From the Guidelines
For localized duodenal neuroendocrine tumors (dNETs), endoscopic resection is the recommended first-line treatment if feasible, as stated in the 2015 guidelines 1. Management of dNETs requires a multidisciplinary approach based on tumor size, grade, and presence of metastases.
- For small, localized, low-grade dNETs, endoscopic resection is typically the first-line treatment, with endoscopic mucosal resection or endoscopic submucosal dissection being preferred techniques.
- Larger tumors or those with muscularis propria invasion generally require surgical intervention, ranging from local excision to pancreaticoduodenectomy (Whipple procedure).
- For metastatic disease, somatostatin analogs like octreotide LAR or lanreotide are recommended to control symptoms and slow tumor growth, as supported by the guidelines 1.
- Additional options for advanced disease include peptide receptor radionuclide therapy (PRRT) with lutetium-177 dotatate, everolimus, or sunitinib.
- Functional tumors producing hormones require symptom management; for instance, gastrinomas benefit from proton pump inhibitors like pantoprazole.
- Long-term surveillance is essential, with endoscopy, cross-sectional imaging, and chromogranin A measurements typically performed every 6-12 months, adjusted based on tumor grade and stage, as recommended in the guidelines 1. The most recent and highest quality study, published in 2015 1, provides the basis for these recommendations, prioritizing morbidity, mortality, and quality of life as the primary outcomes.
From the Research
DNET Tumor Management
- DNETs are a common cause of chronic drug-resistant epilepsy and are known for their favorable surgical outcomes 2.
- The seizure recurrence-free rate is not as favorable if tumorous nodules are present near the main mass, known as satellite lesions (SLs) 2.
- Studies have shown that complete tumor and EZ removal, short epilepsy duration, and lack of cortico-subcortical damage are the main prognostic factors for favorable outcome 3.
- Surgical resection may be restricted to the tumor in type 1 MRI but should be more extensive in other MRI subtypes, especially in type 3 MRI 3.
- Endoscopic resection has been shown to have similar survival outcomes to surgical resection across DNET sizes 4, 5.
- In eloquent areas, surgery for DNETs provides acceptable results regarding functional risks, with complete tumor resection being crucial for long-term favorable outcome 6.
Treatment Options
- Surgical resection is often recommended for larger DNETs (> 2 cm) 4, 5.
- Endoscopic mucosal resection (EMR) is generally used for smaller lesions (< 1-2 cm) 4, 5.
- The choice of treatment depends on various factors, including tumor size, age, and stage 4, 5.
- Studies have shown that there is no difference in progression-free or overall survival between surgical resection and EMR for DNETs 4, 5.
Prognostic Factors
- Complete tumor and EZ removal is a key prognostic factor for favorable outcome 3.
- Short epilepsy duration and lack of cortico-subcortical damage are also important prognostic factors 3.
- Tumor size, age, and stage are also significant factors in determining treatment outcomes 4, 5.
- Satellite lesions (SLs) can affect seizure recurrence rates, with patients who initially presented with SLs having a higher seizure recurrence rate 2.