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Neuroendocrine Cancer Treatment

Neuroendocrine tumors (NETs) — sometimes called carcinoids — are cancers that start in the endocrine cells found throughout the body. Neuroendocrine cells produce hormones, which help the body control many functions from breathing to digestion. NETs form when these cells mutate and multiply, forming a tumor. While NETs are rare and often slow growing, they are all considered cancerous. NETs can occur anywhere in the body and are most common in the small and large intestine, pancreas, appendix and lung.

Neuroendocrine tumors cells are well differentiated, which means they appear to grow slowly under the microscope. When neuroendocrine cancers are poorly differentiated with fast growing cells, they are then called Neuroendocrine Carcinomas (NECs). NECs are treated differently than NETs.

Neuroendocrine Tumors

There are many types of NETs and each requires a different treatment plan. These tumors are grouped based on where they started in the body and if they produce hormones.

  • Carcinoid tumors of the small bowel or large bowel
  • Appendiceal carcinoid
  • Pancreatic Neuroendocrine Tumors
  • Typical Carcinoid tumor of the lung
  • Atypical carcinoid tumor of the lung
  • Pheochromocytoma
  • Paraganglioma

The diagnosis and treatment of NETs depend on the type of tumor, where it is located, what hormones are produced and whether it has spread to other parts of the body. Functional NETs are tumors that produce hormones, while non-functional NETs are tumors that do not produce hormones. For patients with functional NETs, medications like Lanreotide and Octreotide are very important.

Causes and Risk Factors

The cause of neuroendocrine tumors is usually unknown. However, having certain inherited genetic conditions can increase the risk of cancer. Examples include:

  • Multiple endocrine neoplasia, type 1 (MEN 1)
  • Multiple endocrine neoplasia, type 2 (MEN 2)
  • Von Hippel-Lindau disease
  • Tuberous sclerosis
  • Neurofibromatosis

Symptoms

NETs affect the production of hormones. Symptoms can be related to the high hormone levels caused by these tumors. Symptoms may vary depending on the type of cancer and the organs affected. Because these symptoms may be common for many conditions, it is important to see your doctor if you experience them.

  • Fatigue
  • Abdominal pain
  • Abnormal weight loss
  • Swelling in the feet and ankles
  • Skin lesions or rash
  • Diarrhea
  • Nausea

Treatment

Providence Cancer Institute uses the full power of today’s most advanced, evidence-based therapies to treat NETs.

All patients with a new diagnosis of a NET are presented at the Providence tumor boards. Here, a team of experts talk about a patient’s diagnosis and review pathology and imaging to recommend the best treatments. Your personalized treatment plan may include the following treatments:

Surgery

Surgery is an important management strategy for some NET patients, including those with advanced tumors. 

Chemotherapy

Generally only considered for atypical lung carcinoids and pancreatic neuroendocrine tumors.

Peptide Receptor Radioparticle Therapy (PRRT)

Providence Cancer Institute is one of a few centers in the Pacific Northwest that offers PRRT to treat NETs. 
PRRT is a ground-breaking treatment that uses a drug that targets cancer cells along with a radioactive protein. Together, radiation is delivered to the tumor to destroy the cancer cells while sparing healthy cells.

Referrals for PRRT should be sent to Hagen Kennecke, M.D. at the Franz Clinic.

Liver Directed Therapy

Therapies include:

  • Y-90 radioembolization
  • Chemoembolization
  • Radiofrequency ablation
  • Liver radiation

Medications to control hormones

Somatostatin Analogues, such as Octreotide and Lanreotide, are used to slow down the production of hormones.

Radiation therapy

While radiation is not generally required for patients with early stage neuroendocrine tumors, patients with advanced tumors of the bone, liver or other locations may benefit from targeted radiation.

To learn more visit: Providence Radiation Oncology Program

Small Molecule Therapy

Small molecule therapy including everolimus (Affinitor) and sunitinib (Sutent) represent an effective treatment method to control advanced neuroendocrine tumors. There are additional trials underway to evaluate the effectiveness of other small molecule therapies.

Clinical Trials

Earle A. Chiles Research Institute, a division of Providence Cancer Institute, has over 150 open studies across all cancer types. Over 25% of Providence Cancer Institute patients are enrolled in clinical trials compared to a national average of 2% to 4%.

To learn more visit: Research and Clinical Trials at the Earle A. Chiles Research Institute

PRRT Details

Providence Cancer Institute is one of a few centers in the Pacific Northwest that offers PRRT to treat NETs. PRRT is a ground-breaking treatment that uses a drug that targets cancer cells along with a radioactive protein. Together, radiation is delivered to the tumor to destroy the cancer cells while sparing healthy cells.

PRRT is often used when surgery is not an option or when other treatments are not effective at slowing tumor growth. Your doctor will determine if PRRT is an appropriate treatment for you through lab or diagnostic tests.

Referrals for PRRT should be sent to Hagen Kennecke, M.D. at the Franz Clinic.

Treatment details

Treatment usually consists of four sessions, 8-10 weeks apart. PRRT is administered through IV infusion at the Franz Clinic, located at Providence Portland Medical Center. In some cases, the treatment might need to be delayed or stopped.

Treatments take place on weekdays in the outpatient infusion clinic at Providence Cancer Institute Franz Clinic at Portland Providence Medical Center.

Patients are given a firm treatment date about two weeks before the start of PRRT treatment. A patient's last long-acting Octreotide (Sandostatin LAR®) or Lanreotide (Somatuline DEPOT®) injection should be three or more weeks before their PRRT treatment.

Risks and side effects

Possible side effects during or after the treatment are:

  • Fatigue
  • Nausea, mild and occurs in about 10 percent of treatments
  • Mild loss of hair, no baldness. After treatment, the hair grows again.
  • Hormonal crisis (occurring in 1 percent of patients.) This is a temporary risk primarily with tumors that produce a large amount of hormones. There can be a temporary worsening of symptoms. We provide supportive measures to prevent this from happening.
  • Reversible decrease in blood cells, including red blood cells, platelets and white blood cells. These levels are checked before each treatment and if they are too low, the treatment is delayed or decreased or both.

Long term risks

  • Serious deterioration of the kidney function (occurring in less than 1 percent of the patients)
  • Serious deterioration of the bone marrow function (occurring in 2 percent of the patients)

Results of PRRT Treatment

There are strong indications that patients who are treated with PRRT lutetium-177 DOTATE live longer on average and have a better quality of life. The two most important studies that have shown this are published:

Genetics/Clinical Trials

Genetic counseling and testing

Genetics plays an important role in your health. It can help you and your family understand the influence of hereditary gene changes linked to cancer. Genetic counseling and testing is available for cancer syndromes. Our geneticist combines genetic test results, personal factors, family history, and counseling to generate a personalized genetic risk assessment. This assessment estimates future cancer risk for both you and some of your family members. This assessment estimates future cancer risk for both you and some of your family members. Genetic counseling also leads to an improved understanding of your condition by you and your doctor. This improves your treatment planning and future cancer risk reduction and prevention.

If you are a current patient at Providence Cancer Institute talk with your oncology provider about genetic testing.

If you are not a current patient at Providence Cancer Institute, talk with your primary care provider about your risk and family history. Together, you can determine if testing through Providence partner Genome Medical is right for you.

Clinical trials

Clinical trials are research studies that test the safety and success of new medical treatments. Patients who volunteer for clinical trials often gain access to the newest advancements and investigational therapies. At Providence, our doctors and world-class research team work to make appropriate clinical trials a regular part of our patients’ care options.

To learn more visit: Research and Clinical Trials at the Earle A. Chiles Research Institute