Introduction

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas represents around 80 % of incidental-discovered cysts and one of the most frequent causes of access for pancreatic clinical evaluation. Fukuoka guidelines (2017) have been the clinical reference point until the recently published Kyoto guidelines (2024) that bring out various new key points including “stop surveillance” concept as an appropriate approach in specific patients.

Aims & Methods

The aim of the study is to observe clinical validity of new recommendations of Kyoto guidelines, particularly in patients with unchanged BD-IPMN ≤ 20 mm after 5 years of surveillance. This is an observational retrospective single-center study. Patients were evaluated in our Digestive Disease Unit from Jan 2018 to Jan 2024 and they were handled according to Fukuoka guidelines.

Results

A total of 163 patients (M: F=56:107) with a median age of 70 y/o (44-92). BD-IPMN were 152 (93.5%), located in the pancreatic head (70, 46%), body (63, 41.4%) and tail (19, 12.5%). During surveillance, 29 (18%) and 3 (2.2%) patients developed, respectively, ‘worrisome features’ (WF) and ‘high-risk stigmata’ (HRS). Overall, 39 (23.9%) patients underwent surveillance for ≥ 5 years. Particularly, 20 (51.3%) of these patients had BD-IPMN ≤ 20 mm unchanged during a median time follow-up of 77 months (60-144): median IPMN dimension was 12 mm (7-20) and median age was 71 y/o (59-87). They performed a median of 7 (3-10) exams (RMN, CT, EUS) with no evidence of WF or HRS beyond 5 years of follow-up. However, in IPMN ≥ 20 mm with surveillance ≥ 5 years, 14 (35.8%) and 2 (5.1%) patients developed, respectively, WF and HRS at a median of 22 months (6-120) from diagnosis with consequent different timing of follow-up or surgical strategy.

Conclusion

Based on our experience, no one patient with BD-IPMN ≤ 20 mm during FU ≥ 5 years developed any WF or HRS. This confirmed the latest points of view of Kyoto guidelines, providing patients the possibility to stop surveillance. This could have an important role in the patient’s psychological aspects and in decreasing request overload of radiological or endoscopic exams.

References

1. Tanaka M et al. Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas. Pancreatology. 2017 Sep-Oct;17(5):738-753.
2. Ohtsuka T et al. International evidence-based Kyoto guidelines for the management of intraductal papillary mucinous neoplasm of the pancreas. Pancreatology. 2024 Mar;24(2):255-270

Clinical Case Summary

Case report: We report a case of a 51-year-old patient who initially presented in 2014 with thrombocytopenia, and splenomegaly. Liver parameters were within the normal range. Comprehensive diagnostic evaluations, including CT scans and liver biopsy, excluded cirrhosis, vascular occlusions, bypass circuits and other potential etiologies such as toxic, autoimmune, or infectious causes. Additional investigations, including bone marrow and spleen puncture as well as genetic tests did not reveal any hematological or neoplastic diseases.
At the age of 66, the patient suffered an episode of variceal hemorrhage due to grade IV oesophageal varices. Subsequently, after referral to our clinic in March 2020, a transjugular portosystemic shunt (TIPSS) was placed for persistent grade III varices (Figure 1), reducing the varices to grade I (2024). Despite successful management of varices, the patient developed mild hepatic encephalopathy, managed with lactulose and rifaximin.
Biopsy revealed nodular regenerative hyperplasia (Figure 2). We diagnosed idiopathic non-cirrhotic portal hypertension (INCPH), an infrequent yet significant clinical entity characterized by intrahepatic portal hypertension without cirrhosis or other liver diseases. Management challenges included the patient's intolerance to non-selective beta-blockers due to sinus bradycardia and ongoing low-grade hepatic encephalopathy, which precluded further TIPSS modification. Future management may include splenic vein embolization should varices recur.
Conclusion: The diagnosis of INCPH necessitates evident portal hypertension, no cirrhosis or advanced fibrosis, and no hepatic or portal vein thrombosis on imaging. Due to limited data, management typically mirrors that of cirrhosis-induced portal hypertension. This case highlights the diagnostic and management challenges of INCPH, stressing the importance of comprehensive assessments to rule out other causes and customize treatment for complications.