Neuroendocrine Carcinoid/Pancreatic Tumors

The endocrine system is made up of organs called glands. These glands make and release hormones (chemical messengers) into the bloodstream.

Hormones regulate many functions in the body. These functions include the use and storage of energy, growth and development, reproduction, secondary sex characteristics (such as underarm and pubic hair), and the amount of fluid, salt and sugar in the blood.

Neuroendocrine tumors (NETs) develop from the glands that make hormones. They are found along the digestive tract, such as in the appendix, small bowel, pancreas and colon. They are different from other cancers because NETs have the ability to secrete excess hormones into the bloodstream that cause symptoms.

NETs tend to grow slowly and may or may not cause symptoms. They are often found during routine imaging.

Physicians at the New York-Presbyterian/Weill Cornell Medicine Endocrine Oncology Center are highly skilled in diagnosing and treating NETs. We offer patients the newest tests, technology and treatments available and customize care to meet their individual needs.

Our team of experts is dedicated to providing patients the highest quality care and the best treatment experience possible.

Types of Neuroendocrine Tumors

There are different types of NETs. Some tumors produce hormones and others do not. Non-functioning tumors grow without producing hormones. Functional tumors are those that make and release hormones into the bloodstream.

Pancreatic NETs

Pancreatic NETs are slow-growing tumors that can overproduce hormones (functional tumors). These tumors include:

●      Insulinoma (releases excess insulin, which causes extremely low blood sugar)

●      Gastrinoma (releases excess gastrin, which causes ulcers in the stomach and small intestine)

●      VIPoma (releases excess vasoactive intestinal peptide [VIP], which causes severe watery diarrhea)

●      Glucagonoma (releases excess glucagon, causing high blood sugar, skin rashes, blood clots and depression)

●      Somatostatinoma (releases excess somatostatin, which causes gallstones, diarrhea, high blood sugar and weight loss)

Some pancreatic tumors may metastasize (spread) to other organs in the body. For example, 90 percent of insulinomas do not metastasize, whereas 75 percent of glucagonomas can spread outside the pancreas. More than half of non-functioning pancreatic tumors are malignant (cancerous) at diagnosis.

Gastrointestinal NETs

Gastrointestinal NETs (GI-NETs or carcinoids) are rare tumors that can occur anywhere in the digestive tract from the stomach to the rectum (last several inches of the large intestine just before the anus). They are most often found in the appendix (small hollow pouch at the beginning of the large intestine).

Tumors growing in the appendix are almost always benign (non-cancerous). They are commonly treated with a simple procedure called an appendectomy (surgical removal of the appendix), but may require a segment of the intestine around the appendix to be removed for more aggressive tumors.

Tumors growing in the ileum (part of the small intestine) are often malignant. These tumors grow slowly and may not cause symptoms right away. As the tumor gets bigger, patients may experience abdominal pain, bloating or bowel obstruction (blocked intestine). Surgery is typically required to remove the affected segment of intestine.

Gastric (stomach) NETs can behave very differently depending on whether they developed as a result of other medical treatments or on their own.

NETs of the colon and rectum are often recognized in routine colonoscopies and can often be managed by endoscopic means.

GI-NETs can be difficult to diagnose early on and are often found during a CT scan or MRI done for other reasons. Not uncommonly, some tumors only cause symptoms once they have spread to other organs.

It is important to know that neuroendocrine tumors can be non-inherited (develop on their own) or inherited (passed along the family tree). Although rare, the most common genetically inherited conditions involving pancreatic NETs include:

●      Multiple endocrine Neoplasia type-1 (MEN1) 

●      Neurofibromatosis type 1 (von Recklinghausen disease), a condition that causes many symptoms, including birthmarks (café-au-lait spots and neurofibromas (a type of nerve tumor) on the skin, as well as increases the risk for for GI-NETs, pancreatic NETs and pheochromocytomas.

●      Von Hippel-Lindau 

At Weill Cornell Medicine, our patients have access to genetic specialists who study tumor’s genetic information to better understand the condition and most effective treatment options. If you have an inherited condition, you will be referred for evaluation by our geneticists for further evaluation and counseling as needed.

Carcinoid Syndrome

NETs can release other hormones called serotonin and kallikrein into the bloodstream. High amounts of hormones in the body can cause serious illnesses.

When patients have symptoms caused by hormones, a rare disease called carcinoid syndrome can occur. These symptoms should prompt a thorough work-up to find the NET.

Symptoms of carcinoid syndrome include:

●      Flushing (sudden redness or discoloration/darkening of face and upper body)

●      Diarrhea

●      Abdominal pain

●      Difficulty breathing

●      Heart issues (chest pain, shortness of breath, easily tired)

Patients with carcinoid syndrome should see a doctor as soon as possible to find the tumor causing the issue and whether the disease has spread.

Comprehensive Diagnostic Evaluation

Weill Cornell Medicine uses the newest, most effective technologies and techniques.

●      Gallium-68 DOTATATE PET-CT scan

●      Traditional FDG PET-CT scan

●      CT scan

●      MRI

If these studies suggest a pancreatic neuroendocrine tumor, a biopsy procedure to confirm the diagnosis may be necessary. At the Endocrine Oncology program, we will coordinate your biopsy with doctors from either gastroenterology or interventional radiology depending on the specific location of your tumor. 

After information about the tumor is obtained, patients meet with their physician to discuss treatment options, risks and benefits. We encourage patients to ask questions. Knowing what to expect during treatment and recovery helps reduce stress and anxiety. Please share any concerns you have about your diagnosis or treatment plan with your doctor.

Treatment and Follow-Up

Treatment at Weill Cornell Medicine is patient centered.

We work with patients to design a treatment plan that meets their individual needs. Care provided by other specialists, including radiologists, pathologists, and social workers, to make it easy for patients to manage multiple appointments.

Many NETs can be removed surgically. Our surgical techniques are minimally invasive. These techniques require small incisions (surgical cuts) or insertion of thin needles to perform. Minimally invasive techniques include:

●      Laparoscopic surgery that uses a thin, lighted tube inserted through a small incision

●      Robotic-assisted surgery that allows easier access to and clearer view of the tumor

●      Tumor-directed ablation that uses heat to destroy tumor cells

Minimally invasive procedures are better tolerated than traditional surgery (often called “open surgery”). Patients have less bleeding during minimally invasive surgery and less pain during recovery. Other benefits for those who choose these procedures include a shorter hospital stay and quick return to daily activities.

Carcinoid tumors that have spread are treated in a variety of ways. These treatments include:

●      Interventional radiology

●      Chemotherapy

●      Directed molecular therapy to block cancer growth and spread

●      Radioisotopes

In some cases, new therapies and imaging technology will be used to improve your outcome. Your doctor will discuss if you are eligible for these therapies to diagnose and treat your NET.

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