Clinical Case Summary

Pancreatic cystic lymphangioma is extremely rare, accounting for less than 5% of pancreatic cystic neoplasms and less than 1% of all lymphangiomas.¹
A 51-year-old female presented with complaints of recurrent abdominal pain for 4 weeks. She was previously misdiagnosed as acute pancreatitis complicated with pancreatic pseudocyst. There was no history of trauma. Dynamic MRI on the abdomen revealed a large, encysted collection at the pancreatic body suggesting a query pancreatic pseudocyst. EUS examination revealed a large cystic lesion at the body of the pancreas. The cyst was multilocular with characteristic multiple thin septa within the lesion (Figure 1). EUS-FNA with 22-G needle was done with aspiration of about 20 ml of cyst fluid, divided into two bottles, one for cytology examination and mucin stain while the second bottle for Amylase, CEA and TG levels. Aspiration fluid was serous yellowish in colour, with negative string sign. Also, a large focal lesion within the spleen was also seen.The biochemical evaluation of the aspirated cystic fluid revealed low amylase and CEA, and high TG levels. Cytopathological examination showed fairly cellular material featuring numerous predominantly small lymphocytes. There were no epithelial, atypical, or malignant cells. IHC staining was positive for CD-31 and CD2-40. Alcian blue mucin stain was negative. The diagnosis of pancreatic cystic lymphangioma was made based on EUS, cytological and biochemical examination with IHC features. Since, lymphangiomas are benign tumors without malignant potential, treatment depends on the symptoms. Lymphangioma can encroach on adjacent organs and need surgical resection. To relieve symptoms of abdominal, distal pancreatectomy and splenectomy was discussed, but patient refuse surgery. Multidisciplinary team decided a conservative management with a follow-up by dynamic MRI and EUS within 6 months.

References

1. Chen D, Feng X, Lv Z, Xu X, Ding C, Wu J. Cystic lymphangioma of pancreas: A rare case report and review of the literature. Medicine (Baltimore). 2018 Jul;97(28): e11238. doi: 10.1097/MD.0000000000011238.

Clinical Case Summary

We describe a case of an 80-year-old woman with a history of bronchiectasis, nasal polyps, and chronic sinusitis who presented to the emergency department with diarrhea(>10 bowel movements/day),abdominal pain and estimated weight loss of 6 kg over one month. She was previously treated with antiparasitics without clinical improvement. Laboratory tests showed peripheral eosinophilia(10,400/mm3 eosinophils, 51.4% of total leukocytes). Viral serologies and autoimmune study were negative. Microbiological and parasitological analyses of stool samples were negative too. Immunoglobulin E levels were elevated. Abdominal computed tomography(CT) revealed thickening of the rectal ampulla and sigmoid colon. Upper gastrointestinal endoscopy did not show any abnormalities, but rectosigmoidoscopy revealed colorectal mucosa with millimetric whitish dots, some scattered erosions, and a 20 mm ulcer in the distal rectum that was biopsied. Histological evaluation showed inflammatory changes with predominance of eosinophilic polymorphonuclear cells(>50/field). Bone marrow study showed eosinophilic myeloid hyperplasia without blasts. No cytogenetic abnormalities were identified on chromosomal analysis. During hospitalization, she developed pruritic erythematous macules on the sacral region and inner thighs that gradually improved with topical steroids. Idiopathic hypereosinophilic syndrome with gastrointestinal involvement and possible cutaneous and pulmonar, was assumed. Prednisolone 40 mg/day was started with clinical and laboratory improvement, with complete resolution of peripheral eosinophilia. The diagnosis of idiopathic hypereosinophilic syndrome is a challenge because it is a diagnosis of exclusion and manifests in various ways depending on the affected system, with gastrointestinal involvement being possible, as in the case presented here. Recognition and early initiation of appropriate treatment can improve the survival and quality of life of these patients.

Introduction

A previous study, which found that focal parenchymal atrophy of the pancreas is an indirect sign of small pancreatic cancer (PC), documented the changes in the pancreas until cancer onset.［1-3］ However, the pancreas atrophies and changes with age, so issues remain regarding the criteria for determining pancreatic atrophy as a sign of PC. The application of artificial intelligence (AI) technology to quantify changes in the pancreas over time and predict the onset of PC and high-risk groups for PC from objective indicators has the potential to facilitate early detection of PC.

Aims & Methods

Based on the rate of change in the pancreatic volume on computed tomography (CT) using AI technology, the present study predicted PC onset. Forty patients in whom PC was histopathologically diagnosed at Komagome Hospital between 2015 and 2023 and who had a history of three or more follow-up CT examinations over five years before PC onset (the PC group) were analyzed. Forty patients who underwent CT periodically for malignant skin disease but had no pancreatic disease were enrolled into a non-PC group. Patients with pancreatic carcinoma in situ (pancreatic intraepithelial neoplasm), an intraductal papillary mucinous neoplasm or chronic pancreatitis with multiple calcifications were excluded. Changes in the pancreatic volume in the groups over time were then compared using CT images taken one year or earlier before PC onset. The pancreatic volume was calculated using SYNAPSE VINCENT cloud (Fuji Film, Japan), an AI image application program. Age and pancreas volume were combined to create a scatter diagram, and the rate of pancreatic volume change (the temporal pancreatic volume change coefficient) was calculated from the regression line and compared.

Results

The lesion site in PC group included the head (n=17), body (n=7), and tail (n=16). The median maximum tumor diameter was 31 mm (interquartile range: 27-37). A comparison of the two groups found a cStage classification of IB, IIA, IIB, III, and IV in eight, ten, three, eight, and 11 patients, respectively. The median age was 72.5 and 72.5 years in the respective group (P=0.182). The male: female ratio was 23:17 and 24:16 (P=0.820), the median observation period was 83.5 and 107 months (P<0.05); the mean number of examinations was 10.7 and 18; the total number of CT examinations was 430 and 745; the mean total pancreatic volume was 74.4 and 78.15 ml (P<0.01 ); and the mean pancreatic volume change coefficient was -0.141 and -0.036 (P=0.01), respectively. The rate of decrease in the pancreatic volume was faster in the PC group with increasing age, the average being 8.46 ml over five years in the PC group compared to 2.16 ml in the non-PC group. The cutoff value for the coefficient of pancreatic volume change over time was set at -0.125. The sensitivity was 50%, the specificity was 90%, the positive predictive value was 83%, and the AUC value was 0.67.

Conclusion

In comparison with the age-related changes in the pancreas, the rate of decrease in the pancreatic volume was faster in the PC group, suggesting that PC onset may be predicted using images obtained one year or earlier before PC onset. These findings further suggested that identifying patients with a high risk of PC may be possible by creating a pancreatic volume histogram using AI technology based on age at the time of CT, intervals between past examinations, and the rate of decrease in the pancreatic volume.

References

［１］Nakahodo J, Kikuyama M, Nojiri S, et al. Pancreatology. 2020 Dec;20(8):1689-1697.
［２］Nakahodo J, Kikuyama M, Fukumura Y, et al. Pancreatology. 2022 Dec;22(8):1148-1158
［３］Kikuyama M, Nakahodo J, Honda G, et al. Pancreatology. 2023 Jun;23(4):420-428.

Introduction

Despite EUS-guided drainage (EUS-FCD) is the preferred modality for management of peripancreatic fluid collections (PFCs), factors predicting the need for step-up to additional procedures (including radiology, surgery or additional endoscopies) are retrospectively explored in studies restricted to Lumen Apposing Metal Stents (LAMS) or excluding pseudocysts and post-surgical collections, which might require a step-up as well.

Aims & Methods

All consecutive candidates to EUS-FCD with either LAMS or Double-pigtail Plastic Stents (DPPS) between 2020-2023 were included in a Prospective Registry of Therapeutic EUS (PROTECT). The role of baseline clinical and morphological factors, including the Quadrant / Necrosis / Infection (QNI) score, in predicting the need for any step-up to additional procedures was evaluated by stepwise logistic regression.

Results

Twenty-three post-surgical PFCs, 19 pseudocysts and 18 walled-off necrosis [WOPN]) were included, 42% treated with LAMS and 58% with DPPS based on morphology. Clinical success was 92.9%, but 38% of patients required a step-up to necrosectomy (26.7%) or additional procedures. Patients requiring step-up versus those sufficing drainage had a higher number of quadrants involved (OR=3.6 [1.5-8.4]), a more frequent paracolic extension (OR=6 [1.4-26]), a higher necrosis content (>30%, OR=7.2 [1.8-28.4] ; >60% OR=25.2 [4.2-153]) and were in the high-risk group according to the QNI score, with no difference in the diameter and rate of superinfection. At multivariate analysis, being in the high-risk QNI group was the only independent predictor of step-up (OR=9.8 [2.9-32.9]). The score however was not able to stratify in analyses restricted to WOPN only or LAMS only. High-risk QNI patients presented also a longer hospital stay (17.5 [8-45] vs 4 [2-6.5], p=0,001), without any difference in ICU stay and nutritional support.

Conclusion

In this prospective series of consecutive non-selective PFCs undergoing EUS-FCD, QNI score was the only independent predictor of any need for step-up to additional procedures, and strongly correlated to the EUS-FCD allocation to LAMS versus DPPS, unravelling a potential additional utility.

Disclosure

GV reports travel grants from Pentax Medical, Boston Scientific and Euromedical, and lecture fees by Boston Scientific.
All the other authors ​declare no conflict of interest.

Introduction

Increased lipase level is a serological hallmark of the diagnosis of acute pancreatitis (AP) but can be detected in various other diseases associated with lipase leakage due to inflammation of organs surrounding the pancreas or reduced renal clearance and/or hepatic metabolism. This non-pancreatic hyperlipasemia (NPHL) is puzzling for attending physicians during the diagnostic procedure for AP. It would be clinically beneficial to identify the clinical and laboratory variables that hinder the accuracy of lipase diagnosis with the aim of improve it. A more precise description of the NPHL condition could potentially provide prognostic factors for adverse outcomes which is currently lacking.

Aims & Methods

We aimed to provide a detailed clinical and laboratory characterization of a large prospective NPHL patient cohort and assess parameters determining disease outcomes.
A Hungarian patient cohort with serum lipase levels at least three times higher than the upper limit of normal (ULN) was prospectively evaluated over 31 months. Patients were identified using daily electronic laboratory reports developed to support an ongoing observational, multicenter, prospective cohort study called the EASY trial (ISRCTN10525246) to establish a simple, easy, and accurate clinical scoring system for early prognostication of AP. Diagnosis of NPHL was established based on ≥ 3 × ULN serum lipase level in the absence of abdominal pain or abdominal imaging results characteristic of pancreatitis.

Results

A total of 808 patients [male, n = 420 (52%); median age (IQR): 65 (51-75) years] were diagnosed with ≥ 3 × ULN serum lipase levels. A total of 392 patients had AP, whereas 401 had NPHL with more than 20 different etiologies. Sepsis and acute kidney injury (AKI) were the most prevalent etiologies of NPHL (27.7% and 33.2%, respectively). The best discriminative cut-off value for lipase was ≥ 666 U/L (sensitivity, 71.4%; specificity, 88.8%). The presence of AKI or sepsis negatively affected the diagnostic performance of lipase. NPHL was associated with a higher in-hospital mortality than AP (22.4% vs 5.1%, P < 0.001). In multivariate binary logistic regression, not lipase but increased amylase level (> 244 U/L) and neutrophil-to-lymphocyte ratio (NLR) (> 10.37, OR: 3.71, 95%CI: 2.006-6.863, P < 0.001), decreased albumin level, age, and presence of sepsis were independent risk factors for in-hospital mortality in NPHL.

Conclusion

NPHL is a common cause of lipase elevation and is associated with high mortality rates. Increased NLR value was associated with the highest mortality risk. The presence of sepsis/AKI significantly deteriorates the serological differentiation of AP from NPHL.