Types of Neuroendocrine Tumors

While all types of neuroendocrine tumors (NETs) share basic similarities — they start in the same family of cells — their characteristics vary. These uncommon tumors can develop anywhere in the body, but can be found most often in the pancreas, bowel, lungs or adrenal glands.

Some types of neuroendocrine tumors release hormones (functioning tumors) and some don’t (nonfunctioning tumors). Some remain benign (noncancerous), while others turn malignant (cancerous). And while they’re typically slow-growing, even when they metastasize (spread), there are exceptions. Every case is unique — perhaps more so than other tumors.

Experience With the Full Range of Neuroendocrine Tumor Types

While NETs remain uncommon — about 12,000 new cases annually and around 170,000 people living with the disease — diagnoses have been rising. In part, the trend comes from the increase in detailed scans given for other medical reasons. It’s also tied to the emergence of genetic testing for tumors that run in families. But we may not know all the reasons yet.

At Penn Medicine, we’re prepared to help, regardless of the circumstances. We provide the individualized care required when treating all types of NETs. We know the peculiarities of these tumors and the unique challenges they can pose. We have years of experience with a full variety of neuroendocrine tumor types, including the most unusual and complex:

Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs)

GEP-NETs are neuroendocrine tumors in the gastrointestinal (GI) tract or pancreas. Historically, these tumors have been grouped together because they receive similar treatments and share behaviors, such as frequently spreading to the liver. There are differences, however — differences we appreciate.

The greatest number of NETs develop in the GI tract. These tumors are also known as carcinoids, and some release hormones. Pancreatic NETs are less common, but a greater share emit hormones.

Learn more about:

Carcinoid Tumors in the Lungs

The lungs and airways are another common location for NETs. Like GI NETs, lung NETS are also known as carcinoids, and some release hormones.

Learn more about lung neuroendocrine tumors.

Pheochromocytomas and Paragangliomas (Pheo-Paras)

Among the five most common NET types, pheochromocytomas and paragangliomas — similar tumors collectively called pheo-paras — develop the least often. Pheochromocytomas form in the adrenal glands. Paragangliomas start in other parts of the belly, as well as the pelvis, chest, neck and head. Both frequently release hormones.

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Metastatic Neuroendocrine Tumors

When cancerous neuroendocrine tumors spread, a common location is the liver. Other areas depend on the NET involved and may include lymph nodes, bones, lungs, adrenal glands and the brain.

Many metastatic NETs grow slowly and are treatable. People can live for many years, with an estimated 100,000 people in the U.S. living with a metastatic NET at a given moment.

Learn more about one treatment option, liver-directed therapy for NETs.

Well-Differentiated Neuroendocrine Tumors Vs. Neuroendocrine Carcinoma

Most neuroendocrine tumors are well-differentiated, meaning their cells look more or less like normal cells. These tumors are more treatable, with a wider range of options. They tend to grow more slowly than more common tumors found in the same organs or glands.

Occasionally, NETs are poorly differentiated, with distorted cells that can lose some of their neuroendocrine features. These tumors tend to act aggressively, like more common cancers. Examples include:

  • Large cell neuroendocrine carcinoma (LCNEC) in the lungs
  • Neuroendocrine carcinoma (NEC) in the pancreas or GI tract
  • Small cell lung carcinoma (SCLC)

We can still treat these tumors. But when surgery is not enough, we often resort to chemotherapy. Learn more about drug therapy for NETs.

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Gastrointestinal Neuroendocrine Tumors

Gastrointestinal neuroendocrine tumors need a seasoned team and a range of options. Our specialists are known for surgery, liver care and new therapies.

Lung Neuroendocrine Tumors

With a lung neuroendocrine tumor, you need a team that can make an accurate diagnosis and respond with effective therapy at every step of the journey.

Pancreatic Neuroendocrine Tumors

Pancreatic neuroendocrine tumors come in a range of forms. We provide expertise and advanced options, including for liver spread and hormone release.

Paraganglioma

Paragangliomas are challenging to diagnose and treat. Our nationally recognized team provides the needed range of expertise and options not widely offered.

Pheochromocytoma

Pheochromocytomas require experienced care, given their rarity and complexity. Our nationally recognized team features experts in these adrenal tumors.