Dear Doctors: We have a family friend who passed away just three weeks after being diagnosed with neuroendocrine cancer. There were no symptoms at all, and it was found pretty much by accident, during an appendectomy. What kind of cancer is that? Does it always move so fast?
Dear Reader: Neuroendocrine cancer is a rare form of the disease. It occurs in a network of specialized cells that, working together, make up the neuroendocrine system. Known as neuroendocrine cells, they behave both as nerve cells and, because they release hormones, also as glands. These cells help regulate digestion, blood pressure, air flow, blood glucose metabolism and muscle and bone development. The pituitary and parathyroid glands are made up primarily of neuroendocrine cells. They also form the inner layer of the adrenal gland. Neuroendocrine cells are found throughout the gastrointestinal tract, including the appendix, and in the lungs. They are also found in the gall bladder, pancreas, liver, kidneys, as well as in the cervix, testicles and prostate.
This type of cancer can develop anywhere in the body and often doesn’t cause symptoms. it is most often seen in the appendix, small intestine and lungs. A small percentage occurs in the pancreas. The presence of symptoms depends on the part of the body that is affected. For instance, as with your family friend, a tumor in the appendix may only be discovered when it causes the signs of appendicitis. These include inflammation, abdominal pain and nausea. Tumors in the lungs may cause wheezing, a persistent cough or shortness of breath.
Although this is often a slow-growing cancer, some forms can advance quite quickly. When symptoms are not present, which prevents the tumor from being discovered, metastasis also becomes a risk. Treatment of the disease depends on each individual case -- that is, the location of the tumor and the degree to which the cancer has spread. When possible, tumors are surgically removed.
If a tumor is quite large, and if the cancer has spread to other parts of the body, supplementary treatments will be considered. Additional treatments can include targeted therapies, which are drugs that interfere with the life cycle of a cancer cell. Some directly target the genes and proteins that allow cancer cells to reproduce. Others take aim at cells that are involved in cancer growth, such as blood vessels. When dealing with neuroendocrine cancers that have spread to other parts of the body, a treatment known as somatostatin analogs may be used. These are drugs that slow the body’s production of certain hormones, which may help slow tumor growth. Chemotherapy can be an option, but it is most effective on fast-growing tumors.
A newer treatment option, known as peptide receptor radionuclide therapy, combines the use of hormone therapy with radiation. It exploits the fact that most neuroendocrine tumors contain specific hormone receptors. In this form of treatment, the patient receives infusions of hormones armed with radioactive molecules, which then bind directly to the cancer cells. This carries the radiation directly to the tumor and spares the surrounding healthy tissues.
(Send your questions to askthedoctors@mednet.ucla.edu, or write: Ask the Doctors, c/o UCLA Health Sciences Media Relations, 10960 Wilshire Blvd., Suite 1955, Los Angeles, CA, 90024. Owing to the volume of mail, personal replies cannot be provided.)
