Drug Therapy for Neuroendocrine Tumors

Most people with a neuroendocrine tumor (NET) require medication at some point. These medications are systemic, meaning they treat disease across the body. At Penn Medicine, we offer the full range of options.

The specialists on our team have a deep understanding of when to give these treatments, and which ones to recommend. They also think about how each medication may fit into your full course of care and your life. We always consider side effects, in both the short term and the long. We may suggest drug therapy for:

Our team also works to develop new NET drug therapy options and new combinations. Learn more about neuroendocrine tumor clinical trials.

Somatostatin Analogs (SSAs): Lanreotide and Octreotide

SSAs often serve as the backbone of drug therapy for NETs:

  • Treatment goals: SSAs can provide relief from symptoms related to NET hormone release and stop or slow tumor growth. They work better for tumors with cells that are well-differentiated, meaning they look more or less like normal cells.
  • How it works: Many neuroendocrine tumors have receptors for the hormone somatostatin on their cells. The body uses somatostatin to keep the production of other hormones in check, so the receptors can stop NETs from releasing hormones. We can’t give somatostatin as a treatment because it breaks down too quickly. Instead, we use analogs, or lab-made versions. Lanreotide and octreotide remain the most widely used SSAs.
  • How it’s given: Doctors give SSAs as an injection, usually every four weeks. Faster-acting versions can provide prompt relief from a set of hormone-related symptoms called carcinoid syndrome. Many people stay on longer-acting SSAs for years, even when adding other therapies.
  • Which NETs it treats: Most people with GI or pancreatic NETs start with SSAs if surgeons can’t completely remove tumors. They are also an option for lung NETs and some pheo-paras that have spread.
  • Side effects: Many people tolerate SSAs well. The main side effects are mild gastrointestinal symptoms: diarrhea, steatorrhea (oily stools) and dysmotility (changes in digestive speed and processing). We also watch for less common side effects so we can treat them quickly:
    • Bradycardia (slow heartbeat), in people with heart conditions
    • Gallstones (we may recommend gallbladder removal as a preventive step if other surgery is scheduled)
    • Reduced pancreatic enzymes
    • Interference with blood sugar

CapTem Chemo and Other Neuroendocrine Tumor Chemotherapy

We may recommend chemotherapy when tumors have spread more widely:

  • Treatment goals: Chemotherapy can reduce the size of tumors. It typically works most effectively on more aggressive tumors, those with poorly differentiated cells. But a newer combination, capecitabine and temozolomide (CapTem), can provide some control of well-differentiated pancreatic tumors.
  • How it works: Chemotherapy most strongly affects cells that are rapidly dividing and multiplying. That behavior helps destroy cancerous cells but can also harm healthy cells.
  • How it’s given: Traditional chemotherapy is given through intravenous (IV) lines. The CapTem combination comes in pills.
  • NETs it treats: We most often use CapTem for lung and pancreatic NETs. We may also consider it for GI NETs. (We often use temozolomide alone for pheo-paras.) Standard chemo treats more aggressive NETs — small cell lung carcinoma (SCLC), large cell neuroendocrine carcinoma (LCNEC), neuroendocrine carcinoma (NEC) in the pancreas or GI tract and some metastatic pheo-paras.
  • Side effects: Standard chemotherapy can cause a range of side effects, including nausea, vomiting, fatigue, hair loss and lower levels of blood cells. CapTem can cause similar side effects, but to a lesser degree — it’s often gentler. Like other chemotherapy, temozolomide can still damage bone marrow, so we’re careful with the total number of doses. Capecitabine often causes diarrhea, which we’re usually able to manage with over-the-counter antidiarrheals.

Targeted Therapy for NETs

Targeted therapy provides a newer option for treating NETs:

  • Treatment goals: Targeted therapies can slow or stop tumor growth when cancer has spread to nearby lymph nodes or tissue or gone farther. They may work better when the total amount of disease remains limited. When we need to shrink tumors, we may recommend chemotherapy instead.
  • How it works: Targeted therapies act on various features of cancerous cells. For NETs, one option targets a protein driving NET cell division and growth. Another targets a substance the cells use to create new blood vessels.
  • How it’s given: We give targeted therapy as pills.
  • NETs it treats: Doctors have two approved targeted therapies for well-differentiated NETs. Afinitor (everolimus) treats GI and lung NETs that don’t release hormones, and many pancreatic NETs. Another targeted therapy, Sutent (sunitinib), provides a second option for pancreatic NETs. We use the drugs when tumors don’t respond to therapies designed for somatostatin receptors. Other options may include targeted therapy approved for other cancers or new drugs in clinical trials.
  • Side effects: People typically tolerate targeted therapy fairly well. Potential side effects can drive the choice of which drug to use.

Why Choose the Abramson Cancer Center for NET Drug Therapy?

Given the range of NETs and their complexity, you want a team that knows when to turn to drug therapy, and to which options. When you come to our program, you’ll find:

  • Experience and expertise: Our specialists have spent years treating all kinds of NETs. Many sit on groups that create national treatment guidelines. They draw not only on existing evidence, but also on their deep experience when making recommendations. Learn more about our neuroendocrine tumor team.
  • Collaboration: We bring together a wide range of specialists, including oncologists specializing in particular NETs, endocrinologists working with hormones, geneticists focused on inherited cases and many other experts. Team members meet at a weekly tumor board to discuss cases and create personalized recommendations.
  • Innovation: We run a wide range of neuroendocrine tumor clinical trials and research, improving options and creating new ones.
  • Support: A nurse navigator or nurse coordinator arranges your appointment and is always ready to help with any need. We provide gastrointestinal management for NET symptoms and side effects, as well as a wide range of other NET support.

Request an Appointment

To make an appointment, please call 800-789-7366 or request a callback.