From the Guidelines
The patient should undergo a dedicated MRI liver protocol with Eovist contrast to fully characterize the liver lesions, and consideration should be given to surgical resection for the possible local recurrence at the pancreatic resection margin, as indicated by the arterially enhancing focus near the resection margin in the distal pancreatic body, which may correlate with subtle uptake seen on a previous PET/CT 1.
Key Findings
- A subcentimeter arterially enhancing focus in the near resection margin in the distal pancreatic body is concerning for neuroendocrine tumor and may correlate with very subtle radiotracer uptake on the prior OSI PET/CT.
- Two hyperenhancing lesions in the right hepatic lobe consistent with metastases are stable.
- Nonspecific adjacent enhancing nodularity in the inferior right liver could be perfusional or represent another small metastasis.
- Splenomegaly and submucosal gastric varices due to chronic splenic vein occlusion.
- No evidence of thoracic metastasis.
- Resolution of the left pleural effusion.
Management
- Dedicated MRI liver protocol with Eovist for full characterization of the liver lesions.
- Consideration for surgical resection for the possible local recurrence at the pancreatic resection margin, as the current evidence suggests that resection for larger or malignant-appearing tumors should include total removal of the tumor with negative margins and regional lymph nodes 1.
- Surveillance with follow-up 3 to 12 months after resection, or earlier if the patient presents with symptoms, and every 6 to 12 months thereafter with an H&P, appropriate tumor markers, and imaging studies such as CT/MRI as clinically indicated 1.
Prognosis
- Disease recurrence has been observed in 21% to 42% of patients with pancreatic neuroendocrine tumors and can occur after many years 1.
- The patient's prognosis will depend on the extent of the disease, the presence of metastases, and the effectiveness of treatment.
From the Research
Neuroendocrine Tumors
- Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms with an annual incidence of ~35 cases per 100,000 people in the United States 2.
- The updated World Health Organization (WHO) classification system of gastroenteropancreatic (GEP)-NETs categorizes these tumors according to site of origin, clinical syndrome, and degree of differentiation 2.
- Well-differentiated NETs arising from the gastrointestinal tract or lungs (formerly known as carcinoid tumors) are often indolent and slow-growing, while poorly differentiated neuroendocrine carcinomas (NECs) are aggressive and have a poor prognosis 2.
Somatostatin Analogues
- Somatostatin analogues (SSAs) have been approved for the control of clinical syndrome associated with functioning NETs and for tumor growth control in advanced low/intermediate grade NETs 3, 2.
- Long-acting SSAs, such as octreotide and lanreotide, have been shown to have antiproliferative effects and inhibit tumor growth via the somatostatin receptor 2 (SSTR2) 4, 5, 6.
- The PROMID and CLARINET trials demonstrated a statistically significant prolongation of time to progression/progression-free survival (TTP/PFS) upon SSA treatment, compared to placebo 6.
Treatment of Neuroendocrine Tumors
- Somatostatin analogue therapy has been a mainstay of antisecretory therapy in functioning neuroendocrine tumors, which cause various clinical symptoms depending on hormonal hypersecretion 6.
- The combination of SSA with peptide receptor radionuclide therapy (PRRT) in small intestinal NETs has proven efficacy in the phase 3 neuroendocrine tumours therapy (NETTER 1) trial 6.
- PRRT is currently being tested for enteropancreatic NETs versus everolimus in the COMPETE trial, and the potential of SSTR-antagonists in PRRT is now being evaluated in early phase I/II clinical trials 6.