Washington, D.C. group to host a local and virtual event 

eGlobalTech, an IT solutions, cybersecurity, and management-consulting firm based in Arlington, VA, plans a full day of fundraising activities with a 2-mile Family Fun Walk on World NET Cancer Day to benefit NETRF.

No matter where you live, you can participate in the DC Walk for Carcinoid Cancer on Saturday, November 10, 2018. Anyone can be a virtual walker in their hometown. 

Local event 

Those in the metro-D.C. area can partake in the 2-mile walk starting at Quincy Park in Arlington, VA at 10:00 am. The route goes through Arlington’s charming Clarendon and Ballston communities and finishes back at Quincy Park for a fun-filled celebration with music, a playground, moon bounce, food trucks, and arts and crafts. For more information about the event, sponsorship information, and to register, please go to https://www.dcwalk4carcinoidcancer.com/.

All Washingtonians are welcome to participate to raise money for a great cause – the goal is to raise $20,000! Prizes will be given to the most successful fundraisers.

Virtual option

Supporters don’t have to live in the Washington Metro Area to participate in the DC Walk for Carcinoid Cancer. This allows participants to be with patients fighting carcinoid cancer in spirit and to help with a noble cause. By signing up to walk, you can create your own fundraising page, and then walk your own route to raise money for a NETs cure.

In between Sebago Lake and Casco Bay, there’s a Maine community where many people know about NET cancer and can even name someone who has died from it.

The abutting towns of North Yarmouth and Cumberland have lost three community members to NETs. In 2017, 22-year-old firefighter Brandon Thibeau died two years after the biopsy of a swollen lymph node showed a NET.

A year earlier, the former town manager of North Yarmouth, Marnie Diffin, succumbed within months of receiving her NET diagnosis. Thirteen years earlier, a 41-year old EMT and mother of four, Susan Taylor, died less than a year into her journey.

North Yarmouth Town Clerk Debbie Grover was connected to all threee and decided to act. “How could it be that for a rare cancer, I could know three people who have been afflicted with it in my small world of Cumberland and North Yarmouth,” she said.

Again this year, she’s planning a benefit breakfast sponsored by the North Yarmouth Fire Company, on Saturday, October 27 from 7 am to 10 am. Brandon’s family, Debbie’s co-workers, and the firefighters are organizing the logistics for an expected crowd of 300.

Heaping plates of eggs, pancakes, sausages, and home fries will be offered with a free-will donation.

Proceeds from the breakfast benefit the NET Research Foundation. Learn how you can sponsor a fundraiser in your community. 

 We are saddened to hear of the passing of Aretha Franklin, the Queen of Soul, who died of a pancreatic neuroendocrine tumor (pNET). She was a legend in the music industry and it is difficult to find the appropriate words to express our sadness of this loss.

“We understand the challenge and the pain that Ms. Franklin and her family endured as a result of her diagnosis,” says Elyse Gellerman, CEO of the NET Research Foundation.  “These challenges drive all of us at the NET Research Foundation to fund research to find treatments and cures on behalf of the approximately 170,000 people in the US with neuroendocrine tumors.”

Since 2005, the NET Research Foundation has invested more than $20 million dollars in neuroendocrine tumor research. Neuroendocrine tumors are rare and misunderstood. It is an uncommon cancer that is underfunded and under-resourced.

The NET Research Foundation is the leading private funder of NET cancer research and a nonprofit 501(c)(3) organization. The mission of the Neuroendocrine Tumor Research Foundation is to fund research to discover cures and more effective treatments for neuroendocrine cancers. The NET Research Foundation awards large-scale, multi-year research grants to leading scientists at renowned research institutions around the world.

 Give today to support the search for the cure.

Here are the differences between pancreatic cancer and pancreatic neuroendocrine tumors.

About the Pancreas

The pancreas is an organ that can be thought of as having two components – the exocrine pancreas and the endocrine pancreas.

The exocrine pancreas makes pancreatic enzymes and transports these enzymes in ducts that empty into the small intestine. These enzymes help your body digest the foods you eat. 95% of the cells in the pancreas serve an exocrine function.

The endocrine pancreas consists of endocrine cells that are arranged in “islets” and release hormones like insulin into the bloodstream.

Pancreatic Cancer and Pancreatic Neuroendocrine Cancer are two distinct cancers with distinct behaviors that originate in different types of cells in the pancreas.

What is Pancreatic Neuroendocrine Cancer?

Pancreatic neuroendocrine cancer is a cancer that originates in the endocrine cells of the pancreas. Tumors that originate in the endocrine cells are referred to as “pancreatic neuroendocrine tumors” or “pancreatic islet cell tumors.”

What is Pancreatic Cancer?

Pancreatic adenocarcinoma is a cancer of the exocrine pancreas and is often referred to as “pancreatic cancer” or “pancreatic adenocarcinoma.”

(Left to Right) NETRF CEO Elyse Gellerman, Kathy Karpus, George Karpus, NETRF Board Chair Dr. Joe Li.

We are pleased to announce a $100,000 grant from the Karpus Family Foundation, Rochester, NY. The grant will help us discover insights into the life cycle of neuroendocrine cancer cells, which can be translated into improving the precision and efficacy of therapies.

This grant comes at a critical moment in the aggressive push forward on the most promising research studies in neuroendocrine cancer. “This gift is not just impactful, it provides hope and inspiration to the entire NETRF community,” said Elyse Gellerman, NETRF Chief Executive Officer. “Donors like Kathy and George Karpus make it possible for NETRF to be nimble and responsive to new lines of inquiry and to advance the most promising NET studies to the next level.”

“We know our gift is in excellent hands and we are excited to see meaningful results from this important research in NETs,” said Kathy Karpus.

During a recent visit to Boston, Kathy and George Karpus visited with the NETRF team to learn more about how the Foundation is fueling increases in NET research with large, multiyear grants designed to answer compelling scientific questions and smaller pilot grants focused on expanding the number of researchers working on NETs.

Patients in different areas of the U.S. are reporting difficulties accessing a relatively new nuclear medicine imaging technique known as the Gallium 68 (Ga-68) scan. These reports prompted a member of the NETRF team to take action.

Josh Mailman, treasurer, NETRF Board of Directors, and president of NorCal CarciNET is a NET patient advocate with a specialized interest in nuclear medicine. In late July, Josh was invited to give a lunch presentation to directors at the Food and Drug Administration (FDA) in Washington, D.C., including results from a patient survey he conducted online.

“The directors are concerned about Ga-68 availability,” said Josh, “FDA maintains information on drug shortages, but the agency has challenges in tracking radiopharmaceuticals availability.”

A small generator is used to produce the Ga-68, which has a short half-life, of about an hour, limiting the time it can be used. Generators that produce Ga-68 need to be replaced approximately every 300 days. Areas of the country that would like to have Ga-68 availability are competing with communities that need their generators replaced resulting in some areas losing a generator and the availability to produce Ga-68.

The Society of Nuclear Medicine and Molecular imaging (SNMMI) recently sent a letter to FDA outlining the Ga-68 shortage. Some of the actions underway to help address the shortage, include:

• The manufacturer that produces the approved generator continues to ramp up production.
• One manufacturer that has an approved Ga-68 generator is seeking approval to be used for imaging with the FDA-approved imaging agent.
• A manufacturer and distributor of Ga-68 generators announced plans for a North American production site in Texas. This new site will have to undergo rigorous regulatory certification prior to any use in patient care.

SNMMI Patient Advocacy Advisory Board

As a member of Patient Advocacy Advisory Board for SNMMI and a member of the Ga-68 working group, Josh will continue to monitor the issue on behalf of patients.

We appreciate all those that participated in Josh’s survey, which allowed him to bring the voice of the patient before the FDA.

New Potential NET Treatment under Investigation

It is hard for doctors to know which patients will respond to which therapies. New approaches in personalized medicine use predictive “biomarkers” to help guide treatment decisions.

A biomarker is a molecule found in blood, body fluid, or tissue. Research sometimes shows that people with a certain biomarker are more likely to respond to treatment than those who do not have the biomarker. Then doctors can test for that biomarker before starting treatment. That way patients who are most likely to improve are the ones given the treatment.

NETRF has funded a study of patient data to look for shared biomarkers among NET patients who participated in a clinical trial of cabozantinib, an approved treatment for kidney and thyroid cancer.

Jennifer A. Chan, MD, MPH, Dana-Farber Cancer Institute, Boston, MA, led a phase II clinical trial of cabozantinib in patients with carcinoid and pancreatic neuroendocrine tumors (pNET) (NCT01466036). Six out of 41 patients with carcinoid and three of out 20 with pancreatic tumors experienced a partial response, with an overall response rate of 15%. Dr. Chan calls the results encouraging.

With funding from NETRF, Chan will now evaluate blood samples from clinical trial participants to see if those who responded to cabozantinib share any common biomarkers.

If Chan identifies a possible biomarker in the NETRF-funded study, she will try to verify it in a phase III trial of cabozantinib in NETs.

Chan said it will take time before we know for sure whether cabozantinib can help those living with NETs. “The phase III trials do take time so it may be several more years until we complete the accrual to the trial and have results. It may be several years out before this is an approved agent.”

Interested in helping support research like this? Learn how at netrf.org/donate.

How to Decode What your Doctor is Saying

Shock, fear, and anxiety accompany a neuroendocrine tumor diagnosis. On top of the emotional response comes cognitive confusion. A blizzard of medical terms, complicated tests, and different classifications may start flying around during every doctor’s visit.  What’s the difference between tumor grade, stage, and site? And why do they matter?

Listen to neuroendocrine tumor experts from around the country explain what these complicated terms mean.

Results of a global survey of close to 2,000 NET patients published in 2016 found that more than one in three patients did not know or had not been told their tumor grade. 

“It’s important for every patient to understand his or her diagnosis,” said Elyse Gellerman, Neuroendocrine Tumor Research Foundation (NETRF) Chief Executive Officer. “But the learning curve is deep and the terminology can be intimidating. That’s why we want to be there to help people understand NETs.”

NETRF offers web-based information, free patient and caregiver conferences, and a video library to help people better understand this uncommon cancer type. 

What does tumor grade mean?

Tumor grade is a description of a tumor based on how abnormal the cancer cells and tissue look under a microscope and how quickly the cancer cells are likely to grow and spread. Tumor grade is determined using laboratory tests of tumor cells. Generally, a lower grade indicates a better prognosis. A higher-grade cancer may grow and spread more quickly and may require immediate or more aggressive treatment.

Laboratory tests help determine tumor grade.

If the cells of the tumor and the organization of the tumor’s tissue are close to those of normal cells and tissue, the tumor is called “well-differentiated .” These tumors tend to grow and spread at a slower rate than tumors that are “undifferentiated” or “poorly differentiated,” which have abnormal-looking cells and may lack normal tissue structures. Based on these and other differences in microscopic appearance, doctors determine the differentiation of a tumor.    

 Patients should talk with their doctor for more information about tumor grade and how it relates to their treatment and prognosis.

What does cancer stage mean?

Stage refers to the extent of your cancer, such as how large the tumor is, and if it has spread. Knowing the stage of your cancer helps your doctor:

• Understand how serious your cancer is and your chances of survival.
• Plan the best treatment for you.
• Identify clinical trials that may be treatment options for you.
  

  Cancer stage is often determined with imaging tests.

To learn the stage of your disease, your doctor may order x-rays, lab tests, and other imaging tests or diagnostic procedures. Doctors may use numbers to describe your cancer stage.

• Stage 0 —  Abnormal cells are present but have not spread to nearby tissue. These abnormals cells may not yet be cancer, but may become cancer.
• Stage I, Stage II, and Stage III —  Cancer is present. The higher the number, the larger the tumor and the more it has spread into nearby tissues or organs.
• Stage IV —  Cancer has spread to distant parts of the body.

Doctors may also use words to describe the stages of cancer. 

• In situ — Abnormal cells are present but have not spread to nearby tissue.
• Localized — Cancer is limited to the place where it started, with no sign that it has spread.
• Regional — Cancer has spread to nearby tissues or organs.
• Distant — Cancer has spread to distant parts of the body.
• Unknown — There is not enough information to figure out the stage.

The words early stage and late stage may also be used. The stage of your cancer affects your treatment options. In neuroendocrine tumors, surgery is often considered for tumors that have not spread to nearby tissue or organs. Doctors may “restage” cancer if it comes back or spreads to other parts of the body. 

What does “primary site” mean?

Neuroendocrine tumors can form in many different places in the body — anywhere there are neuroendocrine cells. The primary site is used to describe the first place cancer cells formed. Sometimes tumors that begin in a certain place, such as the small intestine, share common characteristics. This knowledge of tumor type helps doctors develop standardize approaches for testing and treatment, based on past research and clinical practice. But it is not always possible to identify the primary site. In those cases, it is called a cancer of “unknown primary.”

Check out NETRF’s YouTube channel for patient videos on many other topics. 

The U.S. Food and Drug Administration (FDA)  approved iobenguane I 131, an intravenous radiotherapeutic, for the treatment of unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma in adult and pediatric patients 12 years and older.  Patients must test positive for the norepinephrine transporter (as determined by an iobenguane scan), and who require systemic anticancer therapy. 

Pheochromocytoma and paraganglioma are neuroendocrine cancers that arise from cells in and around the adrenal glands. These glands are located right above the kidneys and make hormones including stress hormones called epinephrines and norepinephrines. Pheochromocytomas increase the production of these hormones, leading to hypertension (high blood pressure) and symptoms such as headaches, irritability, sweating, rapid heart rate, nausea, vomiting, weight loss, weakness, chest pain or anxiety. When this type of tumor occurs outside the adrenal gland, it is called a paraganglioma. Metastatic pheochromocytoma and paraganglioma may result in unresectable disease with a poor prognosis, including a five-year survival rate as low as 12%.  Treatment options for patients with this disease type were very limited prior to this approval. 

“Many patients with these ultra-rare cancers can be treated with surgery or local therapies, but there are no effective systemic treatments for patients who experience tumor-related symptoms such as high blood pressure,” said Richard Pazdur, M.D., director of the FDA’s Oncology Center of Excellence and acting director of the Office of Hematology and Oncology Products in the FDA’s Center for Drug Evaluation and Research. “Patients will now have an approved therapy that has been shown to decrease the need for blood pressure medication and reduce tumor size in some patients.”

“As the first FDA approved therapy for unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma who require systemic anticancer therapy, iobenguane I 131 provides a new treatment option for physicians and their patients,” said Mark Baker, Chief Executive Officer of the drug manufacturer Progenics. “We are extremely grateful to the patients, their families and the investigators who participated in the clinical development program over many years.”

The efficacy of iobenguane I 131 was shown in a single-arm, open-label, clinical trial in 68 patients that measured the number of patients who experienced a 50 percent or greater reduction of all

Phase 2 Trial Tumor Response Rates

antihypertensive medications lasting for at least six months. This endpoint was supported by the secondary endpoint, overall tumor response measured by imaging. The study met the primary endpoint, with 17 (25 percent) of the 68 evaluable patients experiencing a 50 percent or greater reduction of all antihypertensive medication for at least six months. Overall tumor response was achieved in 15 (22 percent) of the patients studied.

The most common severe side effects reported by patients receiving iobenguane I 131 in clinical trials included low levels of white blood cells (lymphopenia), abnormally low count of a type of white blood cells (neutropenia), low blood platelet count (thrombocytopenia), fatigue, anemia, increased blood clotting, nausea, dizziness, hypertension and vomiting.

As it is a radioactive therapeutic agent, iobenguane I 131 can cause serious side effects including:

• Risks from radiation exposure
• Bone marrow problems
• Other cancers (myelosuppression and secondary malignancies)
• Thyroid problems (hypothyroidism)
• Elevations in blood pressure
• Kidney problems (renal toxicity)
• Respiratory problems (pneumonitis)
• Pregnancy risks (embryo-fetal toxicity)
• Fertility problems.
• Please see Full Prescribing Information.

The FDA granted the approval of iobenguane I 131 to Progenics Pharmaceuticals, Inc.

Last August Hurricane Harvey submerged the landscape of Texas, including the roads to MD Anderson Cancer Center, closing one of the nation’s foremost oncology care sites. This summer, wildfires have raged across the west. In Hawaii, Volcano eruptions have engulfed communities. 

Natural or man-made disasters often occur with little or no notice. Being prepared for a disaster is important for everyone, but cancer patients and survivors need to go beyond preparing for these events—they must also prepare for the disruptions to their cancer care caused by a disaster.

Tips for Those with Cancer

During a disaster, people with cancer need to be concerned with their wellbeing, especially if they become displaced and cannot follow their usual routines. After treatment, and especially during active treatment, people can have weakened immune systems and may be at higher risk for infections, bleeding, fatigue, and injury. When things are fine, you may feel there is too much to do to prepare in advance, but it will be worth it if disaster strikes.

To be prepared, take these important steps:

Plan with your healthcare provider. Talk with your health care provider about:

• How to contact your provider in the event of a disaster
• How your provider can reach you during a disaster

Plan with your family and friends. Make a plan with your family and friends, including your neighbors. Consult resources on Ready.gov, an official website of the Department of Homeland Security, to help you identify:

• What help are you likely to need during a disaster.
• Who can provide that help.
• What resources are available.

Create your personal plan. Make a plan for yourself, including all the information you will need to continue your treatment:

• Know your exact diagnosis, cancer stage, and the medications you take. If you’re receiving chemotherapy or radiation, know where you are in your treatment cycle.
• Get the contact information of your health care provider and others whose help you may need during an emergency.
• Make sure you have all the information you need about your clinical trial if you are participating in one, including the National Clinical Trial (NCT) number, the name of the principal investigator, names of any facilities where you receive treatment, and the exact treatment you are receiving.
• Make sure you have all important contact information written down and with you at all times (cell phones may not work, and batteries may drain)
• Carry your insurance card at all times, so you can contact your insurance provider in case you are displaced and need to seek care.

• Make a kit with items you may need (dressings, antiseptic, medications, thermometer, etc.). Put them in a zip lock bag to keep them dry. Resources are available on Ready.gov to help you build a kit.
• Include a 3-day supply of resources for your pets, including current photos of pets in case you are separated.
• Be aware that food and water may not be safe to consume after a disaster. Consult the website of the Center for Disease Control and Prevention for safety tips on food and water use.
• Keep handy the contact information of the Disaster Distress Helpline in case you experience emotional distress (1-800-985-5990 or text “TalkWithUs” to 66746). This hotline is provided by the Substance Abuse and Mental Health Services Administration to provide immediate crisis counseling following natural or human-caused disasters.

After a Disaster

After the storm, MD Anderson offered patients, families, and caregivers some important advice about staying safe during the cleanup to reduce the risk of infection from standing water.

• Keep your hands clean. Wash them frequently with water and soap.
• Clean and cover wounds.
• Limit exposure to mold and water-damaged spaces. Wear a mask if you suspect mold growth.
• Talk to your doctor about a flu shot or other vaccines.
• Protect yourself from mosquitoes with clothing and insect repellent.

Wallet Card
NCI and the American Society of Clinical Oncology (ASCO) developed a free wallet card for patients. The card guides patients to NCI’s and ASCO’s patient information websites and NCI’s Contact Center (1-800-4-CANCER) where they can get more information during a disaster. It includes space for critical information in case a patient is seen by a doctor who is unfamiliar with them.

NCI Contact Center

The NCI Contact Center can help with disaster preparation, updates on the location of the disaster, and where to receive care in the event that a disaster disrupts care or displaces patients. They provide guidance to people regarding information about their treatment, important medical records to have handy in the event of displacement to another location, and they also refer people who have been displaced to nearby cancer centers or NCI Community Oncology Research Program (NCORP) sites.

To reach the NCI Contact Center:

•  Call 1-800-4-CANCER (1-800-422-6237)
  Monday through Friday from 9:00 a.m. to 9:00 p.m. Eastern Time in English or Spanish. After business hours, recorded information is available.
• Online LiveHelp® chat
  The NCI Contact Center LiveHelp program offers online assistance in English and Spanish, Monday through Friday from 9:00 a.m. to 9:00 p.m. Eastern Time.

General Resources

• CDC: Office of Public Health Preparedness and Response
• American Red Cross: How to Prepare for Emergencies
• Ready.gov: Plan Ahead for Disasters
• Cancer.NET Preparing for Evacuation
• MD Anderson: Preparing for Natural Disasters

Image credits: FEMA