Appointments

Make your appointment or second opinion with Dana-Farber today to meet with an onsite specialist.

Adult Patients:877-442-3324

Pediatric Patients:888-733-4662

Make Appointment OnlineInternational Patients

Online second opinions

Can’t get to Boston? Explore our Online Second Opinion service to get expert advice from Dana-Farber oncologists.

Request a second opinion

Contact & Directions

Email Dana-Farber

Main Number617-632-3000

Toll-Free Number866-408-DFCI (3324)

Maps & DirectionsContact InformationSend us a Question or Comment

How to Help

Discover the ways to give and how to get involved to support Dana-Farber.

Learn More
Give now

  • Neuroendocrine and Carcinoid Tumors Program

    Dana-Farber/Brigham and Women's Cancer Care

    At the Neuroendocrine and Carcinoid Tumors Program at Dana-Farber/Brigham and Women's Cancer Center (DF/BWCC), our goal is to improve the lives of people with neuroendocrine tumors by offering patients a comprehensive range of services based on specialized treatments and innovative research. Our novel therapies and clinical trials give our patients access to the latest and most sophisticated therapies tailored to their tumor.

    About the Neuroendocrine and Carcinoid Tumors Program

    Jennifer Chan MD, MPH at a computer

    In the Neuroendocrine and Carcinoid Tumors Program, our experts specialize in the treatment of neuroendocrine tumors, while continually developing new and better therapies for the future.

    Our program's highly specialized clinical team is made up of experts from two world-class medical centers — Dana-Farber Cancer Institute and Brigham and Women's Hospital — who are changing the way neuroendocrine tumors are studied, diagnosed, and treated. Because we are such a specialized center, we focus on the distinct needs of people with neuroendocrine tumors. This enables us to provide a very personalized approach to care for our patients.

    Our specialists work closely together to offer coordinated, advanced treatments, including access to novel therapies that were discovered in our own labs, and several clinical trials.

    We offer an approach to treatment that includes:

    • A team of oncologists specializing in neuroendocrine tumors.
    • A multidisciplinary approach to care based on each patient's unique situation.
    • Highly advanced diagnostic procedures.
    • Complex surgical procedures.
    • Interventional radiology techniques, including embolization.
    • Access to leading-edge clinical trials.
    • Pathologic and molecular testing of tumor specimens.
    • Genetic testing and counseling for families with a cancer history.
    • Easy access and timely appointments.

    Innovative approach

    Investigators in the Neuroendocrine and Carcinoid Tumors Program have a proven track record of discovering new and promising treatment options. Our physician-scientists' research informs our approach to your individual treatment. We use Dana-Farber Cancer Institute's neuroendocrine tumor database — the largest of its kind — to study the molecular mechanisms underlying the growth and development of neuroendocrine tumors. We are using this database to develop neuroendocrine tumor cell lines, with the goal of using these models to test new biologic hypotheses and evaluate new drugs.

    Our innovative approach is also reflected in our collaborative, multi-disciplinary model of care that includes some of the best practitioners in every medical discipline. Specialists from gastroenterology, pathology, radiology, surgical oncology, surgery, medical oncology, radiation oncology, nursing, research and other specialties meet regularly to review all approaches to treatment, and to ensure that patients receive the best care plan and most effective treatment.

    The program's experts are leaders in their fields, regularly publishing their discoveries about new therapies, underlying mechanisms of these diseases, genetic discoveries, risk factors, and early detection.

    Compassionate care

    At DF/BWCC, we view every patient as an individual, with unique needs and expectations. This understanding guides us in creating a treatment plan that takes your lifestyle and goals into account. Our team is committed to finding the very best way to help patients and their families cope with the physical and emotional effects of neuroendocrine tumors and their treatment.

    Our patients have access to a wide range of services, including nutrition counseling, rehabilitation, and physical therapy. We also understand that cancer affects more than the body. We offer many support programs and resources that help patients and families, including integrative therapies such as massage and acupuncture.

     

    Frequently Asked Questions About Neuroendocrine and Carcinoid Tumors

    research with samples

    How common are neuroendocrine tumors?

    Although neuroendocrine tumors have historically been considered rare, the incidence of such tumors has been increasing in the past few decades. Recent statistics suggest that more than 100,000 people in the United States have neuroendocrine tumors — more than the number of people with other, better known cancers, like pancreatic or stomach cancer. This increase parallels increased awareness and knowledge about how to diagnose neuroendocrine tumors, as well as how to effectively treat them.

    What causes neuroendocrine tumors?

    In rare cases, neuroendocrine tumors may be familial. Certain inherited conditions, including multiple endocrine neoplasia types 1 and 2, Von-Hippel Lindau disease, and others, result in multiple family members being affected.

    However, for the majority of neuroendocrine tumors (more than 95%), no clear cause has been identified. Traditional cancer risk factors, like exposure to environmental toxins or smoking, do not appear to be strongly associated with neuroendocrine tumors.

    Our researchers are actively investigating potential environmental or genetic causes for neuroendocrine tumors.

    Are there different types of neuroendocrine tumors?

    Neuroendocrine tumors originate from neuroendocrine cells that can be found throughout the body. In some cases, these tumors have the ability to secrete hormones, which can cause specific and unique symptoms.

    Neuroendocrine tumors can be classified according to their site of origin, usually either pancreatic neuroendocrine tumors (which start in the pancreas) or carcinoid tumors (which start in other organs). Carcinoid tumors most commonly start in the lungs, small intestine, appendix, or rectum.

    • Pancreatic neuroendocrine tumors: Pancreatic neuroendocrine tumors may arise either sporadically, or less commonly, in patients with multiple endocrine neoplasia type I (MEN 1) and other inherited syndromes.

      About one-third of pancreatic neuroendocrine tumors secrete hormones, including insulin, glucagon, gastrin, or vasoactive intestinal peptide.
    • Carcinoid tumors: Carcinoid tumors are usually classified based on their site of origin:
      • Foregut tumors include bronchial and gastric carcinoid tumors.
      • Midgut tumors include carcinoids tumors of the small intestine and appendix.
      • Hindgut tumors include colon and rectal carcinoid tumors.
      Neuroendocrine tumors, particularly those that start in the small intestine, can produce hormones such as serotonin, which leads to symptoms including flushing or diarrhea. These symptoms are referred to as carcinoid syndrome.

      Bronchial carcinoids often cause symptoms of cough, wheezing, hemoptysis, and recurrent post-obstructive pneumonia.

      Gastric carcinoids usually do not produce notable symptoms and are often found incidentally.

      Small intestine carcinoids may cause symptoms of intermittent bowel obstruction that mimic irritable bowel syndrome. Appendiceal carcinoids are usually found incidentally. About 50% of rectal carcinoid tumors are asymptomatic and found during a routine endoscopy.

    How are neuroendocrine tumors treated?

    • Surgery: When neuroendocrine tumors are found at an early stage (before cancer has spread to other organs), they can usually be removed surgically. In most cases, treatment with chemotherapy or radiation therapy after surgery is not necessary, and patients can be monitored with blood tests and scans.

    If the tumor has spread and surgery is not possible, a variety of different treatments can be used. These include:

    • Liver-directed therapies: In some cases, the liver is the primary or only site where the neuroendocrine tumor has spread. Liver-directed therapy often involves embolization, an interventional radiology procedure to cut off the blood supply to the liver metastases, and can be very effective.
    • Somatostatin analogs: Most neuroendocrine tumors have receptors for the hormone somatostatin. A number of synthetic somatostatin analogs, including octreotide and lanreotide, are available that mimic the action of somatostatin and can be used to treat these tumors. These drugs are usually highly effective in decreasing hormone production and can also help slow tumor growth.
    • Chemotherapy: Chemotherapy has been used for many years as a treatment for neuroendocrine tumors, and in some cases can be highly effective, particularly for pancreatic neuroendocrine tumors. Drugs that are used include streptozocin and temozolomide.
    • Targeted therapies: Unlike most traditional chemotherapy, targeted therapies are molecules that specifically target growth pathways in the tumor cells. In 2011, two targeted therapies were approved for use for pancreatic neuroendocrine tumors:
      • Sunitinib targets angiogenesis (the process by which tumors grow new blood vessels).
      • Everolimus targets a molecule called mTOR, which serves as a central signaling molecule for a number of cancer growth pathways. Everolimus was also approved in 2016 for the treatment of lung and gastrointestinal neuroendocrine tumors.
       
    • Peptide receptor radionuclide therapy (PRRT): PRRT is a form of therapy that combines a somatostatin analog, which binds to the somatostatin receptor on neuroendocrine tumor cells, and a radionuclide that can deliver radiation to the tumor.
     

    Meet Our Specialists

    Matthew Kulke, MD, MMSc

    Matthew Kulke, MD, MMSc, is director of the Neuroendocrine and Carcinoid Tumor Program at Dana-Farber and a professor of Medicine at Harvard Medical School. Kulke has led numerous clinical trials and translational studies in the neuroendocrine tumor field. A frequent author in the field, he currently serves on the gastrointestinal cancer committee for the Alliance for Clinical Trials in Oncology, and the advisory board for the European Neuroendocrine Tumor Society. He is chair of the North American Neuroendocrine Tumor Society, and also chairs the neuroendocrine tumor guidelines panel for the National Comprehensive Cancer Network.


    Jennifer Chan MD, MPH

    Jennifer Chan MD, MPH, is clinical director of the Neuroendocrine and Carcinoid Tumor Program at Dana-Farber and an assistant professor of Medicine at Harvard Medical School. Chan has broad expertise in the treatment of patients with neuroendocrine tumors. In addition to her clinical practice, she has lectured widely and published numerous articles on treatment approaches for patients with pancreatic neuroendocrine and carcinoid tumors. Chan has played a leading role in the development of novel neuroendocrine tumor therapies, and has served as the principal investigator of several investigator-initiated clinical trials. She serves on the executive committee of the North American Neuroendocrine Tumor Society and on the Neuroendocrine Tumor Task Force of the National Cancer Institute.


    Kimberly Perez, MD, is a member of the DF/HCC Gastrointestinal Oncology and Cancer Genetics and Prevention disease centers. She received her undergraduate training at Georgetown University in Washington, DC, and received her MD from George Washington University in Washington, DC. She completed her residency and fellowship training at Rhode Island Hospital in 2010. After completing her fellowship, she joined the medical faculty at Warren Alpert School of Medicine at Brown University and served as a Gastrointestinal Oncologist at the Comprehensive Cancer Center at Rhode Island Hospital. She joined the faculty at Dana-Farber Cancer Institute in 2015 and is currently an Assistant Professor of Medicine at Harvard Medical School and Dana-Farber Cancer Institute.

    Thomas E. Clancy, MD, is a surgical oncologist specializing in pancreaticobiliary and liver surgery at Brigham and Women's Hospital and Dana-Farber Cancer Institute, and is co-director of DF/BWCC's Pancreas and Biliary Tumor Center. He obtained his medical degree from Harvard Medical School, followed by general surgical residency and surgical oncology fellowships at Brigham and Women's Hospital. Clancy's clinical focus is the treatment of gastrointestinal malignancies, specifically pancreatic, biliary, and liver tumors. In addition, Clancy has a specific clinical and research interest in neuroendocrine tumors. A high-volume clinical surgical oncologist, Clancy has expertise in the surgical management of primary small intestinal and pancreatic neuroendocrine tumors and resection of neuroendocrine tumor liver metastases. Clancy has been a leader in the development and adoption of minimally-invasive treatment techniques, including robotic pancreatectomy.

    Robin Sommers, DNP, ANP-BC, AOCNP, joined Dana-Farber in 2002. Sommers cares for a large population of the patients we treat within the Neuroendocrine and Carcinoid Tumor Program. Her expertise has led her to present numerous discussions on neuroendocrine tumors at various conferences and meetings. Her clinical experience and vast knowledge of the treatment of neuroendocrine tumors have made her a huge contributor to our program.

    Nina Grenon, DNP, AGCNP-BC, AOCN

    See the complete list of our gastrointestinal cancer specialists.

     

    Patient Support, Education, and Follow-up Care

    Zakim Center employee giving a foot massage

    Symptom management

    Nutritional guidance and speech therapy

    Patients can work with nutrition counselors from the very beginning of their treatment to create a customized eating plan. Our nutritionists are registered dietitians, skilled at handling your nutrition needs, and answer your questions about the relationship between cancer and nutrition.

    Managing fatigue and depression

    You may find yourself more tired or experiencing feelings of depression or anxiety while going through cancer treatment. Our Psychosocial Oncology Program can help you and your loved ones to manage fatigue and depression and maintain the best quality of life during and after treatment.

    Pain management and care

    Our center offers pain management and palliative care to our patients. Palliative care is often misunderstood. Many associate it with end-of-life care or "giving up" — especially with a serious health diagnosis like cancer. However, palliative care is best explained as extra support that helps reduce the symptoms, pain, or stress associated with illnesses. Early on, it can help make medical treatments more tolerable. At later stages, it can reduce suffering, help you carry on with daily life, assist you in planning for future medical care, and provide support.

    Integrative therapies

    Many of our patients benefit from consulting with experts at the Zakim Center for Integrative Therapies. Services range from acupuncture and massage to Reiki and music therapy. Our patients treated at the Zakim Center credit its services with easing nausea, improving circulation, and reducing pain, stress, and anxiety associated with cancer treatment.

    Exercise classes and consults

    Our team encourages you to remain active during your treatment. Studies show exercise can help both during and after cancer treatment, strengthening the body, improving mood, and reducing fatigue. Our exercise physiologist can design a safe, effective program tailored to your situation and needs.

    Support programs and follow-up care

    We understand that a neuroendocrine tumor diagnosis can be challenging, both physically and emotionally. Because we are a specialized center, many of our patients appreciate the chance to connect with other people with these kinds of tumors. We also hold educational sessions for patients to learn about the disease and how to care for themselves.

    Our Seminar/Support Group Series is specifically for patients and families affected by neuroendocrine tumors. Patients and caregivers are invited for the first hour. Separate patient and caregiver discussion groups follow during the second hour. The sessions are co-led by Jane Bausch, LICSW, and Susan Englander, LICSW. Registration is required: Please call Jane Bausch, LICSW, at 617-632-3309 for more information and to register. Parking is validated in Yawkey Garage.

    For a full list of our support services, see our Dana-Farber/Brigham and Women's Cancer Center's Supportive Resources brochure.

    Survivorship care and follow-up

    We believe that all our patients should receive expert post-treatment care, in a comprehensive, personalized way. We create a care plan for each patient, with clear steps for monitoring and maintaining your health after your treatment ends.

    Some of our patients participate in the Adult Survivorship Program, a member of the LIVESTRONG Survivorship Center of Excellence Network. This program is committed to finding solutions for living well after cancer and providing compassionate, quality medical care after treatment.

    Find additional information, resources, and support for living well beyond cancer.

     

    Research Breakthroughs for Neuroendocrine and Carcinoid Tumors

    Charles Fuchs, MD, MPH, consults with a patient

    Investigators in DF/BWCC's Neuroendocrine and Carcinoid Tumors Program are international leaders in conducting innovative research and providing precise and patient-focused care. Under the direction of Matthew Kulke, MD, the program's multidisciplinary team of experts continually leverage the latest genomic technologies to learn more about the fundamental biology behind this complex set of diseases, and lead breakthrough clinical trials to improve treatment options for patients.

    Our program helped to advance two targeted therapies — sunitinib and everolimus — that were approved by the Food and Drug Administration in 2011 and changed the standard of care for pancreatic neuroendocrine tumors.

    Kulke and his team are building on this pioneering progress by continuing to launch basic scientific studies to identify the molecular mechanisms responsible for neuroendocrine tumor growth. This work is driving the discovery of novel targets and is expediting the development of new and more impactful treatment strategies — expanding the options available for patients.

    Leadership in expanding treatments for carcinoid tumors

    Everolimus: repurposing an existing neuroendocrine drug

    Dana-Farber investigators are leading an array of studies to uncover new treatments for patients with carcinoid tumors. These rare malignancies continue to present treatment challenges due to the historical lack of understanding about their origin and growth. Matthew Kulke, MD, and his team make up one of the leading programs in the world for treating and studying carcinoid tumors, and they are dedicated to developing more impactful therapies to improve patient outcomes.

    For instance, Kulke and his collaborators recently examined the safety and efficacy of the drug everolimus against advanced carcinoid tumors. The study results revealed that everolimus is effective when used to target this set of tumors, and led its approval by the Food and Drug Administration in 2016 as a treatment for carcinoid tumors.

    Telotristat: targeting the effects of carcinoid syndrome

    Kulke led an international study to evaluate the drug telotristat in treating carcinoid syndrome, a set of severe symptoms that can occur in patients with carcinoid tumors. After publishing findings in July 2014 in Endocrine-Related Cancer revealing that this drug was safe and impactful, Kulke helped to lead a pivotal, international clinical trial to test this drug in patients with carcinoid syndrome who had not responded to standard treatments, and found that telotristat helped relieve some of the most challenging symptoms.

    Advancing new treatment strategies

    Multiple approaches to inhibit angiogenesis

    Scientists now understand that angiogenesis — the formation of new blood vessels — plays a particularly important role in driving neuroendocrine tumor growth due to these tumors' highly vascular nature, which makes them especially reliant on building new blood vessels. Kulke led initial studies of the angiogenesis inhibitor sunitinib in pancreatic neuroendocrine tumors, which led to its approval as a standard treatment for these tumors in 2011.

    Jennifer Chan MD, MPH, clinical director of the Neuroendocrine and Carcinoid Tumors Program, and her colleagues are exploring several strategies to inhibit this key process in neuroendocrine tumors. Chan is currently leading a clinical trial to determine if the drug aflibercept, which is approved to treat colorectal tumors, is effective against neuroendocrine tumors. Aflibercept inhibits angiogenesis by binding to certain proteins called vascular endothelial growth factors (VEGFs) that play a key role in this process. Chan aims to learn whether aflibercept can target VEGFs in neuroendocrine tumors to help prevent these tumors from developing the blood vessels necessary to grow and spread.

    Chan has also developed a study to evaluate another promising angiogenesis inhibitor called ramucirumab. This work builds on the findings of Charles Fuchs, MD, MPH, who recently led an international clinical trial in nearly 30 countries that propelled the FDA to approve ramucirumab as the first-ever targeted agent for gastric cancer. Expanding on this discovery, Kulke and Chan are exploring whether this drug can help to treat patients with neuroendocrine tumors. Repurposing existing compounds can expedite the drug development process, so this study could quickly reveal a new treatment option.

    In a separate study, Kulke worked with collaborators to evaluate the drug pazopanib, another VEGF inhibitor. In the June 2015 Lancet Oncology, the team published their discovery that treatment with pazopanib elicited a positive response in patients with neuroendocrine tumors. These findings recently led to a larger-scale national study of the drug to further test its efficacy against neuroendocrine tumors.

    Strategic use of therapies in combination

    In addition to uncovering novel drugs, investigators aim to understand how best to strategically apply each new therapy and whether these agents are more effective when used alone or combined with other compounds. Kulke led a multi-institution cooperative clinical trial to test everolimus in combination with another angiogenesis inhibitor called bevacizumab. He discovered that this combination was effective in patients with advanced or metastatic pancreatic neuroendocrine tumors, and that together, the two drugs helped to improve progression-free survival and response rates. Kulke presented these findings at the 2015 American Society of Clinical Oncology annual meeting.

    View our current neuroendocrine and carcinoid clinical trials.

    Clinical and laboratory research

    Researchers in the Neuroendocrine Tumor Program have been working to unlock the molecular mechanisms underlying the growth and development of neuroendocrine tumors. Dana-Farber Cancer Institute's neuroendocrine tumor database is at the core of this effort. One of the first and largest databases of its kind, it now includes information and tumor samples from more than 1,000 patients.

    Understanding tumor-environment interactions

    While it is critical to understand which alterations are driving tumors, it is also important for investigators to consider the surrounding tumor environment. Understanding how tumors interact with the rest of the body can reveal unique ways to expand treatment options and maximize therapeutic impact.

    Laboratory models: Expediting drug development

    Working with Matthew Kulke, Michaela Bowden, PhD, and Ewa Sicinska, MD, of Dana-Farber's Center for Molecular Oncologic Pathology, are using the carcinoid model developed at Dana-Farber — one of the few such models in existence — to learn more about the drivers of carcinoid tumors. Through an ongoing genome-wide association study in collaboration with investigators at the Harvard School of Public Health, the team has already uncovered new potential targets and alterations that may be linked to tumor growth. They are continuing to advance their laboratory model so that they can further evaluate these targets and test new drugs — a critical step in uncovering new, more impactful treatments for patients with carcinoid tumors.

    The team's studies using this model also revealed that the carcinoid tumor microenvironment — the cellular environment around a tumor — appears to play a significant role in driving carcinoid tumor development and growth. This finding changed the way that Dana-Farber investigators study this disease, expanding their focus from mutations within cancer cells to the surrounding tissue. Kulke, Bowden, and Sicinska are working to identify the precise mechanisms behind how tumors use their environment to spread, with the ultimate aim of uncovering ways to block this activity and thereby halt tumor growth.

    Immunotherapy: Evaluating possibilities

    Kulke and his team are studying the role of the immune environment in neuroendocrine tumors. Using Dana-Farber's biobank — one of the first and largest databases of neuroendocrine tumor samples — the investigators are exploring the expression of key immune proteins in neuroendocrine tumors and in the surrounding microenvironment, as well as assessing any potential associations with clinical outcomes. Through this study, the team aims to better understand how neuroendocrine tumors interact with the immune system, and whether these tumors are likely to respond to existing or future immunotherapies. This work demonstrates the importance of studying the area surrounding a tumor and how that tumor interacts with the rest of the body and its many complex functions.

     

    Contact Us

    To learn more, or to request an appointment, call us at 877-442-3324 (877-442-DFCI) or 617-632-3476, or complete the online form.

    You may schedule your first appointment for as soon as the next day. After scheduling, one of our nurses will call you to answer your questions and help you prepare for the appointment.

    Mailing address

    Neuroendocrine and Carcinoid Tumors Program
    Dana-Farber Cancer Institute
    450 Brookline Avenue
    Boston, MA 02215

     
     
  •   Email
  •   Print
  •   Share
  • Make an Appointment

    • For adults:
      877-442-3324 (877-442-DFCI)

    • Quick access:
      Appointments as soon as the next day for new adult patients


    • For children:
      888-733-4662 (888-PEDI-ONC)

    • Make Appointment Online
  • How Our Gastrointestinal Cancer Patients Rate Dana-Farber

  • Gastrointestinal Cancer Treatment Center

    • Learn more about Dana-Farber/Brigham and Women's Cancer Center's treatment and support for patients with gastrointestinal cancers.
  • Online Resource for Precision Cancer Medicine

    • Clinicians at Dana-Farber/
      Brigham and Women's Cancer Center are now armed with specialized tests to create a precise "tumor profile" for each patient's cancer. Learn about the latest thinking in precision cancer medicine for certain types of gastrointestinal cancers and explore a searchable database of clinical trials that focus on targeted therapies by disease, gene, and mutation.