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Background/Aims@#Most guidelines recommend surgical resection of all main duct (MD) and mixed-type (MT) intraductal papillary mucinous neoplasms (IPMNs) in suitable patients. However, there is little evidence regarding the malignancy risk of enhancing mural nodules (EMNs) that are present only in the main pancreatic duct (MPD) in patients with MD- and MT-IPMNs.Therefore, this study aimed to identify the clinical and morphological features associated with malignancy in MD- and MT-IPMNs with EMNs only in the MPD. @*Methods@#We retrospectively enrolled 50 patients with MD- and MT-IPMNs with EMNs only in the MPD on contrast-enhanced magnetic resonance imaging. We evaluated the clinical characteristics and preoperative radiologic imaging results of MPD morphology and EMN size and analyzed the risk factors associated with malignancy. @*Results@#Histological findings of EMNs were low-grade dysplasia (38%), malignant lesions (62%), high-grade dysplasia (34%), and invasive carcinoma (28%). On the receiver operating characteristic curve, the cutoff value of EMN size on magnetic resonance imaging for best predicting malignancy was 5 mm (sensitivity, 93.5%; specificity, 52.6%; area under the curve, 0.753).Multivariate analysis showed that only EMN >5 mm (odds ratio, 27.69; confidence interval, 2.75 to 278.73; p=0.050) was an independent risk factor for malignancy. @*Conclusions@#EMNs of >5 mm are associated with malignancy in patients with MD- and MTIPMNs with EMNs that are present only in the MPD, in accordance with the international consensus guidelines.
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Background/Aims@#There are limitations in treating ampullary adenomas with intraductal extension using conventional endoscopic modalities. Endoscopic intraductal radiofrequency ablation (ID-RFA) may be useful for treating intraductal (common bile duct [CBD] and/or pancreatic duct [PD]) extensions of ampullary adenomas, but long-term data are lacking. We thus evaluated the long-term outcomes of endoscopic ID-RFA for managing ampullary adenomas with intraductal extension. @*Methods@#Prospectively collected endoscopic ID-RFA database at Asan Medical Center was reviewed to identify consecutive patients with ampullary adenoma who underwent ID-RFA for intraductal extension between January 2018 and August 2021. Technical success, short-term and long-term clinical success, and adverse events were evaluated. @*Results@#A total of 29 patients (14 CBD, 1 PD, and 14 CBD and PD) were analyzed. All patients had undergone endoscopic snare papillectomy prior to ID-RFA. A median of one session of IDRFA (range, 1 to 3) for residual or relapsed intraductal extension of ampullary adenoma were successfully performed (technical success=100%). Both biliary and pancreatic stenting were routinely performed after ID-RFA to prevent ductal stricture. After a median follow-up of 776 days (interquartile range, 470 to 984 days), the short-term and long-term clinical success rates were 93% and 76%, respectively. Seven patients experienced procedural adverse events and three patients developed ductal strictures. @*Conclusions@#Endoscopic ID-RFA showed good long-term outcomes in treating residual or relapsed ampullary adenomas with intraductal extension. Repeated ID-RFA may be considered as an option for managing recurrence. Further studies are needed to standardize the procedure.
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Purpose@#This study evaluated the efficacy of adjuvant chemotherapy (AC) in patients with resected ampulla of Vater (AoV) carcinoma. @*Materials and Methods@#Data from 646 patients who underwent surgical resection at Asan Medical Center between 2000 and 2017 were retrospectively reviewed. @*Results@#The median age of the patients was 62 years, and 54.2% were male. Patients were classified into AC group (n=165, 25.5%) and no AC group (n=481, 74.5%). With a median follow-up duration of 88 months, in patients with stage I, II, III, median recurrence-free survival (RFS) was not reached, 44 months, and 15 months, respectively, and the median overall survival (OS) were not reached, 88 months and 35 months, respectively. Despite no statistical significance, RFS and OS were better in stage II patients with AC than in those without AC (median RFS, 151 months vs. 38 months; p=0.156 and median OS, 153 months vs. 74 months; p=0.299). In multivariate analysis for RFS and OS, TNM stage, R1 resection status, presence of lymphovascular invasion, and perineural invasion remained significant factors, whereas AC (hazard ratio [HR], 0.74; 95% confidence interval [CI], 0.54 to 1.00; p=0.052) was marginally related with RFS. After propensity score matching in only stage II/III patients, RFS and OS with AC were numerically longer than those without AC (HR, 0.80; 95% CI, 0.60 to 1.06; p=0.116 and HR, 0.77; 95% CI, 0.56 to 1.06; p=0.111). @*Conclusion@#AC with fluoropyrimidine did not improve survival of patients with resected AoV carcinoma. However, multivariate analysis with prognostic factors showed a marginally significant survival benefit with AC.
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The emergence of the disease entity of glucocorticoid-responsive systemic immunoglobulin G4 (IgG4)-related pancreatobiliary disease has generated substantial attention among the international gastroenterology society. IgG4-related pancreatobiliary disease includes type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). The typical manifestations of IgG4-related pancreatobiliary disease are cholestatic liver dysfunction, obstructive jaundice, and weight loss, although it may present with no clinical symptoms. Since it mimics tumors on imaging, AIP/IgG4-SC may often be misdiagnosed as pancreatic or biliary cancer. The endoscopic armamentarium for the diagnosis of IgG4-related pancreatobiliary disease includes endoscopic ultrasonography, intraductal ultrasonography, endoscopic retrograde cholangiopancreatography, and cholangioscopy. The role of endoscopic tissue acquisition is two-fold in the diagnosis of IgG4-related pancreatobiliary disease: exclusion of cancer and procurement of histopathological proof for diagnosis of AIP/IgG4-SC, which can also be achieved by adding the immunohistochemistry for IgG4. Our review article addresses the role of various endoscopic examinations in diagnosing IgG4-related pancreatobiliary disease, focusing on the differentiation of this condition from pancreatobiliary malingnancies.
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Purpose@#This study evaluated the efficacy of adjuvant chemotherapy (AC) in patients with resected ampulla of Vater (AoV) carcinoma. @*Materials and Methods@#Data from 646 patients who underwent surgical resection at Asan Medical Center between 2000 and 2017 were retrospectively reviewed. @*Results@#The median age of the patients was 62 years, and 54.2% were male. Patients were classified into AC group (n=165, 25.5%) and no AC group (n=481, 74.5%). With a median follow-up duration of 88 months, in patients with stage I, II, III, median recurrence-free survival (RFS) was not reached, 44 months, and 15 months, respectively, and the median overall survival (OS) were not reached, 88 months and 35 months, respectively. Despite no statistical significance, RFS and OS were better in stage II patients with AC than in those without AC (median RFS, 151 months vs. 38 months; p=0.156 and median OS, 153 months vs. 74 months; p=0.299). In multivariate analysis for RFS and OS, TNM stage, R1 resection status, presence of lymphovascular invasion, and perineural invasion remained significant factors, whereas AC (hazard ratio [HR], 0.74; 95% confidence interval [CI], 0.54 to 1.00; p=0.052) was marginally related with RFS. After propensity score matching in only stage II/III patients, RFS and OS with AC were numerically longer than those without AC (HR, 0.80; 95% CI, 0.60 to 1.06; p=0.116 and HR, 0.77; 95% CI, 0.56 to 1.06; p=0.111). @*Conclusion@#AC with fluoropyrimidine did not improve survival of patients with resected AoV carcinoma. However, multivariate analysis with prognostic factors showed a marginally significant survival benefit with AC.
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Percutaneous transhepatic gallbladder drainage is an effective treatment for acute cholecystitis in poor surgical candidates. Endoscopic gallbladder drainage procedures, such as endoscopic ultrasound-guided gallbladder drainage, endoscopic gallbladder stenting, and endoscopic naso-gallbladder drainage, have been used as alternative treatments for acute cholecystitis. These procedures are associated with increased patient comfort and physiologic drainage. We report a case of endoscopic gallbladder stenting for acute cholecystitis in a 62-year-old male undergoing chemotherapy for metastatic pancreatic cancer. After endoscopic gallbladder stenting, the patient’s acute cholecystitis resolved and he was able to undergo scheduled chemotherapy. The inserted double-pigtail plastic stent will be left in situ permanently. The choice of drainage modality for acute cholecystitis will generally be based on resources, patient preferences, local expertise, and clinical context.
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Pancreatic cancer is one of the most lethal malignancies worldwide. The risk of developing pancreatic cancer increases with age. Pancreatic cancer is seen mostly in the elderly; patients under the age of 30 years are rare. Known risk factors for pancreatic cancer include genetic mutations, smoking, chronic pancreatitis, and diabetes mellitus. We report a case of pancreatic cancer in a 27-year-old man without the PRSS1 mutation. Chronic pancreatitis and smoking may have contributed to the development of pancreatic cancer in this patient. We also conducted a literature review on early onset pancreatic cancer.
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There are two forms of autoimmune pancreatitis (AIP). Type 1 is associated with immunoglobulin G4 (IgG4)-related systemic fibro- inflammatory disease, whereas type 2 AIP is localized to the pancreas and not associated with IgG4. The number of children presenting with type 2 AIP has recently increased. Here, we report a case of type 2 AIP in a 16-year-old adolescent who presented with clinical acute pancreatitis and associated pancreatic masses. He was diagnosed with type 2 AIP based on pancreatic biopsy results showing granulocytic epithelial lesions and supportive radiological imaging and steroid responsiveness.
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Background/Aims@#Interventional endoscopists may utilize contrast-enhanced harmonic endoscopic ultrasound (CEHEUS) for image guidance during radiofrequency ablation (RFA) because of its capability to delineate real-time tumor perfusion dynamics. The purpose of this study was to assess the utility of CEH-EUS for the guidance and monitoring of en-doscopic RFA. @*Methods@#Nineteen consecutive patients with solid abdominal tumors who underwent CEH-EUS and endo-scopic RFA were included. The extent of the ablation was as-sessed by CEH-EUS at 5 to 7 days after RFA. Additional RFAs were performed under CEH-EUS guidance. @*Results@#The diag-noses were as follows: nonfunctioning neuroendocrine tumor (n=13), solid pseudopapillary neoplasm (SPN) (n=2), insu-linoma (n=1), left adrenal adenoma (n=2), and left adrenal oligometastasis (n=1). Pre-CEH-EUS findings revealed that 17 cases showed hyperenhanced patterns and two cases of SPN showed isoenhanced patterns. CEH-EUS-assisted RFA was technically feasible in all 19 patients. After the first RFA session, seven patients of the treated tumors showed the disappearance of intratumoral enhancement on CEH-EUS, whereas 12 showed residual contrast enhancement. Twelve patients with incomplete ablation were further treated with additional RFA under real-time CEH-EUS guidance. Radiolog-ic complete response was observed in 13 patients (68.4%). Among the 35 ablation procedures, the only adverse events were two episodes of pancreatitis (5.7%; 1 moderate and 1mild). During the median follow-up of 28 months, the local recurrence rate was 7.7%. @*Conclusions@#The application of CEH-EUS for RFA could be helpful in assessing early treat-ment response after ablation and targeting residual viable tumors during additional ablation sessions.
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Background/Aims@#The natural history of spontaneous decrease in the size of pancreatic cystic lesions (PCLs) without high-risk stigmata is under investigation. This study aimed to investigate the timing of spontaneous decrease in the size of PCLs without high-risk stigmata and to identify the characteristics associated with their complete resolution. @*Methods@#From 2000 to 2016, patients with spontaneous decreases in PCL size on computed tomography (CT) and/or magnetic resonance imaging (MRI) who had at least 1 year of follow-up were evaluated retrospectively. @*Results@#A total of 78 patients underwent follow-up for an average of 55.7 months. Most patients were asymptomatic, and 35 (37.2%) showed complete resolution. The initial mean PCL size was 1.6±0.9 cm (range, 0.5 to 5.6 cm). The average time to initial decrease in size and complete resolution of PCLs were 32.1 and 41.5 months, respectively. Compared with PCLs that completely resolved, presence of underlying malignancy was associated with partial resolution of PCLs in multivariable analysis (hazard ratio, 0.51; 95% confidence interval, 0.32 to 0.81; p=0.005). Endoscopic ultrasound (EUS) identified detailed findings, especially the presence of septum (p<0.001), calcification (p=0.015) and lobulation (p=0.001) that were not found on CT/MRI. @*Conclusions@#Asymptomatic small PCLs without high-risk stigmata can naturally decrease in size at approximately 3 years, and complete resolution can be expected in the absence of underlying malignancy.Regular follow-up of approximately 3 years with EUS may be a reasonable and safe alternative when planning the initial treatment of small PCLs without high-risk stigmata.
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Purpose@#Since the introduction of nab-paclitaxel plus gemcitabine (nab-P+GEM) as first-line (1L) treatment for metastatic pancreatic adenocarcinoma (mPDAC), optimal second-line (2L) chemotherapy after progression is unclear. We assessed clinical outcomes of 2L chemotherapy for disease that progressed on 1L nab-P+GEM. @*Materials and Methods@#Among the 203 patients previously treated with 1L nab-P+GEM for mPDAC at Asan Medical Center, between February and December 2016, records of 120 patients receiving 2L chemotherapy after progression on nab-P+GEM were retrospectively reviewed. The response rate and survival were evaluated along with analysis of prognostic factors. @*Results@#Fluoropyrimidine-oxaliplatin doublets (FOLFOX or XELOX) were used in 78 patients (65.0%), fluoropyrimidine monotherapy in 37 (30.8%), and liposomal irinotecan plus fluorouracil in two (1.7%). The median progression-free survival (PFS) and overall survival (OS) were 3.29 months and 7.33 months from the start of 2L therapy. Fluoropyrimidine-oxaliplatin regimens and fluoropyrimidine monotherapy did not yield significantly different median PFS (2.89 months vs. 3.81 months, p=0.40) or OS (7.04 months vs. 7.43 months, p=0.86). A high neutrophil-lymphocyte ratio (> 2.2) and a short time to progression with 1L nab-P+GEM (< 6.4 months) were independent prognostic factors of poor OS with 2L therapy. @*Conclusion@#2L fluoropyrimidine-oxaliplatin doublets and fluoropyrimidine monotherapy after failure of 1L nab-P+GEM had modest efficacy, with no differences in treatment outcomes between them. Further investigation is warranted for the optimal 2L chemo-regimens and sequencing of systemic chemotherapy for patients with mPDAC.
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Pancreatic cancer has a poor prognosis due to the difficulty of early diagnosis. Observation is recommended for early diagnosis of pancreatic cancer in elderly patients with risk factors such as newly diagnosed diabetes and chronic pancreatitis. A 66-year-old male suffered from acute pancreatitis of uncertain etiology. Initial pancreatic imaging showed a main pancreatic duct stricture at the pancreas body/tail junction and minimal duct dilatation without a visible mass. Eight months later, however, pancreatic imaging revealed a pancreatic mass at the previous stricture site with progression of the upstream duct dilation. The patient underwent distal pancreatectomy, and a pathologic examination showed stage 1A pancreatic cancer with a predominantly intraductal spreading pattern. We report a case of stage 1A pancreatic cancer that initially manifested as acute obstructive pancreatitis, which enabled early diagnosis of pancreatic cancer.
Subject(s)
Aged , Humans , Male , Carcinoma, Pancreatic Ductal , Constriction, Pathologic , Dilatation , Early Diagnosis , Pancreas , Pancreatectomy , Pancreatic Ducts , Pancreatic Neoplasms , Pancreatitis , Pancreatitis, Chronic , Prognosis , Risk FactorsABSTRACT
Primary lymphoma of pancreatic mucosa-associated lymphoid tissue (MALT) is extremely rare. Initial suspicion of primary pancreatic lymphoma is hampered by its low incidence. However, it should always be included in a differential diagnosis of pancreatic mass with unusual features, because an accurate diagnosis can avoid unnecessary surgical intervention. A 70-year-old woman presented with melena associated with a mass on the pancreatic head. Endoscopic ultrasonography-guided core-needle biopsy of the pancreatic mass revealed MALT lymphoma. The patient is currently undergoing radiation therapy. We present a case of primary lymphoma of the pancreatic MALT with a review of the literature.
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Type I autoimmune pancreatitis (AIP), a form of chronic pancreatitis, is included within the spectrum of immunoglobulin G4-related diseases (IgG4-RD). IgG4-RD can involve the salivary glands, bile ducts, kidneys and retroperitoneum, and the pancreas, and responds well to corticosteroid treatment. However, gastric involvement in IgG4-RD has been rarely reported. Here we report the case of a 56-year-old man with type I autoimmune pancreatitis who presented with a giant gastric ulcer mimicking advanced gastric cancer, which was subsequently determined to be IgG4-RD. Both the patient' symptoms and his ulcerative lesions in the stomach responded to steroid treatment, which obviated the need for major surgery such as gastrectomy. Our case demonstrates the importance of recognizing potential gastric involvement in IgG4-RD, especially in a patient with concurrent AIP/IgG4-RD or a history of the disease, who is evaluated for an intractable and/or atypical gastric ulcer. A prompt diagnosis and proper management will avoid unnecessary surgery.
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Among metastatic pancreatic tumors, malignant melanomas are rare. Here, we report a very rare case of a malignant melanoma that involved only the pancreas and liver, and was difficult to differentiate from a neuroendocrine tumor of the pancreas with a liver metastasis. A 65-year-old- male with no history of a malignant melanoma exhibited hypervascular hepatic and pancreatic tumors on computed tomography and contrast-enhanced endoscopic ultrasonography. He lacked lesions of the skin, eye, and gastrointestinal tract on physical examination, upper endoscopy, and a whole-body positron emission tomography-computed tomography scan; these sites are common primary sites of malignant melanoma. Finally, endoscopic ultrasound-guided pancreatic core biopsy and percutaneous ultrasonography-guided liver biopsy confirmed malignant melanoma of the pancreas and liver.
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Pancreatic cancer has a poor prognosis due to the difficulty of early diagnosis. Observation is recommended for early diagnosis of pancreatic cancer in elderly patients with risk factors such as newly diagnosed diabetes and chronic pancreatitis. A 66-year-old male suffered from acute pancreatitis of uncertain etiology. Initial pancreatic imaging showed a main pancreatic duct stricture at the pancreas body/tail junction and minimal duct dilatation without a visible mass. Eight months later, however, pancreatic imaging revealed a pancreatic mass at the previous stricture site with progression of the upstream duct dilation. The patient underwent distal pancreatectomy, and a pathologic examination showed stage 1A pancreatic cancer with a predominantly intraductal spreading pattern. We report a case of stage 1A pancreatic cancer that initially manifested as acute obstructive pancreatitis, which enabled early diagnosis of pancreatic cancer.
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Afferent loop syndrome (ALS) is a rare cause of recurrent pancreatitis. Recurrent pancreatitis in association with ALS can develop due to impaired outflow of pancreatic juice or reflux of enteric secretions caused by increased intraluminal duodenal pressure. Here, we report a case of ALS presenting as recurrent acute pancreatitis due to chronic intermittent partial obstruction of the third portion of the duodenum caused by postoperative adhesion. Interestingly, pancreatic histology showed a granulocytic epithelial lesion, which is a diagnostic feature of type 2 autoimmune pancreatitis (AIP, idiopathic duct centric chronic pancreatitis [IDCP]). From this case we learned that the diagnosis of type 2 AIP should be made in the appropriate clinical setting.
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Type 1 autoimmune pancreatitis (AIP1) is an IgG4-related systemic disease that mimics tumors. We report a rare case of AIP1 accompanied by mucinous cystic neoplasm (MCN). A pancreatic lesion was incidentally detected in a woman in her 60s. After 6 years of follow-up, the lesion abruptly increased in size. Computed tomography showed a 3.5 cm unilocular cyst in the tail of the pancreas and distal pancreatectomy was performed. On microscopic examination, the cyst was lined by mucinous and non-mucinous epithelial cells with mild cytologic atypia. The surrounding stroma comprised ovarian-type spindle cells with progesterone receptor positivity. The pericystic pancreas exhibited multifocal lymphoid follicles, lymphoplasmacytic infiltrations, obliterative phlebitis, and storiform fibrosis. IgG4-positive plasma cell infiltration (215 cells high-power field) and the IgG4/IgG ratio (57%) were increased. Cases of MCN coexisting with AIP1 are extremely rare; only two such cases have been reported in the English-language literature. This third case featured low-grade MCN with AIP1.
Subject(s)
Female , Humans , Epithelial Cells , Fibrosis , Follow-Up Studies , Mucins , Pancreas , Pancreatectomy , Pancreatitis , Phlebitis , Plasma Cells , Receptors, Progesterone , TailABSTRACT
In the version of this article initially published, the fifth author's name was stated as “Joo Nam Lee.
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BACKGROUND/AIMS: Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. METHODS: Type 2 AIP patients were classified according to International Consensus Diagnostic Criteria. Radiological findings were compared between type 2 AIP presenting as acute pancreatitis and gallstone pancreatitis. RESULTS: Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. Radiological pancreatic imaging such as delayed enhancement of diffusely enlarged pancreas, homogeneous enhancement of focal enlargement/mass, absent/minimal peripancreatic fat infiltration or fluid collection, and multifocal main pancreatic duct narrowings were helpful for differentiating type 2 AIP from gallstone pancreatitis. During follow-up (median, 32.3 months), two patients (2/25, 8%) experienced relapse. CONCLUSIONS: In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology.