What is neuroendocrine cancer?
Neuroendocrine tumors are a rare type of cancer that starts in the neuroendocrine system – where the nervous and endocrine systems work together. Though slow-growing, these tumors can be difficult to diagnose and eventually become life-threatening.

If the tumor produces symptoms – such as flushing of the face, sweating and diarrhea – then they are usually called carcinoid tumors. These are neuroendocrine tumors that produce symptoms as a result of hormones they secrete into the blood stream.

What are the signs and symptoms of neuroendocrine cancer?
Because the neuroendocrine system is highly distributed, this type of cancer can create a variety of signs and symptoms. In many cases, these tumors secrete excessive amounts of different hormones, which cause a range of symptoms. Some of the tumors are distinctive and originate in a particular site in the body. Others are not as easy to classify because they may or may not have “neuroendocrine features.”

Because neuroendorcine tumors – and their symptoms – vary so widely, the specialists at the Kimmel Cancer Center at Jefferson determines treatment based on the type of cancer identified.

What are the different types of neuroendocrine cancer?
The following are some of the different kinds of tumors which fall under the larger category of neuroendocrine cancer:

• Carcinoid tumors. Carcinoid tumors can be benign or malignant. Most begin in one of four places – the appendix, small intestine, rectum or a bronchus (one of the air passages that link the windpipe to the lungs). They can also arise in the stomach, pancreas, colon, liver, ovary, testis or cervix; however, these tumors are less common. Symptoms may include a sudden flushing and reddening of upper body parts (especially the head and neck), a sudden increase in heart rate and blood pressure and diarrhea. Depending on where the tumor is located, other symptoms may include abdominal pain, anemia, pneumonia, cough and spitting of blood. Jefferson’s specialists use a wide variety of imaging techniques and blood tests to determine the precise location and extent of the primary tumor. Those help in identifying the course of treatment – which may include surgery and, in some cases, chemotherapy or radiation therapy.
• Pheochromocytoma. These tumors occur in the adrenal medulla – the area located inside of the adrenal glands – and sometimes arise as part of multiple endocrine neoplasia (MEN) syndrome. This condition can also lead to other cancers of the endocrine system and hormonal abnormalities. Pheochromocytoma itself often causes the adrenal glands to over-produce hormones called catecholamines. Too much of these hormones may cause high blood pressure, rapid pulse, palpitations, anxiety attacks, fever, headaches, nausea and vomiting, and clammy skin. However, most pheochromocytomas are not malignant. Jefferson’s specialists use blood and urine tests in combination with as imaging tests (such as CT scanning) to diagnose or rule out pheochromocytoma.
• Merkel’s cell tumors. Merkel’s cell cancer is a rare type of cancer that forms on or just beneath the skin and in hair follicles and, in some cases, in underlying soft tissue, as well. Also known as neuroendocrine cancer of the skin or trabecular cancer, this type of cancer occurs mostly in Caucasian people who are between the ages of 60 and 80. Merkel’s cell tumors usually appear as firm, shiny but painless skin lumps that are red, pink, brown or blue and range from about a quarter inch to two inches in size. They are most often (but not always) found on the sun-exposed areas of the head and neck. These tumors grow quickly and can rapidly metastasize to other parts of the body. In fact, this type of cancer often spreads to the regional lymph nodes and may also go to the liver, bone, lungs and brain. The preferred treatment for Merkel’s cell tumors is surgery followed by targeted radiation therapy to destroy any remaining cancer cells. When Merkel’s cell cancer has metastasized, it is typically treated with chemotherapy. Even though this type of cancer may respond to chemotherapy, it is not usually cured, and early diagnosis and treatment is critical to decreasing the odds that it will spread.
• Neuroendocrine carcinomas. These tumors are often extremely undifferentiated and can be diagnosed only when a pathologist examines a tissue sample using specialized stains that help clarify cells’ subtype. Also, urine may be tested for elevated levels of specific products that are related to this type of cancer. While some neuroendocrine tumors (Merkel’s cell, for example) possess clear structural characteristics, many are never distinguished beyond the broader category of “neuroendocrine carcinoma.” Neuroendocrine carcinomas can start in a number of locations – including the gastrointestinal tract, lung and brain. Sometimes, the tumor cells produce substances similar to hormones; other times, they produce nothing and cause no symptoms. Treatments include surgery, radiation therapy and/or chemotherapy.

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To schedule an appointment with a Jefferson physician call 1-800-JEFF-NOW or click here.

To contact the Liver Tumor Program team at the Kimmel Cancer Center at Jefferson, please call 215-503-8752 and/or fax your records to 215-503-8755. We aim to schedule your first clinic visit within two weeks of your diagnosis being made and records being received.