Introduction

Solid-pseudopapillary neoplasm (SPN) is a rare tumor of low malignancy that occurs predominantly in young females. Imaging of SPN is often described as a well-defined and heterogeneous tumor with calcifications or cysts, otherwise small size SPN often shows a homogeneously solid tumor. However, the differential diagnosis between SPN and other cystic tumors is often difficult due to the variety of imaging findings. In this report, we describe the clinical features and preoperative diagnosis of SPN we experienced at our hospital.

Aims & Methods

We studied 18 cases of SPN that were pathologically diagnosed by surgical resection at our hospital between 1997 and 2022.

Results

There were 12 female and 6 male patients, with a median age of 39.5 years (range: 9-67) and a median tumor size of 23 mm (range: 9-66). The tumor location was in the pancreatic head in 4 cases, body in 6 cases, and tail in 8 cases. The median follow-up period was 89.5 months (range: 8-265), and recurrence-free survival was achieved in all cases during the follow-up period. The tumors were incidentally detected in 15 cases during health check-ups (mainly by ultrasonography) or other medical examinations, while the other 3 cases were detected due to subjective symptoms that were thought to be caused by SPN. SPN could be diagnosed by contrast-enhanced CT in 10 of 18 cases (56%) and by EUS in 7 of 12 cases (58%). Contrast-enhanced CT showed enhancing mass in 10 cases, cystic component in 9 cases (50%), and calcification in 8 cases (44%). Among the 8 cases in which SPN could not be diagnosed by contrast-enhanced CT, EUS was performed in 7 cases, and we could diagnose SPN in 2 cases. Both cases had small lesions of less than 13 mm without cystic components, which made the differentiation from other tumors difficult on contrast-enhanced CT, however EUS showed well-circumscribed, well-defined, and homogeneously hypoechogenic tumors. MRI was performed in 12 cases, and could diagnose SPN in 5 cases, but MRI did not improve the diagnostic accuracy compared to contrast-enhanced CT. The diagnostic accuracy of EUS-guided fine needle aspiration (EUS-FNA) was 89% (8/9 cases). There were 5 cases in which SPN could not be diagnosed preoperatively. Among them, 3 cases had small tumor sizes (12-16 mm), and 4 cases lacked calcification, making it difficult to differentiate them from neuroendocrine tumors or solid-cystic neoplasms.

Conclusion

Most cases of SPN are detected incidentally, and regular abdominal ultrasonography during medical check-ups is important for early detection. Diagnosis can be more difficult in cases with small tumor diameter, and no cystic component or calcification. However adding EUS to contrast-enhanced CT imaging may improve accuracy. Furthermore, EUS-FNA contributes to preoperative diagnosis with a high diagnostic accuracy.

Introduction

Complete excision of upper gastrointestinal sub-epithelial lesions (SELs) eliminates diagnostic uncertainty, obviates the need for surveillance and may be necessary for definitive diagnosis and management. Current guidelines lack precision and cohesion, and surgery is associated with significant morbidity. Herein, we describe and report on the outcomes of our SEL algorithm for endoscopic (SAFE) resection.

Aims & Methods

SELs were enrolled prospectively over 115 months until March 2023. All underwent axial (CT) imaging and endoscopic-ultrasonography (EUS) to exclude a large exophytic component or invasion into local structures, and assess for muscularis propria involvement.

Results

106 SELs were resected (mean age 60.6±13.4, 51.9% male; 41 oesophageal, 65 gastric). Oesophageal SELs underwent ESD (n=22) or STER if MP involvement was suspected (n=19). Gastric SELs underwent STER (n=6 at cardia), ESD (n=47) or exposing-EFTR (n=12). Technical success was 97.6% and 92.3% respectively. Five (7.7%) were completed laparoscopically due to deep and broad full-thickness involvement. Five (7.7%) required laparoscopic gastrotomy and surgical retrieval after successful resection and closure due to a large lesion size (mean 47 mm). There was no delayed bleeding, perforation or recurrence at a median of 13 months.

Conclusion

Upper GI SELs are effectively and safely treated by endoscopic resection. The SAFE approach provides a framework that facilitates structured decision-making. Oesophageal SELs suspected to involve the MP should undergo STER. Gastric SELs can be best managed by ESD with a view to proceed to exposing-EFTR. A laparoscopic UGI surgeon should be available in case surgical retrieval of the specimen or laparoscopic completion is required.

Disclosure

Michael J. Bourke: Research Support: Olympus, Cook Medical, Boston Scientific.

Introduction

Copeptin is a stable surrogate biomarker for the activation of the arginine vasopressin (AVP) system [1] as occurs in patients with cirrhosis and clinically significant portal hypertension (CSPH). Specifically, CSPH-induced haemodynamic alterations characterized by reduced peripheral vascular resistance and splanchnic hyperemia cause subsequent AVP system activation [2]. We aimed to evaluate serum copeptin levels (i) in different stages of cirrhosis and (ii) their prognostic value for clinical events in a cohort of stable outpatients with cirrhosis.

Aims & Methods

Stable outpatients with cirrhosis undergoing hepatic venous pressure gradient (HVPG) measurements and available copeptin levels were included; while patients with liver transplantation, malignacy, vascular liver disease, transjugular intrahepatic portosystemic shunt, heart failure and infection were excluded. Patients were stratified into 6 subgroups (S0-S5) for their clinical stage of cirrhosis, with S0-S2 representing compensated cirrhosis (S0: subclinical portal hypertension (PH) with an HVPG of 6-9 mmHg, S1: CSPH without varices, S2: CSPH with varices) and S3-S5 decompensated cirrhosis (S3: history of variceal bleeding, S4: non-bleeding decompensation, S5: further decompensation). Competing risk regression models were used to evaluate the association between copeptin levels and the risk of acute on chronic liver failure (ACLF) and liver-related death (LRD). Liver transplantation was considered as a competing event. Our previously established serum copeptin cut-off at >11.4 pmol/L [3] was used to stratify the patients into a high vs. low AVP system activation group.

Results

In total, 475 patients (S0: n=49, S1: n=53, S2: n=75, S3: n=22, S4: n=156, S5: n=120) with male predominance (72.6%; median age: 57.1 years) were included. Median follow-up time was 26.4 months. Median copeptin levels significantly increased throughout the substages of ACLD (from S0: 7.5 pmol/L to S5: 14.3 pmol/L; p<0.001). Patients with ascites had significantly higher copeptin levels (11.3 pmol/L vs. 7.2 pmol/L in non-ascites patients; p=<0.001).
Serum copeptin correlated significantly with C-reactive protein (ρ=0.281; p<0.001) and interleukin-6 levels (ρ=0.416; p<0.001) in patients with decompensated cirrhosis.
AVP system activation (i.e., Copeptin high >11.4 pmol/L) was found in n=194/475 (40.8%) patients. Patients with high-Copeptin had a significantly higher median MELD-Na (15 vs. low-Copeptin: 12; p<0.001) as well as significantly lower median mean arterial pressure (97 mmHg vs. low-Copeptin: 101 mmHg; p=0.027). The cumulative incidence of ACLF and LRD was significantly higher in patients with high-Copeptin at 1 year (ACLF: 24.1% vs. 4.0%; LRD: 21.9% vs. 4.0%), 2 years (ACLF: 32.0% vs. 7.2%; LRD: 26.7% vs. 6.3%) and 3 years (ACLF: 34.4% vs. 11.6%; LRD: 29.9% vs. 11.2%) of follow-up (p<0.001 for ACLF and LRD).
In competing risk regression adjusted for age, Child Turcotte Pugh score, creatinine, sodium and HVPG, higher levels of copeptin were independently predictive of ACLF (adjusted subdistribution hazard ratio [asHR] per pmol/L: 1.01; 95% confidence interval [95%CI]: 1.00-1.01; p=0.014), as well as LRD (asHR: 1.01; 95%CI: 1.00-1.01; p=0.024).

Conclusion

In ACLD, copeptin levels are upregulated with increasing disease severity suggesting progressive AVP system activation, associated with hemodynamic dysregulation and systemic inflammation. High-Copeptin represents an independent risk factor for ACLF and LRD development.

References

1. Abudeif A, Hashim MS, Ahmed NM, Ahmed AO. Serum copeptin is associated with major complications of liver cirrhosis and spontaneous bacterial peritonitis. Clin Exp Hepatol. 2023;9(1):71-8.

2. Kerbert AJ, Weil D, Verspaget HW, Moréno JP, van Hoek B, Cervoni JP, et al. Copeptin is an independent prognostic factor for transplant-free survival in cirrhosis. Liver Int. 2016;36(4):530-7.

3. Hartl L, Jachs M, Desbalmes C, Schaufler D, Simbrunner B, Paternostro R, et al. The differential activation of cardiovascular hormones across distinct stages of portal hypertension predicts clinical outcomes. Hepatol Int. 2021;15(5):1160-73.