For Intraductal Papillary Mucinous Neoplasm (IPMN), who should I refer the patient to?

Referral Recommendations for Intraductal Papillary Mucinous Neoplasm (IPMN)

Patients with IPMN should be referred to a pancreatic surgeon with expertise in pancreatic resection, as surgical management is the definitive treatment for high-risk IPMNs and those with invasive components. 1

Initial Evaluation and Referral Pathway

• IPMNs require thorough evaluation to determine malignant potential, which guides the need for surgical intervention versus surveillance 1
• The diagnostic investigation for an IPMN-associated invasive cancer should follow the same algorithm used for pancreatic cancer 1
• Pathologic evaluation is crucial to rule out invasive carcinoma, requiring extensive sampling of the lesion 1, 2

Indications for Surgical Referral

• Main duct IPMN (MD-IPMN) carries a significant risk of malignancy and should be referred for surgical evaluation regardless of symptoms 3
• Branch duct IPMN (BD-IPMN) with high-risk features should be referred for surgical evaluation 4
• High-risk features that warrant surgical referral include:
  • Obstructive jaundice in a patient with a cystic lesion in the pancreatic head 4
  • Mass lesion >30 mm on radiological imaging 4
  • Enhanced solid component within the cyst 4
  • Main pancreatic duct size ≥10 mm 4
• "Worrisome features" that should prompt consideration for surgical referral include:
  • Main pancreatic duct size 5-9 mm 4
  • Cyst size <3 cm with concerning features 4

Surgical Considerations

• The surgical strategy should be based on the type of surgical resection required, patient's age, comorbidities, and patient preference 1
• Targeted pancreatic resection with frozen-section analysis of margins is recommended when surgery is indicated 3
• Pancreatoduodenectomy or distal pancreatectomy is appropriate for the majority of cases 3
• Total pancreatectomy is necessary in approximately 10% of patients with diffuse disease 3

Post-Surgical Follow-up

• Lifelong surveillance is recommended following resection of an IPMN as long as the patient is fit and willing to undergo surgery if indicated 1
• Patients with evidence of an IPMN-associated invasive carcinoma should be followed up in the same manner as those with resected pancreatic cancer 1
• IPMN with high-grade dysplasia or MD-IPMN should have close follow-up every 6 months for the first 2 years, followed by yearly surveillance 1
• Follow-up imaging using MRI or EUS is recommended 1

Special Considerations

• The 5-year survival of patients after surgical resection for noninvasive IPMN is 77-100%, while for those with invasive carcinoma, it is significantly lower at 27-60% 4
• Different histological subtypes (gastric, intestinal, pancreatobiliary, oncocytic) have different prognostic implications and should be considered in management decisions 2
• Patients with a family history of pancreatic cancer who have an IPMN should be managed similarly to those without a family history 1
• Patients who have undergone organ transplantation and have an IPMN should be managed the same as non-transplanted patients 1

Pitfalls to Avoid

• Avoid using the term "malignant IPMN"; instead, document the highest grade of dysplasia in the non-invasive component separately from any invasive component 1, 2
• The term "minimally invasive" should be avoided; instead, document invasion size with appropriate staging 1
• Frozen section analysis should be performed highly selectively, with appreciation of its shortcomings in detecting discontinuous (skip) lesions, which occur in 6-42% of cases 1, 2