At Penn Medicine, patients with neuroendocrine tumors receive their care from a multidisciplinary team of nationally recognized experts in the diagnosis, treatment and research of cancer.
Penn's Neuroendocrine Tumor Program treats the following:
Penn Medicine offers one of the only dedicated NET programs in the country with the combined expertise to treat both GEP-NETs and PHEOs. In addition, Penn's program is the first and only one of its kind in the mid-Atlantic region.
Penn Medicine's multidisciplinary approach to cancer diagnosis and treatment provides better outcomes and gives patients access to the most advanced treatment, surgical techniques and clinical trials.
Because navigating a cancer diagnosis and treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can help them make an appointment with the right physician.
To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN (7366).
Neuroendocrine tumors (NETs) form from cells that release hormones in response to a signal from the nervous system. Some examples of neuroendocrine tumors are carcinoid tumors, islet cell tumors, phechromocytomas and Merkel cell cancers.
Neuroendocrine tumors are often small and can be malignant (cancerous) or benign (non-cancerous). Carcinoid tumors most commonly develop in the gastrointestinal tract including the esophagus, stomach, small intestine, appendix and colon. Rarely, carcinoid tumors occur in the lungs and bronchial tissue. Pancreatic neuroendocrine tumors (PETs) typically develop in the pancreas and duodenum.
Not all NETs cause symptoms. However, because they originate from hormone producing tissues, the symptoms they cause can be linked to the release of various hormones into the blood stream causing:
There may be hereditary reasons for developing NETs. People with:
Other reasons for developing NETs are sporadic, meaning they are isolated in a family and acquired over a lifetime.
There are no known prevention measures for neuroendocrine tumors. Some studies have suggested that smoking doubles the risk of carcinoid tumors in the small intestine so quitting smoking, or not starting to smoke, may reduce risk.
People who have hereditary conditions that may predispose them to developing neuroendocrine tumors should be screened for tumors on a regular basis. Genetic testing may be useful under special circumstances.
The Gastrointestinal Cancer Risk Evaluation Program and the Cancer Risk Evaluation Programs at Penn Medicine offers knowledge about the presence of genetic risk factors for cancer and provides patients with important, sometimes life-saving options.
GEP-NETs occur in the digestive tract. They can be classified as alimentary tract carcinoid tumors or pancreatic endocrine tumors (PETs). GEP-NETs are rare, and account for less than 2 percent of all GI tumors. But, their prevalence is high because they are generally inactive tumors.
Alimentary tract tumors can occur anywhere within the digestive tract including the esophagus, stomach, small and large intestine and rectum. Physicians may refer to this area as the “gut” and further classify a tumor, by its location, as being in the foregut, midgut or hindgut.
Alimentary tract tumors are further classified into two groups:
Most alimentary tract tumors are initially non-functional.
Foregut tumors occasionally are functional and can produce an atypical carcinoid syndrome; midgut tumors often are functional, but the carcinoid syndrome may only develop after metastases have developed; hindgut tumors are generally non-functional.
Pancreatic endocrine tumors originate within the pancreas, or in some cases, in nearby tissues.
PETs are also classified into two groups: those tumors that are functional, or secrete hormones, and those that do not.
If the tumors secrete a hormone, they are classified by the type of hormone they produce. Some of these include:
PHEOs are rare tumors found in both the adrenal and extra-adrenal locations, located above the kidneys.
Because the adrenal glands produce hormones, tumors on these glands can create an overproduction of hormones causing side effects such as high blood pressure and a rapid heart rate. People with PHEOs are often diagnosed with hypertension.
PHEOs are even more rare than GEP-NETs and account for less than two in 100,000 people with neuroendocrine tumors. Only about 800 people in the United States are diagnosed each year with these tumors. Penn Medicine treats 30 to 40 of these patients per year. These patients often have very high blood pressure.
Neuroendocrine tumors are usually slow to develop, and may not cause hormonal symptoms, making the condition difficult to diagnose and stage. Also, because most of the symptoms that do occur are often vague and intermittent, they can be easily confused with other more common conditions.
Staging systems provide doctors with a common language for describing tumors. After a NET is first diagnosed, a series of tests are used to determine the extent of the tumor and to see whether it has spread to other parts of the body from where it started. Staging is a way of recording the size and growth of a tumor, and developing the plan for treatment. By understanding the stage of their tumor, patients can make informed decisions about their treatment.
Cancer specialists at Penn use the TNM staging method as generally accepted by the World Health Organization.
Cancer specialists may also classify each gastrointestinal carcinoid tumor into one of three general stages:
After being diagnosed with a neuroendoctine tumor, patients at the Abramson Cancer Center may be evaluated through the Neuroendocrine Tumor Center, which provides expert support and evaluation to discuss treatment options and individualized treatment plans.
Patients with NETs are treated by a multidisciplinary team of cancer specialists who see more patients with gastrointestinal (GI) cancers in one year than many doctors see in their careers.
In addition, the Neuroendocrine Tumor Center at Penn offers patients:
The Penn Neuroendocrine Tumor Treatment Program is an interdisciplinary program centered on the diagnosis, staging and treatment of neuroendocrine tumors.
GEP-NETs and PHEOs are extremely rare. The tumors are usually slow to develop and difficult to diagnose. Symptoms can be as vague as wheezing, flushing, diarrhea, heart palpitations and cardiac disease. Due to the rarity and slow development of GEP-NETs and PHEOs and their possibly life-threatening complications, early diagnosis depends largely on the experience and expertise of physicians and access to advanced imaging and laboratory facilities, genetic testing and multidisciplinary treatments: A combination of advantages unique to Penn Medicine.
The NET program at Penn was developed under the direction of David C. Metz, MD, professor of medicine, a Penn gastroenterologist and associate chief of the GI division, and Debbie Cohen, MD, associate professor of medicine and a Penn nephrologist. Together with the nurse coordinator, they provide a comprehensive, interdisciplinary approach to the diagnosis, staging, and medical and surgical treatment of NETs.
Penn Medicine offers one of the only dedicated NET programs in the country with the combined expertise to treat both GEP-NETs and PHEOs. In addition, Penn’s program is the first and only one of its kind in the Philadelphia region.
The dedicated NET management team at Penn encompasses a variety of clinical disciplines, including:
Patients also benefit from the resources of the Ruth and Raymond Perelman Center for Advanced Medicine and the Abramson Cancer Center. Adjacent to the Hospital of the University of Pennsylvania, the Perelman Center links Penn physicians and researchers in a common commitment to translational medicine and excellence in care.
Every step of cancer treatment – from cancer diagnosis, to surgery and treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.
Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, patient support specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.
Learn more about Patient Navigation specialists at the Abramson Cancer Center.
An accurate diagnosis from trusted tumor specialists is the first step in getting personalized treatment options to treat neuroendocrine tumors.
Patients who choose Penn Medicine, benefit from a multidisciplinary team of cancer and tumor specialists including gastroenterologists, radiologists, pathologists and surgeons who work together to provide a diagnosis and treatment plan designed specifically for each patient with neuroendocrine tumors.
Cancer specialists at Penn Medicine are highly experienced in using the most advanced techniques for diagnosing cancer and are actively researching better and more precise ways to detect neuroendocrine tumors.
Navigating a cancer diagnosis and treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can help them make an appointment with the right person. Penn Medicine’s contact center has experienced cancer nurses available and ready to guide patients in finding the cancer specialist right for them.
To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN (7366).
Penn Medicine’s multidisciplinary treatment team includes nationally recognized pathologists and radiologists who are experts at finding and diagnosing tumors.
After a thorough medical exam, if a physician suspects a patient may have a NET, he might order one or more of these diagnostic tests:
Staging is a way of describing a tumor: Where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor’s stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's chance of recovery.
Physicians at Penn Medicine use the TNM staging system.
The T stage represents the extent of the primary tumor itself.
The N stage represents the degree of the involvement of the lymph nodes.
The M stage represents whether or not there is spread of cancer to other parts of the body.
The “staging diagnosis” combines the T, N and M groups into four stages (Stage I-IV); some of which have subtypes (a or b) according to overall tumor burden.
Cancer specialists at Penn often classify tumors by whether or not they can be resected, or removed with surgery. There are three ways physicians look at tumors:
Penn Medicine offers one of the only dedicated neuroendocrine tumor (NET) programs in the country with the combined expertise to treat both gastroenteropancreatic neuroendocrine tumors (GEP-NETs) and pheochromocytomas and paragangliomas (PHEOs). In addition, Penn Medicine’s program is the first and only one of its kind in the region.
Following the diagnosis and staging of neuroendocrine tumors, cancer specialists at Penn’s Abramson Cancer Center develop a personalized treatment plan. Penn’s treatment options for neuroendocrine tumors include:
Because navigating a cancer diagnosis and its treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can assist in making an appointment with the right physician.
To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN.
Many carcinoid tumors are successfully treated with surgery, and surgery is the first line of treatment for patients with NETs. Complete removal of the entire tumor is the standard treatment whenever possible. When complete removal of the tumor is not possible, surgeons often remove as much of the tumor as they can to provide some relief from symptoms and possibly improve outcomes.
Penn Medicine has one of the largest gastrointestinal surgical programs in the United States with a nationally recognized record for high-quality patient care and survivorship. Penn Medicine has better outcomes for patients with resection surgeries.
There are two types of surgery:
Surgeons remove the primary tumor and a margin of tissue around the primary location in local NETs. Lymph nodes may also be removed in local-regional surgeries. Most localized tumors can be surgically removed through a skin incision, but rectal or gastroduodenal carcinoid tumors may be removed using an endoscope.
Debulking surgery removes resectable (able to be removed) sections of the tumor, in particular areas of metastases or where the tumor has spread. It often helps relieve or reduce hormonal or tumor symptoms and may improve outcomes.
A liver transplant removes the entire liver and replaces it with a healthy donated liver. Liver transplantation is rarely used in the treatment of carcinoid tumors, but it may help younger patients whose tumors cannot be removed by resection.
Sometimes used for carcinoid tumors and metastases, radiofrequency ablation destroys the tumor by heating it with an electric current.
Penn Medicine specializes in a team approach to chemotherapy treatment with interdisciplinary care and innovative approaches to target tumors prior to surgery.
Hormonal therapy manipulates the endocrine system. Because NETs derive from hormonally responsive tissues, and may produce secretions that affect hormone levels in the body, this treatment may alleviate symptoms and inhibit tumor growth.
Octreotide is a synthetic hormone that inhibits the release of multiple peptides in the body relieving symptoms from NETs.
Proton pump inhibitors (PPIs)
PPIs are a group of drugs that reduce gastric acid production .
Chemoembolization of the hepatic artery
Injecting an anticancer drug into the hepatic artery, which supplies blood to the liver, blocks blood flow to the liver and disrupts the blood supply to the tumor.
Penn’s medical oncologists are combining personalized care with personalized medicine to create targeted therapies for the treatment of cancer. Targeted therapies act as certain molecular pathways leading to tumor suppression.
Chemotherapy uses drugs delivered through the bloodstream to kill cancer cells throughout the body. Chemotherapy for a carcinoid tumor is most often used when the tumor has spread to other organs or is causing severe symptoms. This type of tumor rarely responds to chemotherapy alone, and other treatments may be necessary.
Because NETs can occur in multiple locations, and may be different types of tumors (functioning vs. non-functioning) the type of radiation therapy used for NETs depends on the type of tumor.
Radiation oncologists at Penn Medicine are recognized for developing and using techniques that target radiation precisely to the disease site while sparing nearby tissue.
External beam radiation therapy, most common type of radiation treatment, is delivered by a machine outside the body. A radiation therapy schedule usually consists of a specific number of treatments given over a specific time. External-beam radiation therapy is the most common radiation treatment for carcinoid tumors, and it is most often used to relieve symptoms from cancer that has spread to the bone.
Today, more and more people are surviving cancer. Clinical trials, many of which are conducted at Penn Medicine, benefit patients through breakthrough therapies and treatments. These new advances in cancer treatment are occurring every day, giving patients hope that even greater discoveries lie ahead. Through clinical trials:
Penn Medicine is the only health care facility in the country that is using proton therapy to treat recurrent gastrointestinal tumors in clinical trials. Also, radiation oncologists at Penn Medicine will soon use proton therapy to treat gastrointestinal cancers in the upper digestive tract.
In addition to standard treatments and clinical trials, patients at Penn Medicine may wish to add additional therapies and treatments such as massage therapy, acupuncture and art therapy. These therapies do not have curative intent, and are designed to complement standard treatments, not take their place.
At Penn Medicine, integrative medicine and wellness services can supplement traditional cancer treatments such as chemotherapy, surgery and radiation therapy. While conventional medicine plays a critical role in eradicating cancer, integrative medicine and wellness programs offer patients ways to enhance the quality of their lives, minimize or reduce the side effects of cancer and cancer treatment, and promote healing and recovery.
Cancer specialists at Penn Medicine are knowledgeable and supportive of complementary cancer treatments. The cancer team works with patients and families to integrate these supportive programs into the overall care plan, while ensuring the safety and health of patients.
The Abramson Cancer Center’s range of integrative supportive services is designed to help patients cope with the cancer experience and improve their overall sense of well-being. Services include:
The Abramson Cancer Center at Pennsylvania Hospital at Pennsylvania Hospital offers a variety of supportive care programs for patients and families, from diagnosis through survivorship. These programs are available at no cost to the patients treated at Pennsylvania Hospital, and some are open to patients treated elsewhere. These services include social work counseling, nutrition counseling, psychological counseling and spiritual counseling.
The Cancer Appetite and Rehabilitation Clinic focuses on patients experiencing loss of appetite and weight.
The Supportive Care Clinic helps to manage cancer-related symptoms. Integrative support programs include:
Support groups and educational programs are available at Pennsylvania Hospital throughout the year.
Palliative care provides medical and non-medical interventions to ease the symptoms of cancer and its treatment. Palliative care includes physical, emotional and spiritual care that can enhance the quality of life for cancer patients.
Palliative care can be used to complement traditional cancer therapies, to treat symptoms or to improve quality of life and when curative therapies are no longer an option.
Palliative care is an approach to patient care that can be integrated with curative therapies at any point from diagnosis to survivorship or end-of-life care.
Palliative care services include chemotherapy, radiation therapy and surgery as well as psychological counseling, art therapy and support groups for patients and families. The goals of palliative care are to enhance the quality of life for cancer patients and their families, and provide emotional and spiritual support to enhance personal growth.
Penn Home Care and Hospice Services offer a full range of home health care needs by partnering three top-level home health care services under one roof:
Every step of cancer treatment – from cancer diagnosis, through treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.
Oncology Navigation Specialists at the Abramson Cancer Center are available to the experience as seamless as possible. As experts in navigating complex health care situations, Patient Navigation specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.
Learn more about Patient Navigation specialists at the Abramson Cancer Center.
Patients with neuroendocrine tumors may require more follow-up care than other patients.
Some neuroendocrine tumor patients may require follow-up treatment using biotherapies, or other medications. Others may require more frequent imaging tests. Patients at Penn Medicine receive a personalized survivorship care plan that addresses all issues from follow-up care to the physical, emotional and financial complications that can occur long after their therapy is complete.
Survivorship programs at Penn Medicine are a distinct phase of neuroendocrine tumor care and are designed to help patients' transition from treatment to a post-treatment lifestyle.
At the end of treatment, neuroendocrine tumor patients should schedule a survivorship visit with their nurse navigator. At this time, patients are provided with a summary of the treatment received as well as a plan for follow-up care.
The survivorship care plan includes information on:
A survivorship care plan encourages patients to review the information with their health care team and become active participants in their follow-up care.
Penn also offers neuroendocrine tumor patients a number of support programs and groups to enhance their survivorship care plans.
Every step of cancer treatment; from a cancer diagnosis, through treatment, to forming a survivorship plan; comes with different needs and issues that should be addressed.
Oncology Navigation Specialists at the Abramson Cancer Center are available to make the patient experience as seamless as possible. As experts in navigating complex health care situations, patient support specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.
Learn more about patient navigation specialists at the Abramson Cancer Center.
Penn Medicine’s Living Well After CancerTM Program is a nationally recognized program that focuses on issues facing cancer survivors. In 2007, the Abramson Cancer Center was designated a LIVESTRONG ™ Survivorship Center of Excellence Network. The Abramson Cancer Center is only one of eight LIVESTRONG centers in the United States, and is the only LIVESTRONG Survivorship Center of Excellence in the Philadelphia region. The program focuses on survivorship, a distinct phase of care.
Prescription for Living: The Cancer Survivorship Program at the Abramson Cancer Center at Pennsylvania Hospital at Pennsylvania Hospital, provides patients with a summary of important information about specific cancer diagnoses and treatments, as well as follow-up information and steps to take towards recovery, supportive care and education to help patients adjust to their lives as cancer survivors.
Penn offers cancer patients support programs and groups to enhance their survivorship care plans.
The Abramson Cancer Center and Abramson Cancer Center at Pennsylvania Hospital at Pennsylvania Hospital provide materials and host a wide range of activities for education and support to address key areas of concern including survivorship for cancer patients and their loved ones.
Focus On: Gastrointestinal Cancers and Focus On: Neuroendocrine Tumors are day-long conferences that addresses issues for patients with neuroendocrine tumors, as well as their loved ones.
Peter O'Dwyer, MD, professor of Hematology-Oncology and program director of Development Therapeutics in the Abramson Cancer Center, was interviewed on NBC10's 10! Show about Penn's work as part of the Stand Up to Cancer Dream Team... Read more