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Neuroendocrine Tumors and When to Operate

Neuroendocrine Tumors and When to Operate

Twenty years ago, physicians discovered neuroendocrine tumors when there was a telltale symptom of hormone secretion, like ulcers in Zollinger-Ellison syndrome or low blood sugar from insulinomas. Today, most patients diagnosed with neuroendocrine tumors are asymptomatic; their tumors are discovered incidentally through CT imaging for something unrelated.

Timothy Pawlik, chief of surgical oncology, and Christopher Wolfgang, chief of hepatobiliary and pancreatic surgery, see the majority of neuroendocrine tumors in the pancreas. Occasionally, they see carcinoids, or neuroendocrine tumors that originate in the small intestine, lungs, appendix or rectum.

Usually, a CT scan enables a diagnosis of a pancreatic neuroendocrine tumor (PNET), but if the surgeons don’t see anything in the pancreas, they order an endoscopy and additional imaging to locate any tumors. “The primary site for a small set of carcinoids can be as tiny as a grain of rice. We don’t see it with imaging or an endoscopy but will find it when we do surgery,” says Pawlik.

An initial clinical assessment may find metastasis to the liver, lungs or other organs, but in most cases, neuroendocrine tumors grow more slowly than other types of cancer. In advanced stages, the tumors can be fatal, but, as Wolfgang says, “surgery for neuroendocrine tumors can reduce the symptoms and extend survival, even at stage 4.”  

When a patient has a PNET smaller than approximately 1 centimeter and is asymptomatic, Pawlik and Wolfgang may monitor the situation before planning surgery. If the tumor is larger than 2 centimeters, they operate to control any spread of disease.

When a PNET is functional and causes symptoms from excess hormone production, or the patient has a carcinoid, Pawlik and Wolfgang operate and use complementary chemotherapy or targeted therapy. “We work side by side with our colleagues in medical oncology, interventional radiology and diagnostic radiology to coordinate the best possible treatment,” says Wolfgang.

If a neuroendocrine tumor is on the smaller side and doesn’t involve blood vessels, the team can employ minimally invasive techniques, such as laparoscopic and robotic operations, at The Johns Hopkins Hospital. They also provide minimally invasive surgery for the liver when needed.

To understand the quality of life for patients after surgery for neuroendocrine tumors, including a liver resection, Pawlik conducted two retrospective studies. The results were published in 2015, and in both studies, says Pawlik, “patients with the worst preoperative quality of life benefited the most from surgery.”

To refer a patient: 443-997-1508

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