cells and shrinking tumors using high-energy x-rays.
Targeted radionuclide therapy
A radionuclide is a chemical element that releases radiation. In targeted radionuclide therapy, a radionuclide
is attached to a molecule (such as octreotide) injected into your bloodstream. The molecule will attach to
certain types of cells in your body and collect in specific areas where the cancer may have spread. The
radionuclide targets these cancer sites and kills the tumor cells with radiation.
Participation in a clinical study
Clinical studies, or trials, are continually being conducted to try to find new and better therapies for NETs. One
of the websites that lists these studies is www.clinicaltrials.gov. Your doctor can help decide if participation in
a clinical study is an option for you.
19
Managing your disease
Why multidisciplinary care is important
The cancer was diagnosed
immediately, and within
15 days we got in touch
with the biggest specialist
in our region.
Multidisciplinary care simply means that many medical specialists, such as nurses, surgeons, oncologists,
endocrinologists, gastroenterologists, and others, all share in treating you. Many times, multidisciplinary
teams specialize in a certain type of cancer, such as NETs. Your multidisciplinary team will join together to
discuss your specific case and decide on the best management strategy for you and your specific needs.
This can be important because NETs are not as common as other forms of cancer and may pose unique
challenges. It may make sense for you to be evaluated at a center that sees many NET patients. There, you
will have access to multiple management options and a variety of highly experienced specialists who are
knowledgeable in treating NETs.
You may have already been referred to a NET
specialty center in your area. This is a center in
which a multidisciplinary team works to provide
you with the best possible care. Your healthcare
team may include some or all of the specialists
shown in the figure on the facing page.
I didn’t have regular cancer.
This is an irregular cancer…
it’s just not run-of-the-mill, and
if you don’t have someone who’s
familiar with it, you may not be
getting the best treatment.
20
Your multidisciplinary healthcare team
Gastroenterologists
Nuclear medicine
specialists
Endocrinologists
Surgeons
Nurses
Social workers/
case managers
Cardiologists
Oncologists
Interventional
radiologists
Pathologists
Oncologist: Specializes in diagnosing and managing cancer, including administering drugs. The oncologist is
usually in charge of your care.
Gastroenterologist: Specializes in diseases of the digestive tract and their symptoms.
Endocrinologist: Specializes in diseases of the endocrine system, such as NETs and diabetes.
Surgeon: Performs surgery, including the removal of tumors.
Pathologist: Specializes in diagnosis and classification of diseases by lab tests, such as looking at cells under
a microscope. The pathologist can determine whether a tumor is benign or malignant.
Cardiologist: Specializes in diseases of the heart and blood vessels, including heart problems that may be caused
by certain types of NETs.
Nuclear medicine specialist: In charge of imaging tumors using radioactive substances. (See “Imaging tests,” page 15)
Interventional radiologist: Uses radiology treatments on tumors that have spread to other parts of the body
(for example, the liver).
Nurse/social worker/case manager: Provides support services for patients with NETs.
You may want to talk to your doctor about NET specialty centers and whether it might be helpful for you to be
referred to one, either for a consultation or for treatment. Perhaps your doctor has already recommended that
you see a specialist or that you look into a particular NET specialty center. Be sure you understand why your
doctor is making a particular referral or recommendation for your care.
21
Living with your disease
Living with an advanced disease like a NET opens the door to many challenges and questions about what will
happen next. Of course, you can’t control the future, but you can make a difference in what you do today. No
matter what type of NET you have, you do have a life and a future.
It’s important to maintain your current quality of life, to communicate with your family and friends about how
you feel and what you are going through, and to take care of any personal matters that you may have. This
section provides some suggestions to help you recognize the things you can control.
Make time for yourself
Don’t let a NET steal your identity. You’ve been
diagnosed with a disease — that doesn’t mean the
disease has to control your life! Make sure you take
time for all the things in your life that empower you
and give you joy. Look forward to holidays and family
celebrations. If spirituality is important to you, make
sure you continue to honor this part of your life as well.
Set goals
Set goals for yourself, but don’t hesitate to take life
one step at a time. Many people talk about “fighting”
their cancer. This helps them feel more in control of
what’s happening to them. You can fight your NET by
doing all that you can to manage your disease and
continuing with your daily activities.
Stay active
Consider starting something new, such as planting a garden or volunteering at a local library or senior center.
You may prefer to start a new hobby, such as painting, or take up a home improvement project to keep active
and gain a sense of accomplishment. Some people may find comfort in continuing their daily routine, such
as walking after dinner. It is important to check with your physician before starting to exercise, or about eating
certain fruits and vegetables that can cause problems for carcinoid patients. Regular, mild exercise is good for
your body and your mental health. The important thing is to do what you enjoy.
22
Diet and rest
Try to eat well. If your appetite has changed recently, eat smaller amounts but more often, and include lots
of fresh fruits and vegetables. If you are having nutritional issues, be sure to keep a food diary, tracking what
and how much you eat, and what/when side effects occur (diarrhea, nausea, etc). If you have any kind of
digestive NET, it can be helpful to review your food diary with an oncology dietitian to help resolve any issues
you may have.
If you have trouble resting, try joining a relaxation class. Getting plenty of sleep is important…take naps if you
feel tired.
Stress
If you are doing everything possible to manage your disease, worrying about your condition will not help.
Always remember that you have the support of a team of healthcare professionals, your family, and your
friends. If feelings of stress start to invade your life, turn to people who inspire and support you most,
including those in a cancer advocacy group.
Don’t be afraid to ask for help!
It is not a sign of failure to ask for help.
If you need to take some time off, or
are feeling a little overwhelmed, don’t
hesitate to ask family members and
friends to help you with whatever you
need. Once people know how you are
feeling, they will be able to give you
more support.
23
Living with your disease
Emotions
A diagnosis of cancer is one of the greatest challenges you can face. It is normal to feel anxious, frightened,
and worried about the future. There are so many things to think about, you may feel overwhelmed. Even
though you and your healthcare team will be focused on managing your physical well-being, it is also
important to take care of your emotional and social well-being.
Many patients diagnosed with an advanced disease suffer from anxiety or depression. This can be a vicious
cycle because if you are anxious or depressed, it may affect your ability to keep doctors’ appointments,
take medication, or even interact with your friends and family. If you think anxiety or depression is having a
negative impact on your life, talk to your doctor. He or she may prescribe counseling or medication as part of
your treatment.
One way to deal with the emotional toll of cancer is to connect with others in the same situation. The easiest
way to do this is to get involved in a support group. The support group websites listed on page 32 of this
booklet can give you a head start. By talking to others facing the same challenges, you’ll realize you are not
alone…and you may even find some helpful advice on how to cope with certain situations.
Counseling
In all walks of life, there are times when anyone can benefit from counseling. Dealing with a NET may be one
of those times. If you or your family need help coping with the realities of your disease, there are professional
counselors and mental health experts ready and willing to help you. It doesn’t matter if the issues troubling
you are major (“Am I going to die?”) or minor (“How do I ask my neighbor to buy my groceries?”). Counseling
professionals may give the encouragement or support you need to stay on track. If your doctor’s office or
multidisciplinary team includes a medical social worker, he or she can help explain available counseling
services. Or, your doctor or nurse can help with
this. Patient websites, like those listed on page 31,
often offer counseling services as well.
24
Building your network of support
When you’re dealing with a NET, there’s no
need to “go it alone.” Surround yourself with
a personal support group — anyone and
everyone who can be a positive force in your
life and help keep you motivated when times
get tough.
• Start with your doctors, because they
want to provide you with the best
care possible. Think of your doctors
as partners in managing your disease.
Discuss your condition with them; ask
them whether they’ve seen any new
research or can point you to Internet
sites with new information. Inquire
about conferences or seminars that
you can attend
• Involve your family. Talk with them about your goals, hopes, and dreams. Tell them how they can
help you
• Make spending time with friends and loved ones a priority, either by going out with them or inviting
them to visit you at your home
• Talk with other patients and patient advocacy groups. One way to accomplish this is to attend a
patient conference. Ask your doctor if there are any groups near you, or check the list on page 32
• Start your own patient support group with other patients who live near you. All it takes to get started is
the desire to connect with other people like you. Your group can be as small or as large as you want.
Ask your doctor for help in locating other NET patients
25
Living with your disease
Communicating with caregivers and loved ones
Credit the doctors first,
but after the doctors,
it’s friends and family.
Talking with caregivers
Whether you get help from a professional caregiver, a family member, or friend, open and honest
communication is the best way to get the care you need. A caregiver can help you with day-to-day activities
and coping with your condition, just by being there for you. He or she can accompany you to doctors’ visits to
make sure all your questions are answered, help you take notes, and provide overall emotional support.
Talking with children
If you have children, you may be concerned about what to tell them. Experienced counselors recommend that
you give your children age-appropriate, truthful information. Children can usually sense when there is something
wrong, so it’s a good idea to give them accurate information instead of leaving it to their imaginations.
There are a few tips when talking to children.
• Children need basic information, including
–– The name of the cancer and the body part affected, such as “lung cancer” or “stomach cancer”
–– What your doctors plan to do about the cancer
–– How their lives will be affected
• Reassure them that
–– They did nothing to cause your illness
–– Cancer is not contagious, and no one in the family will “catch” it from you
• Encourage children to ask you questions. The questions in a young child’s mind may be very different
from what you are expecting. Anticipate additional questions as time goes on. Children will feel better
if they have information on your treatment progress and any other important health-related matters
• Children may worry that a sick parent is going to die. Whether or not they ask, you can explain that
cancer is a long-term disease but not always a deadly one. Reassure them that you are getting the
best possible care
26
Talking with other family members and friends
Your family and friends can be your biggest support group. When you inform family members and friends of
your diagnosis and treatment plans, they most likely will be supportive and caring. But they may also feel the
same emotions that you are experiencing — feelings of anger, anxiety, depression, or disbelief. The same
strategies listed here for your use (such as joining a support group) may help family and friends as well.
Practical issues
Preparing for doctors’ visits
Many patients with NETs will see their doctors and other
healthcare professionals on a regular basis. To make the
most of each visit, do the following:
• Prepare for each visit
–– Bring records of previous visits/tests with you
–– Write down any questions or concerns before
you go
• Know your healthcare team
–– Know what each specialist’s role/focus is
–– Ask why certain referrals, consultations, or tests
are being requested
• Talk openly with your doctors and nurses
–– Ask questions
–– Take notes
• Keep track of and write down all your symptoms, even if
they seem minor or unrelated to your NET. This includes
–– Changes in frequency or severity
–– New symptoms
–– Changes in your eating habits or daily routine
I hear one thing, my
wife hears another,
and then we go back
and talk about what
we THINK we heard.
• Have a friend or caregiver go with you for emotional
support and to take notes so that you can keep track of
everything that was discussed
For additional tips and helpful materials, you can visit www.thenetalliance.com.
27
Living with your disease
It’s going to help you immensely to
talk to your insurance company and
doctors ahead of time. Sometimes it’s
hard to do that when you’re not feeling
well, but take 5 minutes so that there
aren’t any surprises.
Managing your health insurance coverage
Sometimes health insurance issues may seem overwhelming. The bills and insurance paperwork from your
healthcare professionals can be confusing. Each country handles health insurance differently, but here are a
few general tips that may help you:
• Make sure you understand your health insurance coverage
–– Do not hesitate to contact your health insurance company for clarification of your coverage or to
have them answer any questions you may have
• Know whom to talk to within your insurance system. You may be able to ask to have a case manager
or a single individual assigned to you. This way, just one person will handle your questions, approvals,
bills, reimbursements, and other issues
• If you don’t understand what your insurance covers, ask someone knowledgeable to explain it to you
• Get a notebook just for your health insurance coverage
–– Take notes during conversations you have on the phone or in person
–– Be sure to add the dates of your conversations and the names of people you speak with
28
End-of-life issues
At times, despite doctors’ best efforts, all options for care may no longer be effective at treating your cancer.
In some cases, after long, physically and mentally demanding periods of medical care, patients decide to stop
receiving care.
Although it may be difficult and emotional to talk about death with your family and healthcare professionals,
it is important to make your final wishes known. Experts recommend that everyone should have a will. If you
do not have a will, now may be the time to make one. Another way to make your final wishes known is to
establish an advance directive. An advance directive is a legal document detailing your decisions concerning
your medical care in advance, in the event that you can no longer communicate your wishes. Types of
advance directives include a living will and power of attorney.
As some patients approach the end of life, they may decide to receive palliative care or hospice care,
either at home or at a specialty center. Palliative care is given during all stages of cancer, including end-stage
cancer. Programs and services vary by country, but their goals are the same: to keep patients comfortable
and pain free. Hospice care is a form of palliative care for patients who are primarily in the final stage of life.
When a person decides to switch from palliative care to hospice care, a palliative care specialist makes the
process easier.
No matter what decisions you make, be sure to let your loved ones know how you feel about certain issues
now, when you’re able to think clearly and make choices for yourself.
I want to see my children grow up.
I want to live a long time with my
wife. I have so many things to do
I just can’t give up.
29
Resources to help you
My NET group gets me in contact with
people who have lived with their disease
for 10 years or more…and they still live
quite well, and that makes me feel better
and more confident.
Your healthcare team should be your first source for information about your disease. However, people
diagnosed with less common cancers, including NETs, often express interest in making contact with others
who have the same or similar cancer. Support groups exist to help people like you learn more about your
disease and share experiences with others. Sharing both practical advice and emotional concerns can help
you cope with the challenges of living with an advanced NET.
The single best thing I did was join a support group,
and I’m not a support group kind of person…
I realized the room was full of people just like me.
30
NET patient groups
The following list of websites may be of help in finding a group you are comfortable sharing with. These
websites are independently operated. Please note that this list may not be an exhaustive directory of all
websites that provide information about neuroendocrine tumors. Novartis Pharmaceuticals Corporation
has no control over the content of these websites, assumes no responsibility for these sites, nor does it
endorse or verify the accuracy of any of the information contained on third-party websites, such as the
ones listed here.
Australia
The Unicorn Foundation
www.unicornfoundation.org.au
Canada
Carcinoid-Neuroendocrine Tumour Society –
Canada (CNETS-Canada)
www.cnetscanada.org
France
APTED: L’Association des Patients porteurs
des Tumeurs Endocrines Diverses
http://apted.fr/default.aspx
Germany
Netzwerk Neuroendokrine Tumoren
www.glandula-Net-online.de
Bundesorganisation NET
www.net-shg.de
Netherlands
Stichting NET-groep
www.net-kanker.nl/
Norway
CarciNor
www.carcinor.no
Poland
Stowarzyszenie Pacjentow/Osob Wspierajacych
chroych na Guzy Neuroendokrynne
www.rakowiak.pl/statut.htm
Singapore
Carcinoid & Neuroendocrine Tumor Society
(CNETS) Singapore
www.cnets.org/index.htm
Sweden
CARPA (The Carcinoid Patient Association)
www.carpapatient.se/
United Kingdom
NET Patient Foundation
www.netpatientfoundation.org
United States
Carcinoid Cancer Awareness Network
www.carcinoidawareness.org
The Carcinoid Cancer Foundation
www.carcinoid.org
Caring for Carcinoid Foundation
www.caringforcarcinoid.org
Please note that this is not a comprehensive list of all NET patient groups.
Other NET patient groups may be available.
31
Resources to help you
NET awareness (sponsored by Novartis Pharmaceuticals
Corporation) education
Worldwide NET Cancer Awareness Day
www.netcancerday.org
The NET Community
www.thenetalliance.com
CarcinoidLink
www.carcinoid.com
Clinical trials involving NETs
www.clinicaltrials.gov
General cancer information and advocacy groups
American Cancer Society
www.cancer.org
Cancer Society of New Zealand
www.cancernz.org.nz
APOZ and Friends in Bulgaria
www.oncobg.info
Danish Cancer Society
www.cancer.dk
Association for International Cancer Research
www.aicr.org.uk
Irish Cancer Society
www.cancer.ie
Ayrshire Cancer Support
www.ayrshirecs.org
National Cancer Institute (NCI)
www.cancer.gov
Canadian Cancer Society
www.cancer.ca
OncoLink
www.oncolink.org
Cancer Adventures
www.canceradventures.org
Rarer Cancers Foundation
www.rarercancers.org.uk
CancerCare, Inc.
www.cancercare.org
Swedish Cancer Society
www.cancerfonden.se
Cancer Research UK
www.cancerhelp.org.uk
Ulster Cancer
www.ulstercancer.org
32
Notes:
33
Frequently asked questions
Okay, it’s an unusual cancer.
It’s rare, but there are some other
people who have this cancer.
Q. Why are NET cancers often misdiagnosed?
A. NET cancers are difficult to diagnose. When a person first seeks medical help for a NET, he or she usually
has vague symptoms or no symptoms at all. These symptoms can be associated with many different
diseases. For example, the most common location for a NET is the gastrointestinal system, or digestive
tract. However, many people have disorders of the digestive tract. So, doctors are less likely to suspect a
NET during the initial examination, because NETs are not as common as other diseases.
Q. Can NETs be inherited?
A. Most NETs are not genetically passed on from parent to child, but there are cases where NETs are part of
a familial endocrine cancer syndrome. These NETs include MEN1, MEN2, neurofibromatosis type 1, and
von Hippel-Lindau. If you are concerned that your children might have a genetic link to your cancer, talk
to your doctor.
Q. What are clinical trials?
A. A clinical trial is a research study that is designed to test the effectiveness and safety of a new medical
treatment. In a clinical trial, you may have access to a new therapy or combination of therapies that
has never been used before, and you participate in the trial under careful medical supervision. The new
therapy either may be effective in treating the type of cancer you have or may not help you at all. But
clinical trials are one way that medical researchers make progress in developing new therapies and
different management strategies for diseases.
Clinical trials are not for everyone, and only you and your doctor can decide if they may be an option for
you. For more information on clinical trials, talk to your doctor. Also, see the “Clinical trials” website in the
“Resources” section on page 32.
34
Q. Because I have advanced NET disease, what kind of outlook can I expect?
A. It’s important for you to know that every patient is different, and that even when a tumor has spread, the
disease and its symptoms can often be managed for many years. The rate at which NETs grow varies
from patient to patient making it hard to determine the typical life expectancy of a patient with an
advanced NET.
Q. Since I found out about my disease, I’m not interested in the things I used to love doing.
How can I tell if I’m depressed?
A. According to the US National Cancer Institute, depression is a disabling illness that affects about 15%
to 25% of cancer patients. It affects men and women with cancer equally. Patients with advanced NET
disease may be worried about
• Death
• Interruption of life plans (vacations, etc)
• Changes in self-esteem
• Money and legal concerns
Symptoms of depression include difficulty sleeping, loss of appetite, anxiety, loss of interest in usual
activities, and a preoccupation with worries about the future. Counseling may help, but if symptoms
continue, more intensive treatment may be needed. Talk to your doctor if you experience signs and
symptoms of depression.
Q. What is an advance directive, and do I need one?
A. The acceptance of advance directives, and the laws governing their use, is different in each country.
An advance directive, sometimes called a living will, is a written legal document that allows you to make
medical decisions ahead of time in the event that you cannot make those decisions later on. It also
provides a way for you to communicate your wishes to your family, friends, and healthcare professionals,
and to avoid confusion if you are ever too ill to speak for yourself.
A living will usually addresses issues such as
• The use of dialysis and breathing machines
• Whether you wish to be resuscitated, if your breathing or heartbeat stops
• Tube feeding
• Organ or tissue donation
Another form of advance directive is to authorize someone to make decisions on your behalf, such as a
lawyer, spouse, or sibling, in the event you are incapacitated.
35
Glossary of terms
Ididn’t even know how to spell chemoembolization…now I do.
Following is a list of commonly used terms that you may need to know:
Advance directive: A legal document that allows you to make important decisions about your healthcare in
advance, if you cannot make decisions for yourself later on.
Carcinoid syndrome: A set of symptoms (including facial flushing, diarrhea, and abdominal pain) that occur
when a functional NET that secretes serotonin begins to spread or metastasize. The symptoms may be sudden
or severe.
Chemoembolization: A procedure in which the blood supply to the tumor is blocked, either surgically or
mechanically, and anticancer drugs are administered directly into the tumor. This allows a high concentration
of drug to be in contact with the tumor for an extended period of time. Also called transarterial
chemoembolization (TACE).
Chemotherapy: The use of drugs to destroy cancer cells, usually by affecting their ability to grow.
Cushing syndrome: A disorder that occurs when your body produces high levels of the hormone cortisol. Most
people with Cushing syndrome have upper body obesity, and a round, full face.
Differentiation: Differentiation refers to how developed tumor cells are. Well-differentiated tumor cells
resemble normal cells. Poorly differentiated tumor cells do not have the structure or function of normal cells,
and they grow uncontrollably. Poorly differentiated tumor cells are often resistant to treatment and have a
greater chance of being malignant.
Endocrine system: A group of glands and organs that regulate and control various body functions by
producing and secreting hormones into the bloodstream. Ovaries, testes, thyroid, and adrenals are examples
of endocrine glands.
Functional [NET]: A NET that secretes hormones and may cause a variety of symptoms.
Gastroenteropancreatic NET (GEP-NET): A NET that most often originates in the gastrointestinal tract (GI)
or pancreas.
Gastrointestinal tract: Another name for the digestive system, which includes the mouth, throat, esophagus,
stomach, small and large intestines, rectum, and anus.
36
Glucagon: A hormone secreted by the pancreas that raises glucose (sugar) levels in your blood.
Grade: A system of classifying tumor cells. Cells are graded on a 1-to-4 scale, based on how abnormal
they look under a microscope and how quickly the tumor is likely to grow and spread. High-grade tumors
(grade 3 and 4) are more aggressive compared with low-grade tumors (grade 1 and 2).
Hepatic artery embolization: A technique used to treat tumors that are not operable. Blocking agents
are injected into the hepatic artery to cut off the supply of blood and nutrients to a tumor. May be used in
conjunction with anticancer drugs. See “chemoembolization.”
Hormone: A chemical substance, usually a protein, that is released into the body by endocrine glands.
Hormones travel through the bloodstream to different organs and help regulate how some of the organs work
in the body. Testosterone and estrogen are examples of male and female hormones. Another example of a
hormone is serotonin, which sends signals between nerve cells. When serotonin levels in the brain change it
can cause changes in mood.
Hospice care: A type of palliative care that is specifically designed to minimize suffering for dying patients and
their family members.
Hyperglycemia: The condition of having high levels of glucose (sugar) in your blood. Symptoms include dry
mouth; thirst; frequent urination (including at night); blurry vision; and dry, itchy skin.
Hypochlorhydria: The condition of having low levels of hydrochloric acid in your stomach. Symptoms
include bad breath, a metallic taste in the mouth, heartburn, bloating or belching immediately after eating,
and indigestion.
Hypoglycemia: The condition of having low levels of glucose (sugar) in your blood. Symptoms include
dizziness, headache, fatigue, and confusion.
Insulin: A hormone secreted by the pancreas that lowers glucose (sugar) levels in your blood.
Living will: A type of advance directive that contains a written set of instructions outlining your wishes about
the type of medical care you may or may not want. For example, you may want the medical team to use
cardiopulmonary resuscitation (CPR) if your breathing or heart stops. Or you could choose to have Do Not
Resuscitate (DNR) orders, which inform medical personnel that you do not wish to receive CPR if your breathing
or heart stops.
37
Glossary of terms
Lymph nodes: Bean-shaped masses of tissue located in lymphatic vessels throughout the body. Lymph nodes
are the primary site where immune reactions start.
MEN1 (multiple endocrine neoplasia type 1): An uncommon inherited disease in which individuals inherit the
gene that causes overactivity and enlargement of the pituitary gland, the parathyroid glands, and the pancreas.
MEN2 (multiple endocrine neoplasia type 2): A very uncommon inherited disease in which individuals inherit
the gene that causes enlargement of 3 glands: adrenal, parathyroid, and thyroid.
Metastasize: Spread of a tumor from one part of the body to another. The terms “localized,” “regional,” and
“distant” are used to describe how extensively a NET has metastasized. Localized means the NET is at its
original location. Regional usually means it has spread to the lymph nodes. Distant means it has spread to
other parts of the body, such as the liver.
Neurofibromatosis type 1: A rare genetic condition that causes brown spots and tumors on the skin, freckling
in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system,
muscles, bone, and skin.
Nonfunctional [NET]: A NET that does not secrete symptom-causing hormones, or the amount of hormones
secreted is not enough to cause any symptoms at this time.
Palliative care: A type of care that focuses on symptom relief and patient comfort, instead of diagnostic
testing and life-prolonging treatments. The goal of palliative care is to keep patients comfortable and
pain free.
Pancreas: A gland that produces hormones and enzymes that aid in the digestion of food.
Power of attorney: A type of advance directive that designates a person to make medical decisions for you if
you become unable to make them yourself. This person has authority only over medical decisions and not other
matters, like finances.
Stage: The extent of a cancer in a person’s body. Staging is usually based on the size of the tumor, ranked
as stages 1 through 4. In stage 1, the tumor is less than 1 cm in size and has not spread to other parts of
the body, whereas a stage 4 tumor is any size and has spread to at least 1 lymph node and other parts
of the body.
Syndrome: A group of symptoms that occur together and suggest the presence of a certain type of NET.
Thymus: An organ near the base of the neck that produces infection-fighting cells.
38
Thyroid: A small gland in the front of the neck, just under the skin. It produces thyroid hormones, which help
control metabolism.
Tumor: An abnormal growth or mass in the body caused when cells grow at an accelerated rate or fail to die
when they are supposed to. Tumors can be benign (not cancer) or malignant (cancer).
von Hippel-Lindau: A rare, genetic multisystem disorder characterized by the abnormal growth of tumors in
certain parts of the body.
Verner-Morrison syndrome: A syndrome that causes patients to have large-volume, watery diarrhea.
Zollinger-Ellison syndrome: A syndrome characterized by severe, recurrent peptic ulcers.
39
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