/clinical/,/clinical/pted/,/clinical/pted/hffy/,/clinical/pted/hffy/cancer/,

/clinical/pted/hffy/cancer/6587.hffy

201607207

page

100

UWHC,UWMF,

Clinical Hub,Patient Education,Health and Nutrition Facts For You,Cancer, BMT, Hematology

Neuroendocrine Tumors (6587)

Neuroendocrine Tumors (6587) - Clinical Hub, Patient Education, Health and Nutrition Facts For You, Cancer, BMT, Hematology

6587


Neuroendocrine Tumors

Neuroendocrine tumors arise from cells that release a hormone in response to a signal from the
nervous system. “Neuro” refers to the nervous system. “Endocrine” refers to the hormones.
These tumors may secrete large amounts of hormones. This release of hormones can cause a
range of symptoms. Neuroendocrine tumors can arise in many sites, such as the gastrointestinal
(GI) tract, lung, and brain.

Neuroendocrine tumors may be fast growing, or high grade tumors. Tumors such as small cell
cancers, pheochromocytomas and Merkel cell cancer are high grade tumors. There are also
slower growing, or low grade tumors. Tumors such as carcinoid (cancer-like) tumors and islet
cell tumors are low grade tumors. As a rule, the treatment and prognosis for high grade tumors
are much alike. This also true for most low grade tumors.

High Grade Neuroendocrine Tumors
There are many types of high grade tumors. We will address two types of high grade tumors
here.

Pheochromocytoma
This is a cancer of the chromaffin cells that
release the hormone adrenalin during times
of stress. These cells are found in the
adrenal glands. These glands are on the top
of the kidneys. Most of these tumors are
found in one adrenal gland. A few are found
in both or outside of the adrenal glands.

Risk Factors
ξ Age – The tumor most often occurs
between the ages of 40 and 60.
ξ Gender – Men are more likely than
women to get the tumor.
ξ Family history – Ten percent of the
tumors have genetic links.
ξ Immune suppression – People with
suppressed immune systems because
of HIV/AIDS or an organ transplant
have a higher risk of getting a tumor.
ξ Family history of multiple
endocrine neoplasia, type 1
(MEN1) – MEN1 is a hereditary
condition that increases the chance
of getting a cancer of the endocrine
system.

Symptoms
ξ High blood pressure
ξ Fast pulse and heart rate
ξ Palpitations
ξ Anxiety attacks
ξ Fever
ξ Headaches
ξ Nausea and vomiting
ξ Clammy skin

Diagnosis
ξ A biopsy removes a small sample of
tissue. The tissue is looked at under
a microscope.
ξ Urine tests look for a high level of
adrenalin in the body.
ξ Blood tests include a glucagon
stimulation test and a clonidine
suppression test. These tests
measure adrenalin levels in the
ξ CT/MRI I-131-MIBG scan special
scans. They provide detailed images
of the body.



Staging
There is no standard staging system for
pheochromocytoma. The tumors are
placed in one of two groups. The tumors
are either
ξ Localized and benign
ξ Malignant and metastatic

Treatment
The standard treatment is surgery to
remove the tumor. If the tumor has
spread, surgery can still be used to
remove all visible disease. Doing this
will decrease the extra hormone
produced. This will decrease symptoms.
Closely watching blood pressure before,
during, and after surgery is vital.

Targeted radiation therapy may be an
option for some patients. Patients whose
tumor cannot be removed, has recurred,
or has spread, may receive this
treatment. Clinical trials are taking place
to find a useful chemotherapy treatment.

Merkel cell cancer
Merkel cell cancer is an aggressive and very
rare cancer. It starts in the hormone-
producing cells just beneath the skin and in
the hair follicles of the head and neck.

Risk Factors
ξ Age – Merkel cell cancer is most
common in people after the age of
70.
ξ Gender – Men are more likely to get
this cancer than women.
ξ Race – White people are most likely
to get Merkel cell cancer.
ξ Immune suppression – People
infected with HIV/AIDS and people
whose immune systems are
suppressed because of an organ
transplant have a higher risk of
getting a tumor.

ξ Arsenic exposure – Exposure to the
poison arsenic may increase the risk.
ξ Sun exposure – Some doctors
believe that the amount of time spent
in the sun may be a risk factor.

Symptoms
ξ Painless, firm, shiny lumps on the
skin that are red, pink, or blue in
color. They are found in the head and
neck region.

Diagnosis
ξ Biopsy – A biopsy removes a small
sample of tissue. The tissue is
looked at under a microscope.
ξ CT/MRI scan – These special scans
provide detailed pictures of the body.
The scans help find the extent of the
disease.

Staging
ξ Stage IA – The tumor is smaller than
2 cm. There is no spread to the
lymph nodes or distant sites.
ξ Stage IB – The tumor is larger than
2 cm. There is no spread to the
lymph nodes or distant sites.
ξ Stage II – The cancer has spread to
the lymph nodes close to the tumor,
but has not spread to distant sites.
ξ Stage III – The cancer has spread to
distant sites.

Treatment
Surgery is the most helpful treatment.
To destroy any cancer cells that may
remain, radiation might be used.
Clinical trials are taking place to find a
useful chemotherapy treatment.





Low Grade Neuroendocrine Tumors
There are many types of low grade neuroendocrine tumors. We will address these two types of
low grade tumors here.

Gastrointestinal (GI) Carcinoid
These tumors arise from neuroendocrine,
hormone-making cells in the GI tract. The
GI tract includes the stomach, small
intestine, large intestine, and rectum. These
cells produce hormones that help control
digestive juices. They also affect muscle
activity in the GI tract. A GI carcinoid tumor
may produce these hormones in large
amounts. This can cause a group of
symptoms. This group of symptoms are
called carcinoid syndrome. More than half
of carcinoid tumors are found in the GI tract.
They are found most often in the stomach,
appendix, small intestine, and rectum.
Twenty-five percent are found in the lungs.
Most often, these types of tumors are slow
growing.

Risk Factors
ξ Family history of multiple
endocrine neoplasia, type 1
(MEN1) – MEN1 is a hereditary
condition. It is linked to an
increased chance of getting cancers
of the endocrine system.
ξ Having certain conditions that
affect how well the stomach is able
to produce stomach acid. This
includes atrophic gastritis, pernicious
anemia, or Zollinger-Ellison
Syndrome.
ξ Smoking tobacco
ξ Age – The average age at diagnosis
is 61


Symptoms
There may be no symptoms in the early
stages of the tumor. Carcinoid
syndrome is caused by high levels of
serotonin produced by the tumor. The

syndrome may occur if the tumor has
spread to the liver. It occurs most often
when tumors are found in the small
intestine or the right side of the large
intestine (ascending colon).

Symptoms of Carcinoid Syndrome
ξ Flushing, redness, or a feeling of
warmth in the face and neck
ξ Diarrhea or cramping
ξ Shortness of breath
ξ Rapid heart rate
ξ Fatigue
ξ Wheezing
ξ Heart disease
ξ Swelling of the ankles or feet
ξ Pain or a feeling of fullness in the
abdomen

Diagnosis
ξ A Biopsy removes a small sample of
tissue. The tissue is looked at under
a microscope.
ξ Blood tests can point to the presence
and extent of the tumor.
ξ A 24-hour urine collection can help
confirm a diagnosis of carcinoid. It
may also be used to check its
progress.
ξ CT/MRI scans – These special
scans give detailed pictures of the
body. These scans help find the
extent of disease.
ξ Octreoscan – A small amount of
octreotide (Somatostatin®) is given
intravenously (IV) along with a
short-lived radioactive tracer. This
medicine will attach itself to tumor
cells only. The tumor cells can then
be seen on the scan. It is useful in
finding the extent of disease.

Staging
There is no standard staging system.
Tumors tend to be grouped by where they
are found in the body.
ξ Localized – Cancer found in the
stomach, appendix, rectum, or small
intestine only.
ξ Regional – Cancer has spread from
the stomach, appendix, rectum, or
small intestine to nearby tissues or
lymph nodes.
ξ Metastatic – Cancer has spread to
other parts of the body, for example,
the liver.

Treatment
ξ Surgery to remove the tumor is the
standard treatment for cure. Since
these types of tumors are often slow
growing, repeat surgery for tumors
that return may be an option.
ξ Radiation therapy may be used to
ease symptoms caused by disease
that has spread to other parts of the
body.
ξ Chemotherapy has seen little
success in the treatment of these
tumors. Clinical trials are looking
for better treatments. Ask your
doctor if there is a clinical trial you
could be a part of.
ξ Octreotide (Sandostatin®)
injections may be helpful to ease the
symptoms of carcinoid syndrome. It
helps by lowering the amount of
serotonin made.
ξ Radiofrequency Ablation (RFA)
uses a special probe. The probe
releases high-energy radio waves
that kill cancer cells. This treatment
may be used to treat metastatic
disease in the liver or lung.
ξ Cryosurgery uses a device to freeze
the tumor.
ξ Hepatic Artery Embolization
blocks the hepatic artery. This is the
main blood vessel that brings blood
to the liver. Blocking the flow of
blood to the liver and tumor, kills
cancer cells growing there.

Islet Cell Tumors
These tumors are found in the pancreas and
are very rare. Other names for islet cell
tumors include:
ξ Pancreatic islet cell tumor
ξ Pancreatic endocrine tumor
ξ Islet of Langerhans tumor
ξ Neuroendocrine tumor.

Islet Cell Tumors can be either benign (not
cancer) or malignant (cancer). The Islets of
Langerhans are special cells in the pancreas.
These cells produce hormones, the most
important being insulin. Insulin helps
control the amount of sugar in the blood.

Islet cell tumors can be functioning or
nonfunctioning. Functioning islet cell
tumors produce hormones which cause
symptoms. There are five main types of
functioning islet cell tumors.

ξ Gastrinoma also called Zollinger-
Ellison syndrome. With this
syndrome large amounts of gastrin
are produced. This hormone causes
large amounts of acid to be made in
the stomach. As a result of the
excess acid, ulcers may form and
severe diarrhea may occur. Other
symptoms may be abdominal pain
and weight loss.
ξ Insulinomas make too much insulin.
This causes hypoglycemia (low
blood sugar). Weight loss, fatigue,
lightheadedness, and weakness are
common symptoms. Confusion can
also occur. These tumors are rarely
malignant.


ξ Glucagonomas produce too much
glucagon. This causes
hyperglycemia (high blood sugar).
Symptoms of include frequent
urination, increased thirst, skin rash,
and sore tongue.
ξ VIPoma, (Verner-Morrison
syndrome) occur when too much
vasoactive intestinal peptide (VIP) is
made. This is a hormone that affects
water transport in the intestine. Too
much VIP causes large amounts of
watery diarrhea, flushing, fatigue,
nausea, and low potassium.
ξ Somatostatinoma is a tumor that
most often occurs in the head of the
pancreas. This type of tumor makes
too much of a hormone called
somatostatin. This hormone slows
the secretion of other hormones,
including insulin and gastrin. Too
much somatostatin can cause
electrolyte imbalances, diabetes,
weight loss, loss of gastric acid, and
poor absorption of food in the
intestine.

Most islet cell tumors are nonfunctioning.
This means they do not produce large
amounts of hormones. These types of
tumors do not cause the symptoms
mentioned above. As a result, they are often
found at a more advanced stage.

Diagnosis
ξ The tests listed under Carcinoid
Tumors are the same tests used to
diagnose islet cell tumors.

Staging
There is no standard staging system for
islet cell tumors. Often these types of
tumors are placed into one of three
groups:
1. Tumors within the pancreas, and
only occur in one site

2. Tumors within the pancreas, and
occur in many sites

3. Tumors that have spread to lymph
nodes or other parts of the body

Treatment
ξ Surgery to remove the tumor is the
standard treatment for cure. In cases
where a cure can not be achieved
through surgery, debulking may be
done. Surgery in these cases serves
to reduce the amount of tumor.
Doing this may be helpful to ease
symptoms.
ξ Radiation therapy has not been
shown to work well in treating islet
cell tumors.
ξ Chemotherapy has seen only some
degree of success in the treatment of
islet cell tumors. Clinical trials are
looking for treatments that will work
better. Ask your doctor if a clinical
trial is an option for you.
ξ Octreotide (Sandostatin®)
injections may be helpful to ease the
symptoms of an islet cell tumor. It
helps because the amount of
serotonin produced is decreased.
ξ Radiofrequency Ablation (RFA)
uses a special probe. This probe
releases high-energy radio waves.
These waves kill cancer cells. This
treatment may be used to treat
disease that has spread to the liver or
lung.
ξ Cryosurgery uses a device to freeze
the tumor.
ξ Hepatic Artery Embolization
blocks the hepatic artery. This is the
main blood vessel that brings blood
to the liver. Blocking the flow of
blood to the liver and to the tumor
kills cancer cells growing there.


If you have questions of concerns about this handout please discuss them with your doctor or
nurse.










































































Your health care team may have given you this information as part of your care. If so, please use it and call if you
have any questions. If this information was not given to you as part of your care, please check with your doctor. This
is not medical advice. This is not to be used for diagnosis or treatment of any medical condition. Because each
person’s health needs are different, you should talk with your doctor or others on your health care team when using
this information. If you have an emergency, please call 911. Copyright © 7/2016 University of Wisconsin Hospitals
and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6587