Until I had received the diagnosis, I had personally never heard of neuroendocrine cancer. Needless to say, I had to do an extensive amount of research to even begin to understand what was going on with my body. For others in this predicament, I recommend proceeding with caution; there is a lot of information on the internet, some of it is helpful and some of it is, let’s be honest, simply terrifying.
For others in this predicament, I recommend proceeding with caution; there is a lot of information on the internet, some of it is helpful and some of it, let’s be honest, is simply terrifying.
So to help those who are seeking answers, the following information is a simple breakdown of the disease, its many manifestations, and what symptoms to look for in terms of carcinoid syndrome.
The Basics of Neuroendocrine Cancer
Neuroendocrine cancer is a slow growing cancer that originates with the enterochromaffin cells of the endocrine system. These cells will normally form in the appendix, ileum, or the rectum. Since this is a slow growing cancer, it can be a long time between presentation of the cancer and diagnosis, which is normally made after the cancer cells have metastasized to other organs, primarily the liver. At this point, the cancer becomes incurable, not terminal. However, if the cancer is located before it has metastasized, it is curable with surgical intervention, so early detection and treatment is of the utmost importance.
NET tumors make themselves known in a variety of ways. It is important to note any changes in health and do not take any symptoms, however minor, for granted. Since early detection is crucial, make sure to share with your physician any symptoms that are not normal for you. For many NET cancer patients, myself included, diagnosis can be delayed because the symptoms are similar to other minor conditions such as crohn’s disease or irritable bowel syndrome. Here is a list of symptoms to share with your physician:
- intermittent abdominal pain
- early satiety
- weight loss
- overt anorexia
Maria J. Gonzalez in her book, But You Look So Good…Stories by carcinoid cancer survivors, shares that roughly 1/3 of persons with NET cancer of the small bowel will experience years of intermittent abdominal pain before receiving a proper diagnosis; again, it is crucial to make sure to keep a list of all abnormal symptoms, leave nothing to chance and share everything with your treating physician (5).
Roughly 1/3 of persons with NET cancer of the small bowel will experience years of intermittent abdominal pain before receiving a proper diagnosis.
For me personally, I experienced gut-wrenching abdominal pain for several years before being diagnosed. It was not until the pain became so unbearable I was forced to head to the Emergency Department that we finally discovered that tumors in the liver were the potential culprit.
If you are wondering if it is necessary to seek medical treatment, there are several other factors that should be considered as well. Not every stomach ache and pain is potentially a carcinoid tumor. You are the best judge of what your body is trying to tell you; however, there are other factors that should be considered when contemplating whether or not to seek treatment. Factors to consider are:
- Age- most cases of NET cancer are diagnosed in people in their 40s and 50s
- Race- there is a greater risk factor for African-American men than Caucasian men
- Gender- NET cancer is more prevalent in men than women
- Family history- there is a higher chance of having NET cancer if it has been diagnosed in other family members
- Preexisting stomach conditions that relate to the production of stomach acid may increase the incidence of tumors developing in the GI tract
- Smoking- the American Cancer Society estimates that smoking may increase the risk of developing carcinoid tumors in the small intestine
- Genetic predisposition- there are three hereditary syndromes where genetic mutations may increase the risk of developing carcinoid tumors. These are: multiple endocrine neoplasm syndrome type 1, Von Hippel-Landau disease, and neurofibromatosis type 1 (Medifocus 24).
In my case I did not have any of these risk factors, I am female, young, non-smoker with no preexisting stomach conditions or genetic predispositions; again, I cannot stress enough that if you are experiencing strange, unexplained symptoms (especially “dry” hot flashes) to seek the advice of physician for further evaluation.
Types of Neuroendocrine Tumors, Their Location, and Testing Information
Neuroendocrine tumors can be found in any organ system of the body, each presenting with its own set of symptoms; however, there are three general areas where it tends to manifest. The most common site for NET tumors to develop is in the foregut which includes the lung, stomach, pancreas, and thymus gland. Secondly, these tumors can develop in the midgut, including the intestines from the duodenum to the furthers most point of the colon (Gonzalez 7). Finally, these tumors can also develop in the hindgut, which includes the left side of the colon and rectum (Gonzalez 7). Symptoms to look for if tumors are developing in these areas are:
- abdominal pain
- bowel obstruction
- weight loss
- early satiety
There are several different options for detecting whether or not pain is related to neuroendocrine tumors. In making a diagnosis, your doctor may employ any or a combination of the following diagnostic tests:
- CT with and without contrast
- PET or a Gallium 68 DOTA PET if available
- Capsule endoscopy
- OctreoScan with SPECT CT
If any lesions are located during these exams, a biopsy will be performed to determine whether or not the lesions are cancerous and if so, what type. Pathologists will be testing for chromograin A levels, Ki-67 to determine how fast the cells are multiplying, and the mitotic count to determine the rate of cellular growth (Gonzalez 14-15). Blood work will also be performed to determine the following levels:
- serum sertonin (or a 24 urine catch may be performed to determine the 5HIAA levels)
- chromogranin A
- Neurokinin A
These tests help the doctor to determine the aggressiveness of the disease and give an approximate projection to longevity. The higher the numbers, the more likely the cancer is to be aggressive and advanced in nature.
This is only a very brief look at the basics of neuroendocrine cancer and potential testing. Each case will be unique and a doctor should be consulted if NET cancer might be suspected based on symptoms.
Gonzalez, Maria J. But You Look So Good…Stories By Carcinoid Cancer Survivors. Bloomington: iUniverse. 2013. Print.
Medifocus. Medifocus Guidebook on: Carcinoid Tumor: a comprehensive guide to symptoms, treatment, research, and support. Silver Spring: Medifocus. 2014. Print.