How to discuss neuroendocrine tumors of the pancreas with patients

Discussing Pancreatic Neuroendocrine Tumors with Patients

When discussing pancreatic neuroendocrine tumors (PanNETs) with patients, begin by explaining that these are rare tumors that behave very differently from pancreatic cancer—they grow more slowly and have significantly better survival rates, with 5-year survival ranging from 60-100% for localized disease, though all PanNETs are considered malignant and require treatment. 1, 2

Initial Conversation Framework

Explain the Diagnosis Clearly

• Clarify that PanNETs are NOT the same as pancreatic cancer (adenocarcinoma): These tumors arise from hormone-producing cells in the pancreas and have a much more favorable prognosis than the common pancreatic cancer patients may have heard about 3, 4

• Distinguish between functioning and nonfunctioning tumors: Functioning tumors (40-55% of cases) produce excess hormones causing specific symptoms like low blood sugar (insulinoma), severe ulcers (gastrinoma), or diarrhea (VIPoma), while nonfunctioning tumors (45-60%) are often discovered incidentally or when they grow large enough to cause symptoms 1, 2

• Explain the grading system in simple terms: The tumor's aggressiveness is determined by how fast the cells are dividing (Ki-67 index)—Grade 1 (≤2%) grows slowest, Grade 2 (3-20%) is intermediate, and Grade 3 (>20%) is most aggressive 1, 5

Discuss Prognosis Honestly but Optimistically

• Provide stage-specific survival data: For localized disease, 5-year survival is 60-100%; for regional spread, 40%; and for metastatic disease, 29% 2

• Emphasize that surgery offers excellent outcomes: When tumors are completely removed, 5-year survival rates reach 80-100% for localized tumors 2

• Acknowledge the possibility of late recurrence: Disease recurrence occurs in 21-42% of patients and can happen many years after initial treatment, which is why long-term follow-up is essential 1

Treatment Discussion

For Localized, Resectable Disease

• Explain that surgery is the primary curative treatment: Complete removal of the tumor with negative margins offers the best chance for cure 1, 2

• Describe the specific surgical approach based on tumor location and size:

  • Small peripheral tumors (<2 cm) may be enucleated (scooped out), preserving most of the pancreas 1, 2
  • Tumors in the body/tail of the pancreas typically require distal pancreatectomy with spleen removal 1, 2
  • Tumors in the head require pancreatoduodenectomy (Whipple procedure) 1
  • Larger tumors (>2 cm) require more extensive resection including lymph nodes due to higher risk of spread 1, 2

• Discuss potential surgical complications: Pancreatic fistula occurs in approximately 22% of cases but can usually be managed conservatively without reoperation 6

For Advanced or Metastatic Disease

• Explain that even with metastases, aggressive treatment can provide long-term survival: Patients who undergo liver resection for metastatic disease can achieve 10-year survival rates of 50.4% 1

• Outline the treatment options for unresectable disease:

  • For asymptomatic patients with low tumor burden and stable disease: Observation with imaging every 3-12 months is appropriate until progression occurs 1
  • For symptomatic patients or those with significant tumor burden: Multiple treatment options exist including targeted therapies, chemotherapy, and hormone-blocking medications 1

• Discuss FDA-approved targeted therapies with proven benefit:

  • Everolimus (10 mg daily) extends progression-free survival to 11.0 months versus 4.6 months with placebo, though side effects include mouth sores, high blood sugar, and rarely pneumonitis 1, 5
  • Sunitinib (37.5 mg daily) extends progression-free survival to 11.4 months versus 5.5 months with placebo 1, 7

• Explain somatostatin analogs for symptom control: Octreotide (100-600 mcg/day) or lanreotide (120 mg every 4 weeks) can control hormone-related symptoms in up to 60% of patients and may slow disease progression 2, 5

Follow-Up and Surveillance

Set Expectations for Long-Term Monitoring

• Emphasize the need for lifelong surveillance: Initial follow-up occurs 3-12 months after surgery, then every 6-12 months indefinitely, as recurrences can occur many years later 1

• Explain what surveillance involves: Regular blood tests for tumor markers (chromogranin A, specific hormones if functioning tumor) and imaging studies (CT or MRI) to detect recurrence early 1, 5

• Clarify that recurrence doesn't mean the end: If disease recurs and is resectable, repeat surgery is recommended and can still provide excellent outcomes 1

Addressing Common Patient Concerns

Genetic Testing Considerations

• Discuss screening for hereditary syndromes: Approximately 10-20% of PanNETs occur in patients with genetic syndromes like MEN-1 or von Hippel-Lindau, which affects family members and surveillance strategies 3, 4

Quality of Life Issues

• For functioning tumors, explain symptom management: Hormone-related symptoms can be controlled with medications before and sometimes after surgery 2

• For insulinomas specifically: Blood sugar levels must be stabilized before surgery with frequent meals and/or medications to prevent dangerous hypoglycemia 2

• For gastrinomas: High-dose proton pump inhibitors control severe acid production and ulcers before definitive treatment 2

• For glucagonomas: Warn about increased blood clot risk and discuss perioperative anticoagulation 1, 2

Managing Uncertainty

• Acknowledge that these are rare tumors: Referral to a specialized center with expertise in neuroendocrine tumors is strongly recommended for optimal outcomes 1

• Be transparent about the need for individualized treatment planning: While guidelines provide a framework, the specific approach depends on tumor grade, stage, location, functional status, and patient factors 1, 2

Critical Pitfalls to Avoid

• Never equate PanNETs with pancreatic adenocarcinoma: This causes unnecessary panic and misrepresents the prognosis 3, 4

• Don't minimize the malignant potential: Even small, low-grade tumors can metastasize on long-term follow-up, so dismissing them as "benign" is dangerous 4

• Avoid recommending observation for tumors >2 cm: These have significant lymph node metastasis risk and require complete resection with lymph node dissection 1, 2

• Don't forget to discontinue proton pump inhibitors at least 1 week before measuring chromogranin A: PPIs cause falsely elevated levels that can mislead diagnosis and monitoring 2