Treatment for Neuroendocrine Pancreas Tumor in Body
Surgery is the primary treatment for pancreatic neuroendocrine tumors located in the body of the pancreas, with distal pancreatectomy (with or without splenectomy) being the standard approach, and complete resection with negative margins and regional lymph node dissection is essential for tumors >2 cm. 1, 2
Surgical Management Based on Tumor Size and Characteristics
For Nonfunctioning Tumors >2 cm
- Perform distal pancreatectomy with splenectomy and regional lymph node dissection for tumors in the body/tail of the pancreas 1, 2
- Complete resection with negative margins is mandatory, as these tumors carry significant risk of lymph node metastases 2
- Spleen preservation may be considered for smaller tumors not involving splenic vessels 1
For Nonfunctioning Tumors 1-2 cm
- Resection with lymph node dissection is recommended due to the real but small risk of lymph node metastases 2
- The surgical approach should still be distal pancreatectomy for body/tail location 1
For Functioning Tumors (Insulinomas, Glucagonomas, VIPomas)
- Distal pancreatectomy with peripancreatic lymph node dissection and splenectomy is the standard approach for tumors in the body/tail 1
- For insulinomas specifically, stabilize glucose levels preoperatively with diet and/or medication before surgery 2, 3
- For glucagonomas, consider perioperative anticoagulation due to increased risk of pulmonary emboli 1
Management of Metastatic or Unresectable Disease
Surgical Cytoreduction
- Even with metastatic disease, surgical debulking plays an important role in reducing tumor mass and can be performed before or with medical treatment 1
- Surgical resection of both primary tumor and liver metastases should be considered when feasible, performed in staged or synchronous fashion 1
- When performing staged procedures, hepatectomy should precede pancreatic resection to reduce risk of perihepatic sepsis from contaminated biliary tree 1
Medical Management for Advanced Disease
First-line therapy:
- Somatostatin analogues (octreotide 100-600 mcg/day in 2-4 divided doses, or lanreotide 120 mg every 4 weeks subcutaneously) for symptom control and disease stabilization 1, 4
- These agents control hormone-related symptoms in up to 60% of patients 1
Targeted therapies:
- Everolimus and sunitinib are FDA-approved for advanced pancreatic NETs based on phase III trials showing improved progression-free survival 1
- These should be considered for progressive disease on somatostatin analogues 1
Chemotherapy:
- Reserve cytotoxic chemotherapy for tumors with higher proliferative activity (higher Ki-67 index) 1
- Options include streptozocin + 5-FU ± doxorubicin, or temozolomide alone or in combination with capecitabine 1
- Temozolomide-based regimens show promise with 70% response rates in some series 1
Local Ablative Therapies
- Radiofrequency ablation, laser therapy, and embolization of liver metastases are important cytoreductive procedures 1
- Peptide receptor radionuclide therapy (PRRT) should be considered for patients with high uptake on Octreoscan scintigraphy 1
Follow-Up Protocol
Post-Curative Surgery
- Follow every 3-6 months for >5 years with biochemical markers and imaging 1
- CT or MRI every 6 months 1
- Measure chromogranin A (discontinue PPIs for at least 1 week before testing) 2
- Disease recurrence occurs in 21-42% of patients and can happen after many years 1
During Medical Treatment
- Follow at 3-month intervals during cytotoxic or biological therapy 1
- Include specific biochemical markers based on tumor functionality 1
Critical Pitfalls to Avoid
Octreotide use in insulinomas:
- Do NOT use octreotide in insulinoma patients without confirming octreotide-avidity, as it can precipitously worsen hypoglycemia and cause fatal complications 3
- Insulinomas are less consistently octreotide-avid than other pancreatic NETs 2
Inadequate lymph node dissection:
- Failure to perform adequate lymph node dissection leads to recurrence, as pancreatic NETs frequently have lymph node metastases even when not clinically apparent 3
Liver-directed therapy timing:
- Liver-directed therapies following pancreatoduodenectomy carry increased risk for perihepatic sepsis and liver abscess 3
Incomplete cytoreduction:
- Planned incomplete (R2) resection in asymptomatic/nonfunctional disease remains controversial and should be avoided unless clear symptomatic benefit expected 3