Primary Treatment for Solid Pseudopapillary Neoplasm (SPEN) of the Pancreas
Radical surgical resection should be performed for all solid pseudopapillary neoplasms of the pancreas, regardless of size or presentation, as this is the definitive treatment associated with positive long-term outcomes. 1
Understanding SPEN
Solid pseudopapillary neoplasm (SPEN) is a rare pancreatic tumor that:
- Predominantly affects young females
- Has low malignant potential but requires definitive treatment
- Can occur in any part of the pancreas, though most commonly in the tail/body (70%) 2
Surgical Management Algorithm
Step 1: Preoperative Assessment
- Cross-sectional imaging (CT/MRI) to determine tumor location and size
- Rule out metastatic disease
- Assess resectability and relationship to adjacent structures
Step 2: Surgical Approach Based on Location
- Head of pancreas: Pancreatoduodenectomy (Whipple procedure) 2
- Body/tail of pancreas: Distal pancreatectomy (with or without splenectomy) 2
- Central pancreas: Central pancreatectomy (less common) 2
Step 3: Surgical Principles
- Complete tumor removal with negative margins
- Regional lymph node assessment
- Consider minimally invasive approach for appropriate cases, but only by experienced surgeons to avoid tumor rupture 2
Special Considerations
Locally Advanced SPEN
For locally advanced, metastatic, or recurrent SPEN, an aggressive surgical approach with complete resection is still indicated 1. Unlike many other pancreatic malignancies, SPEN has excellent outcomes even with advanced disease when complete resection is achieved.
Malignant SPEN
Approximately 13% of SPEN cases present with malignant features (T4, N1, and/or M1 disease) 3. Even in these cases:
- Complete surgical resection significantly improves survival 3
- Resection should be considered even for stage IV disease 3
- Chemotherapy has not shown benefit for malignant SPEN 3
Outcomes After Surgical Resection
- Excellent prognosis with complete resection
- Low recurrence rates (typically <5%) 4
- Long-term survival rates >95% 5
- Even with capsule invasion or tumor rupture during surgery, recurrence remains rare 2
Pitfalls and Caveats
- Tumor rupture: Care must be taken during laparoscopic resection to avoid tumor rupture, which should only be performed by experienced surgeons 2
- Incomplete resection: R0 resection should always be the goal, as this provides the best long-term outcomes 4
- Surveillance: Despite excellent outcomes, long-term follow-up is recommended as late recurrences can occur 1
- Adjuvant therapy: Unlike pancreatic adenocarcinoma, adjuvant therapy is not routinely recommended for SPEN as there are no studies proving its efficacy 1
The European evidence-based guidelines on pancreatic cystic neoplasms strongly recommend surgical resection for all SPEN (Grade 1B), emphasizing that this approach is internationally accepted and associated with positive long-term outcomes 1.