Lung neuroendocrine tumors

Neuroendocrine tumors (NETs) are a rare type of pulmonary neoplasm (growth). They make up less than two percent of all lung tumors. These tumors can develop in the trachea (windpipe), bronchi (airways leading into the lungs), or bronchioles (small airway branches).

Cases of these uncommon tumors appear to be increasing, but researchers aren’t sure why. Many lung NETs are noncancerous (benign). But for more than a quarter of people diagnosed, the disease has grown into nearby tissue or spread farther (metastasized).

Neuroendocrine tumors in the lungs require a team that knows what to do—not only for diagnosis, but also for treatment planning. Penn Medicine’s neuroendocrine tumor specialists have experience with all forms of these rare tumors. When tumor removal is possible, our surgeons look to spare lung tissue. And if you need more, we offer a range of options, including some not widely available.

Neuroendocrine lung tumors may not cause symptoms until they grow larger or spread. When they do occur, many symptoms can resemble asthma and may include:

• A cough that won’t go away
• Coughing up small amounts of blood
• Feeling very tired (fatigue)
• Frequent chest infections, like pneumonia
• 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.

We can help you understand your history through genetic testing for neuroendocrine tumors and genetic counseling services.

Penn Medicine’s neuroendocrine tumor specialists have deep experience with lung NETs and their unique challenges. We offer you many options, whether you need a first evaluation or a second opinion.

When you come to us for care, 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 Medicine 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.