Lung neuroendocrine tumors

Neuroendocrine tumors in the lungs require a team that knows what to do — not only for diagnosis, but also for treatment planning. The Penn Neuroendocrine Tumor Program has experience with all forms of these rare tumors, to help with whatever you need.

When tumor removal is possible, our surgeons look to spare lung tissue. And when you need more, we offer a range of options, including some not widely available. We carefully consider all recommendations — we care about your life back home, not just the disease in front of us.

Neuroendocrine lung tumors may not cause symptoms until they grow larger or spread. When they do occur, many symptoms are asthma-like. They include:

• Cough that you can’t get rid of, though it may vary in intensity throughout the day
• Coughing up small amounts of blood
• Fatigue
• Frequent chest infections (such as pneumonia), or an infection that resists treatment
• Pain in the chest or shoulders
• Shortness of breath

Lung NET symptoms tied to hormones

Some neuroendocrine tumors release hormones, which can cause their own sets of symptoms. While only 10 percent of lung NETs cause this complication, it’s still something doctors need to watch carefully.

Most hormone spikes from a lung NET involve adrenocorticotropic hormone (ACTH). High ACTH can cause a condition called Cushing’s syndrome, marked by high blood pressure, low potassium and weight gain. We watch for these symptoms and treat them as needed.

Less frequently, people with a lung NET experience carcinoid syndrome, from the tumor releasing serotonin or related hormones. They may experience diarrhea, wheezing and flushing (temporary redness in the face.) Our gastrointestinal symptom management for endocrine tumors can help.

We often don’t know why someone develops a neuroendocrine lung tumor. But scientists have identified a few risk factors, such as long-term smoking and asbestos exposure.

Very rarely, people inherit a particular genetic mutation, or variant, tied to lung NETs. About 5 percent of people with the genetic condition MEN1 develop a lung NET. MEN1 can also lead to tumors in the parathyroid gland, pituitary gland and pancreas.

Learn more about our genetic testing for neuroendocrine tumors and genetic counseling services.

Many lung NETs get discovered through a chest X-ray or CT for symptoms tied to the lungs, cancer screening or other medical reasons. These scans can show nodules (small, unusual growths). Doctors may watch a nodule at first to see if it grows; not all nodules are cancerous.

If a nodule is suspicious, doctors will perform a biopsy to determine what it is. For nodules that are reachable through the windpipe or blocking the windpipe, doctors use a bronchoscope (camera and thin tube) to access the growth. Deeper in the lung, they may take a tissue sample (biopsy) with a needle or remove the nodule entirely with surgery.

Generally, lab analysis can reveal if the growth is a tumor and, if so, what kind. For lung carcinoids, pathologists try to tell if the tumor is typical or atypical.

Lung NET evaluation at Penn

Not all lung NETs get diagnosed when they’re small or slow growing. At Penn, our team has experience evaluating the full range of cases. We offer:

• Standard imaging: In addition to bronchoscopy and CT, lung function tests can signal a possible lung NET. Our team may also use MRI to confirm a lung NET diagnosis and determine the extent of the disease.
• Hormone testing: We run blood and urine tests to look for signs of hormone release related to lung NETs.
• Review of previous evidence: If a tumor has already been biopsied elsewhere, our specialized pathologists review the results, providing the best chance at accurate interpretation. They can often determine whether a carcinoid is typical or atypical. But even if they can’t give that definitive label, our team knows to watch for other clues.
• Possible biopsy: Biopsy can come with some risks, so we weigh the benefits and possible downsides before we perform one. Some tumors may also prove hard to reach for biopsy. If we don’t have a biopsy to determine tumor type, our expert tumor board uses other clues to plan care.
• Functional imaging: Other tools include imaging with nuclear medicine, a newer approach. A small, injectable dose of radioactive material binds to various substances taken in by tumor cells. This type of imaging can distinguish between carcinoids and poorly differentiated tumors. It can also show how far the disease may have spread and what treatment may work.

At the Penn Neuroendocrine Tumor Program, we have deep experience with lung NETs and their unique challenges. We’re able to offer you as many options available: whatever you need, whether that’s an initial evaluation, a second opinion or further treatment.

When you come to our Neuroendocrine Tumor Program, you’ll find:

• Expertise: Specialists across our teams are used to working with lung NETS. For example, our pathologist focuses solely on lung NETs and other lung tumors. This background helps when determining the type of lung NET.
• Collaboration: When cases require more involved treatment, teamwork plays a key role for timing and therapy choice. Many of our providers regularly attend the weekly neuroendocrine tumor board. They’re joined as needed by lung specialists including thoracic oncologists, thoracic surgeons and pulmonologists. Cases may also go to the lung tumor board.
• Options: Sophisticated surgeries at Penn can preserve more lung tissue, protecting lung function. Our surgeons also consider minimally invasive approaches when possible. For disease that has grown or spread, we offer options not widely available, including nuclear medicine for lung carcinoids.
• Support: From the moment you contact us, our nurse navigator helps answer your questions, schedule your appointments and attend to your other needs. We make your experience as easy and stress-free as possible. Once your care starts, we offer a range of additional support for neuroendocrine tumors.