Association for
Multiple Endocrine
Neoplasia Disorders

Summary of NANETS Position Statement

The North American Neuroendocrine Tumor Society (NANETS) recently released a position statement regarding COVID-19 vaccination for those who have experienced neuroendocrine tumors or are currently under monitoring or treatment for neuroendocrine tumors.

As a brief summary, NANETS supports COVID-19 vaccination for anyone who has had or currently has neuroendocrine neoplasms, assuming other reasons do not limit that person from receiving a vaccine. Neuroendocrine neoplasms include neuroendocrine tumors such as pancreatic neuroendocrine tumors, medullary thyroid carcinoma, and carcinoid tumors and neuroendocrine carcinomas. Additionally, they recommend prioritizing anyone who is actively receiving cancer treatments, has completed treatments in the last six months, or is expecting to be on treatment soon.

Below are links to the position statement as well as NANETS’s general information regarding COVID:

• NANETS full position statement regarding COVID-19 Vaccination and Neuroendocrine Tumor Patients
• General NANETS COVID-19 Resources

Dr. Blau

We know that some risks are similar as the general population and some would be more specific to the MEN community. For example, people who are over 65 are known to have an increased risk to contract the virus, presumably due to less robust immune systems. In addition, people with certain underlying conditions have an increased risk to develop COVID. These conditions include asthma, chronic lung disease/COPD, people who have serious heart conditions, severe obesity, diabetes, chronic kidney disease on dialysis, or liver disease. Particularly for the MEN1 community, we would want to protect patients who may have type 2 diabetes, or post-pancreatectomy diabetes, or type 1 diabetes.

Dr. Blau

The best way to prevent illness is to avoid being exposed to this virus, and this is no different in the MEN population than in the general population.

• All MEN patients should follow the guidelines set forth by the CDC/NIH. Further information can be found here: https://www.nih.gov/health-information/coronavirus and https://www.coronavirus.gov/. Patients who are already at a higher risk (older age, underlying condition considered high risk such as diabetes, chemotherapy (including targeted therapies like Afinitor (everolimus) or Cometriq/Cabometyx (cabozantinib)), PRRT, heart disease and/or liver disease should follow all guidelines to minimize exposure to the virus.

Stay home if you can.

• As the virus is thought to spread mainly from person-to-person, stay home if you can. We are not only protecting ourselves by doing this, but we are protecting our most vulnerable (including older MEN patients/grandparents/aunts/uncles, etc) as well as those who are undergoing life-saving chemotherapy. Everyone should maintain 6 feet of distance from other people to avoid each other’s respiratory droplets. This is especially important because some recent studies have suggested that COVID-19 may be spread by people who are not showing symptoms.

Wash your hands.

• Everyone should wash their hands often with soap and water for at least 20 seconds especially after being in a public place, blowing your nose, coughing, or sneezing. If soap and water are not available, use a hand sanitizer that contains at least 60% alcohol. Cover all surfaces of your hands and rub them together until they feel dry.

Be hand and touch-aware gurus, and wear a cloth face covering in public.

• As hard as it is, avoid touching your eyes, nose, and mouth with unwashed hands. CDC now recommends that everyone should wear a cloth face cover when they go out in public, for example to the grocery store or to pick up other necessities, which is meant to protect other people in case you are infected.

Keep your home area as virus free as possible

• Lastly, keep your home and other areas that are “frequently-touched areas” clean AND disinfect these surfaces daily. This includes tables, doorknobs, light switches, countertops, handles, desks, phones, keyboards, toilets, faucets, and sinks.

Dr. Kebebew

The following conditions warrant extra caution:

• adrenal insufficiency
• diabetes
• recent treatment with chemotherapy (not including somatostatin analogs), PRRT, or targeted therapies such as everolimus and cabozantinib

Dr. Blau

As far as we know, MEN1 patients don’t have compromised immune systems, however, patients with MEN1 who are undergoing chemotherapy treatments for metastatic neuroendocrine tumors would want to be especially careful (we don’t consider somatostatin analogs chemotherapy).

Dr. Blau

For patients who have undergone a recent surgery, it is important to follow the guidance of your surgeon and to abide by standard wound care precautions. In addition, it is important to keep surfaces and hands clean, as is normally important for wound management. Surgery itself is not considered an underlying condition.

Drs. Blau and Kebewew

Patients on replacement steroids, such as a chronic replacement dose of hydrocortisone (or thyroid/estrogen/testosterone/growth hormone replacement) for pituitary insufficiency related to prior surgery, or adrenal resection, are not considered at increased risk. These hormone medications simply “replace” what is supposed to be made in the body.

There is increased risk for patients who are on very large doses of steroids for a long period of time (conditions that may require this are typically autoimmune or chronic lung conditions, for example).

Also patients on steroid replacement because of adrenal insufficiency could be at higher risk of Addisonian crisis (if they get an infection). All adrenal insufficiency patients should follow sick day rules if they get a fever and/or become ill, and check to be sure that they have an emergency bracelet or necklace to alert emergency responders/physicians that they require extra steroid replacement when ill. In addition, they should inform family members to let their emergency response team know they have adrenal insufficiency if they are unable to do so.

Dr. Blau

There is also no evidence to suggest that dopamine agonists for pituitary adenomas would increase risk.

Dr. Blau

Patients may wonder if chronic intermittent hypercalcemia and/or hypocalcemia could infer risk, and at this point, we don’t believe having high or low calcium would increase risk.

Dr. Del Rivero

Patients with “active” pheochromocytoma associated with MEN2 are at an increased risk for coronavirus infection because excess of hormones (catecholamines and metanephrine.) The excess of hormones related to pheochromocytomas are at risk to develop diabetes, hypertension and heart disease such as heart failure, heart arrythmias and pulmonary edema and those patients should follow up with the U.S. Centers for Disease Control and Prevention (CDC) recommendations. See https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/people-with-medical-conditions.html (Link Updated Feb 26, 2022).

Dr. Del Rivero

Patients with MEN2 and medullary thyroid cancer who are receiving tyrosinase kinase inhibitors such as vandetanib or cabozantinib are at an increased risk for severe illness from coronavirus due to their immune system being weakened by the cancer itself and its treatments and should follow the U.S. Centers for Disease Control and Prevention (CDC) recommendations. See https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/people-at-higher-risk.html.

Patients with advanced medullary thyroid cancer have an overall slower growth rate and delaying treatment between 4-8 weeks it is not considered to worsen the cancer growth.

Dr. Blau

At this time, there is no evidence to demonstrate that surgical removal of part of the thymus at the time of surgery for hyperparathyroidism has a significant effect on the immune system, as the initial stock of T-cells are built during fetal and early post-natal life, with the early years having the biggest influence on T-cell development. In adults, the thymus naturally shrinks by your early 30s.

Typically it is just a partial removal of the upper part of the thymus at the time of surgery for hyperparathyroidism, as a full resection of all of the thymus would require a more extensive surgery. This means that most patients with MEN1 who have had a “thymectomy” usually still have remnant thymus left.

Dr. Del Rivero

While surgery to resect the thyroid is necessary in patients with MEN2, the likelihood is quite low that the thyroid cancer could worsen or spread to other organs if the surgery is delayed for a few months. However, if patients with medullary thyroid cancer have difficulty swallowing or difficulty breathing or if the tumor invades other areas of the neck, the thyroid surgery would need more urgency.

Dr. Blau

All patients would want to be sure that they have enough medications. Most critically would be proton pump inhibitors (omeprazole, pantoprazole, lansoprazole, etc) for those with Zollinger-Ellison Syndrome as a sudden stopping of this medication can be life-threatening, calcium & calcitriol replacement for those with hypoparathyroidism, insulin and or other diabetic medications for diabetes, somatostatin analogs for metastatic neuroendocrine tumors, and steroid replacements (especially thyroid and hydrocortisone) to last them at least 4-6 weeks to avoid having to go out during the recovery phase. Patients should check their health care supplies and let their health care provider know if they may need more.

Dr. Blau

Importantly, if you feel like you are developing symptoms, stay home and call your doctor, and seek a test either through your doctor or locally. Tell them that you have or may have COVID-19. This will help them take care of you and keep other people from getting infected or exposed. If you are not sick enough to be hospitalized, you can recover at home. If you develop emergency warning signs for COVID-19 get medical attention immediately. In adults, some emergency warning signs include difficulty breathing or shortness of breath, persistent pain or pressure in the chest, new confusion or inability for a family member to arouse, bluish lips or face. In these situations, urgent help and transportation to the local ER is the safest step.

Dr. Blau

The clinical symptoms of patients with COVID-19 are varied. Some people develop high fever, cough, shortness of breath and require hospitalization. Others only have mild symptoms (low fever, fatigue, diarrhea/vomiting), and after consultation with their health care provider, are able to recover at home. Using available preliminary data, the median time from onset to clinical recovery for mild cases is approximately 2 weeks and is 3-6 weeks for patients with severe or critical disease. However, everyone is different.

Many patients may experience shortness of breath and weakness/fatigue that can linger for a few weeks. Maintaining adequate water hydration, rest and nutrition is important during recovery. For patients who have a history of kidney stones, dehydration can increase the risk to precipitate stone formation. For these patients, trying to ensure adequate hydration would be important and communicate with your health care provider if you begin to experience symptoms of a stone.

In addition, for patients who have been infected, it is important they wear a cloth face covering, over your nose and mouth if you must be around other people or animals, including pets (even at home, but not if you are alone). If you can’t put on a cloth face covering (because of trouble breathing for example), cover your coughs and sneezes in some other way. Try to stay at least 6 feet away from other people. This will help protect the people around you.

• https://www.coronavirus.govhttps://www.cdc.gov/coronavirus/2019-ncov/index.html
• https://www.nih.gov/health-information/coronavirus
• https://www.cdc.gov/coronavirus/2019-ncov/daily-life-coping/managing-stress-anxiety.html
• https://netrf.org/for-patients/living-with-nets/coronavirus-disease-covid-19-outbreak/