Types
of Endocrine Pancreatic Cancer
Endocrine tumors (also
called neuroendocrine tumors or islet cell tumors) account for less
than 5% of all pancreatic tumors. Islet cells of the pancreas
produce hormones including insulin, glucagon, and somatostatin.
A major function of these hormones is to control the amount of sugar
in the blood. Endocrine tumors may be benign or malignant,
and tend to be slower growing than exocrine tumors. In addition,
these tumors are categorized as functional (produce hormones) or
nonfunctional (produce no hormones). The majority of functional
endocrine tumors are benign. However, 90% of nonfunctional
endocrine tumors are malignant, or cancerous. The following
table describes the different types of endocrine tumors.
| Type |
Description |
| Gastrinoma (Zollinger-Ellison Syndrome) |
Gastrinomas are often associated with recurrent ulcers, abdominal
pain, and diarrhea. Diagnosis is dependent
on elevated gastrin hormone levels and imaging
studies. When this tumor is inherited as part of a genetic syndrome
called Multiple Endocrine Neoplasia Type 1 (MEN1), there may
be multiple tumors located within the head of the pancreas and/or
the duodenum. Most gastrinomas are malignant or have the ability
to become malignant. Improved early screening techniques and
increased awareness are leading to increased diagnoses when
tumors are still benign. |
| Glucagonoma |
Glucagonomas are most commonly located in the body and tail
of the pancreas. They are usually large, often metastasize,
and approximately 70% are malignant. This type of pancreatic
cancer may be associated with a severe skin rash, diabetes,
and signs of depression or confusion. |
| Insulinoma
|
Insulinomas are the most common islet cell tumors and tend
to be small and hard to find. They are usually benign and cause
low blood sugar. |
| Multiple Endocrine Neoplasia
Type-1 (MEN1) (Wermer Syndrome) |
MEN1, or Wermer Syndrome, often involves multiple tumors
in the pancreas and parathyroid glands ; the
tumors in the pancreas may be malignant. Symptoms ,
such as fatigue, muscle weakness/pain, constipation, kidney
stones, or thinning of bones, generally appear in individuals
in their 30's and 40's. |
| Nonfunctional Islet Cell
Tumor |
Nonfunctional islet cell tumors do not cause defined clinical
symptoms such as overproduction of one or more pancreatic hormone
(such as insulin). Most are malignant. |
| Somatostatinoma |
Somatostatinomas can occur anywhere in the pancreas and may
also occur at the ampulla of Vater, where the pancreatic duct
enters the duodenum. Like most pancreatic endocrine tumors,
the potential to become malignant varies. If not removed, this
type of tumor can spread to other parts of the body. |
| Vasoactive Intestinal
Peptide-Releasing Tumor (VIPoma or Verner-Morrison Syndrome)
|
VIPoma, or Verner-Morrison Syndrome, tumors are located mostly
in the body and tail of the pancreas. They are often associated
with severe diarrhea and the resulting loss of large amounts
of potassium (hypokalemia). Two-thirds of VIPomas are found
in women. The syndrome is also known as Watery Diarrhea and
Hypokalemia Achlorhydria (WDHA) Syndrome. |
Additional
resources:
Johns
Hopkins: Islet Cell/Endocrine Tumors of the Pancreas
National
Cancer Institute - Islet Cell Carcinoma (Endocrine Pancreas)
Pancreas, Islet Cell Tumors
|
