Clinical Case Summary

We present the case of a 67-year-old man with a history of arterial hypertension who consulted for frontal headache, vomiting and unsteady gait for a week. Cranial CT scan on admission showed no alterations. Lumbar puncture was performed with findings suggestive of leptomeningeal carcinomatosis: 190 red blood cells/mcL, 10 leukocytes/mcL, glucose 45 mg/dL, protein 29 mg/dL; cytology suspicious for malignancy. A thoracoabdominal-pelvic CT scan was completed in search of an occult tumour with no evidence of lesions. In addition, colonoscopy was performed in which 6 0-IIa Paris polyps of 7-10 mm were observed in the left colon (photo 1A), and gastroduodenoscopy showed a poorly distensible and hard stomach on biopsy, together with the presence in the duodenum of 3 raised lesions of about 5 mm, excavated in the centre (photo 1B), also biopsied. Histology results reported mucosal and submucosal infiltration by poorly differentiated carcinoma, with positive immunohistochemistry for CK19 and CK7 and negative for inhibin, synaptophysin, chromogramin and S-100, all suggestive of infiltration by poorly differentiated carcinoma of probable gastric origin. Unfortunately, the patient presented a progressive clinical deterioration and died 2 weeks later.
Leptomeningeal carcinomatosis is the diffuse seeding of malignant cells in the cerebrospinal fluid. Its association with gastric cancer is extremely rare, with a prevalence of 0.14-0.24% of patients, mostly described in undifferentiated and signet ring tumours. Similarly, gastric metastases in the colon, and even more so in the duodenum, have been poorly described, being mainly associated with seeding and extension of implants through the gastrointestinal lumen. The benefits of intrathecal chemotherapy are currently controversial. In light of this case, the clinician should consider in the differential diagnosis of leptomeningeal carcinomatosis, the possibility of an occult gastric tumour, even in early stages.

References

Park, Kwang-Kug et al. "A case of metastatic leptomeningeal carcinomatosis from early gastric carcinoma". World J Surg Onc 10, 74 (2012).
Sonoda, Hirofumi et al. “Lymphogenous metastasis to the transverse colon that originated from signet-ring cell gastric cancer: A case report and review of the literature.” Clinics and research in hepatology and gastroenterology vol. 41,6 (2017): e81-e86.

Clinical Case Summary

We collected clinical, laboratory, radiological, cholangiographic, endoscopic and pathological findings from a 58 years old male, obese, with type 2 diabetes and with cardiovascular comorbidity. During 2020-2021, he was admitted several times at the Emergency Department for abdominal and thoracic pain, nausea and cold sweating, with night onset. Radiological, including abdominal Computed Tomography (CT) and laboratoristic findings were persistently negative. In 2022 he was hospitalized for acute mild biliopancreatitis. Abdominal Ultrasound (US) resulted negative for bile duct dilatation. During hospitalization, symptomatology improved, but he developed jaundice. Abdominal CT with contrast medium was performed, confirming mild pancreatitis (CT Severity Index 2), thickening and hyperemia of the common bile duct wall. Magnetic Resonance Cholangiopancreatography (MRCP) showed cholelithiasis, choledocolithiasis with multiple chained filling defects, without bile ducts dilatation. Endoscopic Retrograde CholangioPancreatography (ERCP) was then performed, demonstrating a 2 cm filling defect, with extraction of mucus and sludge, mixed with intraluminal tissue. Histopathology described a Bile Duct Intraductal Papillary Mucinous Neoplasm (IPMN-B) with low grade (and focally high grade) dysplasia. Clinical onset and imaging were misleading and ERCP findings were unexpected. IPMN-B is a rare premalignant disease, presenting as an intraluminal papillary mass, sometimes secreting mucus, and leading to abdominal pain, obstructive jaundice and cholangitis. Associated lithiasis is not rare and can be confusing. Pathological and radiological classification have been proposed. Here we focus on clinical presentation and radiological appearance aiming to raise the awareness about this rare entity.

References

1. Endoscopic image showing mucus and sludge, mixed with friable tissue, draining from papilla maior
2. Magnetic Resonance Cholangiopancreatography demonstrating focal lack of signal in common bile duct
3. Retrograde Cholangiogram showing a “peculiar” filling defect in a fusiform dilated choledocus

Introduction

Intraductal Papillary Mucinous Neoplasm (IPMN) is the most frequent type of pancreatic cystic neoplasm found in the general population. Due to the advances in imaging technologies, the rate of detection of pancreatic cystic neoplasms has been increasing in the past years. A large-scale single-center cohort study conducted in Seoul revealed a prevalence of 1.7% in asymptomatic healthy individuals [1]. Polycystic liver disease (PLD) is a rare disorder characterized by the presence of at least 10 parenchymal cysts in the liver. It may be inherited in an autosomal dominant fashion and, depending on the most prominent phenotype, PLD can be an isolated manifestation in autosomal dominant polycystic liver disease (ADPLD) or it can be an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD) [2,3]. In ADPKD, cysts can be detected also in the pancreas, seminal vesicles and arachnoid membrane [4]. In literature there are isolated reports of association between Intraductal Papillary Mucinous Neoplasm and Autosomal Dominant Polycystic Kidney Disease [5,6].

Aims & Methods

A retrospective observational study was conducted to include all PLD patients followed at CEMAD (Centro Malattie Apparato Digerente) Fondazione Policlinico Universitario A. Gemelli to determine the prevalence of IPMNs in PLD patients (isolated or associated to ADPKD) using abdominal resonance imaging (MRIs) and/or abdominal CT scan, in order to evaluate whether IPMN can be a not acknowledged manifestation of PLD.

Results

Abdominal MRIs and/or abdominal CT scans were examined for 92 PLD patients (56 ADPKD and 36 isolated PLD). IPMNs were detected in 8 patients (8.7% of total), 4 with isolated PLD (11.7% of total isolated PLD patients) and 4 with PLD in ADPKD (7% of total ADPKD patients). As for the IPMN site, body-tail localization of the IPMN was the most frequent detected (50% of total IPMNs).

Conclusion

A high prevalence of IPMN in PLD patients was found, which seems to be higher than prevalence found in the general population (8.7% vs 1.7%). Moreover, IPMN appeared to be more frequent in isolated PLD than in PLD associated with ADPKD (11.1% vs 7%). Our results suggest we should consider the possibility of IPMN being another extrahepatic manifestation of PLD. Of course, further studies in a larger population of patients are needed in order to confirm our findings.

References

References:

1. Chang YR, Park JK, Jang JY, Kwon W, Yoon JH, Kim SW. Incidental pancreatic cystic neoplasms in an asymptomatic healthy population of 21,745 individuals: Large-scale, single-center cohort study. Medicine (Baltimore). 2016 Dec;95(51):e5535.

2. Lesioni benigne epatiche 2022: linea guida multisocietaria per la pratica clinica

3. EASL Clinical Practice Guidelines on the management of cystic liver diseases. J Hepatol. 2022 Oct;77(4):1083-1108. doi: 10.1016/j.jhep.2022.06.002. Epub 2022 Jun 18. Erratum in: J Hepatol. 2023 Nov;79(5):1338.

4. Harris PC, Torres VE. Polycystic Kidney Disease, Autosomal Dominant. 2002 Jan 10 [updated 2022 Sep 29]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024.

5. Sato Y, Mukai M, Sasaki M, Kitao A, Yoneda N, Kobayashi D, Imamura Y, Nakanuma Y. Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease. Pathol Int. 2009 Mar;59(3):201-4.
   

6. Naitoh H, Shoji H, Ishikawa I, Watanabe R, Furuta Y, Tomozawa S, Igarashi H, Shinozaki S, Katsura H, Onozato R, Kudoh M. Intraductal papillary mucinous tumor of the pancreas associated with autosomal dominant polycystic kidney disease. J Gastrointest Surg. 2005 Jul-Aug;9(6):843-5.

Introduction

Post-ERCP pancreatitis (PEP) is the most frequent adverse event (AE) following ERCP with a 3.5-9.7% overall incidence across studies. ERCP is performed also to treat post-liver transplantation (LT) biliary complications. The occurrence of PEP in LT patients is not well studied, however in some series a slight increase in PEP incidence has been described.
The aim of this study is to perform a case-control study to evaluate the risk for PEP in post-LT patients.

Aims & Methods

This is a case-control retrospective study performed at a single high-volume endoscopy center. All consecutive patients undergoing ERCP for the first time (native papilla) for post-LT biliary complications from January 2015 to September 2022, were reviewed from a prospectively maintained ERCP database. The control group consisted of patients with native papilla undergoing ERCP for indications different than post-LT biliary complications at the same unit from January 2019 to December 2019. Demographic data, technical data, and post-ERCP AEs were recorded. Rectal indomethacin was administrated in all cases as PEP prophylaxis. General anesthesia or deep sedation was used in all cases. Statistical analysis was performed with SPSS 20 software. Descriptive data are reported as mean/median ± standard deviation/range, or percentage. Categorical data were compared using the χ₂ test. P values <0.05 were considered statistically significant.

Results

One hundred and six post-LT patients were included in the “case” group and 452 in the “control” group. Patients’ characteristics are summarized in Table 1. PEP prevalence was significantly higher in the case group (8/106, 7.5%) compared with the control group (15/452, 3.5%) (OR: 2.37, 95% CI: 0.98-5.75, P= 0.048). Double guide-wire cannulation prevalence (considered a criterion of difficult cannulation) was significantly higher in the case group (21/106, 19.8%) compared with the control group (54/442, 12.2%) (OR: 1.78, 95% CI: 1.02-3.10, P= 0.041). PEP prevalence resulted still higher in LT patients (OR: 4.19, 95% CI: 1.01-6.06, P= 0.04) after exclusion from the control group of pancreatic cancer patients (n=104), considered at lower risk for PEP assuming pancreatic duct obstruction as a protective factor.

Table 1: Patients characteristics (n: number, LT: liver transplantation, AEs: adverse events, PEP: post-ERCP pancreatitis).

Conclusion

LT patients are at higher risk of PEP when compared with patients undergoing ERCP for other indication. Biliary cannulation seems to be more difficult in LT patients and this could explain the higher risk of PEP. The main limitations of this study are the retrospective design and the presence of a historic cohort group as controls. Prospective studies are needed to confirm these data.

References

[1] Kochar B, Akshintala VS, Afghani E, et al. Incidence, severity, and mortality of post-ERCP pancreatitis: a systematic review by using randomized, controlled trials. Gastrointest Endosc 2015; 81: 143-149.e9.
[2] Moy BT, Birk JW. A review on the management of biliary complications after orthotopic liver transplantation. Journal of clinical and translational hepatology. 2019 Mar 28;7(1):61.
[3] Kochhar G, Parungao JM, Hanouneh IA, Parsi MA. Biliary complications following liver transplantation. World J Gastroenterol. Published online 2013. doi:10.3748/wjg.v19.i19.284.

Introduction

Endoscopic ultrasound-guided gastroenterostomy (EUS-GE) offers advantages over duodenal stenting and surgical bypass. EUS-GE technique, however, remains non-standardized. A parallel enteric tube (PET) facilitates luminal distention, a critical step during EUS-GE.

Aims & Methods

We primarily aimed at standardizing PET-assisted EUS-GE, secondarily assessing reproducibility and outcomes.
Prospective multicenter study on consecutive adult patients with unresectable malignant gastric outlet obstruction undergoing primary EUS-GE between August 2019-April 2021. Hierarchical task analysis predefined procedural steps into tasks and subtasks. Subtasks were further categorized into essential (performed in all centers and in over 85% of the procedures) or optional. Subtask methodology was considered established if performed similarly in all centers or variable if not. Procedure times, injected fluid volume, luminal diameters obtained, accessories used, and clinical outcomes were recorded.

Results

Seven endoscopists performed EUS-GE in 65 patients (50.8% male, median [IQR] age 77.5 [65.7-86.5] years). EUS-GE was categorized into 4 tasks and 10 subtasks (Table 1). Five essential subtasks involved an established methodology (guidewire and PET placement, endoscope exchange, delivery system insertion and LAMS deployment).
Technical and clinical success rates were 98.5% and 83.3%, respectively. AEs occurred in 10 (15.4%) patients (2 mild, 7 moderate, 1 severe). Success and AE rates were not different between expert and non-experts. Median (IQR) procedure time was 26.2 (19.7-37.1) min. The short and long axis of the targeted small bowel loop was 27 (23-30) x 40 (33-60) mm. Procedure time was longer (35 [30.6-43.7] vs 21.8 [16.4-29.5] min, p<0.001) and injected fluid volume higher (510 [439-870] vs 415 [255-480] ml, p=0.01) in non-experts.

Table 1. Proposed taxonomy for the PET-assisted EUS-GE

Conclusion

PET-assisted EUS-GE was standardized, identifying its key steps and technique variants. PET-assisted EUS-GE appears to be a reproducible procedure among advanced endoscopists with different levels of experience. (Clinical Trials Identification Number: NCT04660695).

Disclosure

Carlos Chavarríareceived research and training support by a grant from The Foundation of the Institute of Health Sciences Studies of Castilla y León (IECSCYL).
Vanessa Martín-Álvarezhas nothing to declare.
José Ramón Aparicio is a consultant for Boston Scientific.
Jose Carlos Subtil has nothing to declare.
Francisco Javier Garcia-Alonso has nothing to declare.
Juan J. Vila is a consultant for Boston Scientific and speaker for Cook Endoscopy and Olympus.
Belén Martínez-Moreno has nothing to declare.
Victoria Busto-Bea has nothing to declare.
Ramon Sanchez-Ocaña has nothing to declare.
Carlos de la Serna-Higuera has nothing to declare.
Manuel Perez-Miranda Miranda is a consultant and speaker for Boston Scientific, Olympus, and M.I.Tech.