CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype

The intraductal oncocytic papillary neoplasm (IOPN) of the pancreas has been recognized by WHO classification as a unique intraductal papillary mucinous neoplasm (IPMN) category. IOPN is composed of oxyphil cells, usually expressing MUC5AC, MUC6, and Hep Par-1, and harboring PRKACA/B fusion genes as their genetic hallmark. Although IOPNs are associated with an infiltrative adenocarcinoma in up to 30% of cases, the survival rate after surgical resection approaches 100%. This highlights the importance of the correct IOPN diagnosis, above all in cases with an associated invasive component. In this study, the immunohistochemical expression of CD117 was investigated in 111 IPMNs, including 17 oncocytic, 45 gastric, 20 pancreatico-biliary, and 29 intestinal IPMNs. We also tested the expression of MUC5AC, MUC6, and Hep Par-1 in the IOPN cohort. CD117 positivity was significantly more frequent in IOPNs compared to the other IPMN subtypes (p < 0.0001). Furthermore, within IOPN, a lower or absent CD117, MUC5AC, MUC6, and Hep Par-1 expression tended to be associated with the presence of an infiltrative component. Our findings shed light into the biology of these complex lesions, which are confirmed to be a distinctive IPMN subtype; notably, CD117 emerged as a potential, additional tool in the differential diagnosis of IPMNs.

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A mixed-type intraductal papillary mucinous neoplasm of the pancreas with a histologic combination of gastric and pancreatobiliary subtypes in a 70-year-old woman: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-020-02464-z ◽

2020 ◽

Vol 14 (1) ◽

Author(s):

Sawsan Ismail ◽

Rama Darwisho ◽

Mohammed Ali ◽

Maen Haidar ◽

Mohammad Adib Houreih ◽

...

Keyword(s):

Histological Examination ◽

Intraductal Papillary Mucinous Neoplasm ◽

Mixed Type ◽

Epigastric Pain ◽

The Body ◽

Diagnostic Methods ◽

Pancreatic Ducts ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms

Abstract Background Intraductal papillary mucinous neoplasms are rare papillary pancreatic neoplasms arising from major pancreatic ducts, characterized by duct dilation and mucin secretion. They comprise approximately 1% of all exocrine neoplasms and are classified according to their anatomical sites into main duct-type, branch duct-type, and mixed-type intraductal papillary mucinous neoplasms. Histological examination plays a crucial role in distinguishing and classifying intraductal papillary mucinous neoplasms into gastric, intestinal, pancreatobiliary, and oncocytic subtypes. Case presentation We present the case of a 70-year-old Syrian woman who was admitted to our hospital due to an intermittent epigastric pain accompanied by diarrhea and weight loss with a recent diagnosis of diabetes mellitus. Following clinical, laboratory, and radiological examination, distal pancreatectomy involving the body and the tail of the pancreas was performed. Interestingly, histological examination of the resected specimens revealed the diagnosis of a mixed-type intraductal papillary mucinous neoplasm with a unique combination of gastric and pancreatobiliary subtypes. Conclusion To the best of our knowledge, the combination of multiple histological subtypes of intraductal papillary mucinous neoplasms has been recorded in a few studies with reference to the challenging histological detection. Herein, we report a rare case with a significant histological combination, highlighting the difficulties in differential diagnosis due to the absence of ancillary techniques, with a brief review on diagnostic methods, histological characteristics and surgical recommendations.

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The Role of Positron Emission Tomography in Clinical Management of Intraductal Papillary Mucinous Neoplasms of the Pancreas

Cancers ◽

10.3390/cancers12040807 ◽

2020 ◽

Vol 12 (4) ◽

pp. 807 ◽

Cited By ~ 1

Author(s):

Simone Serafini ◽

Cosimo Sperti ◽

Alessandra Rosalba Brazzale ◽

Diego Cecchin ◽

Pietro Zucchetta ◽

...

Keyword(s):

Positron Emission Tomography ◽

Morphological Characteristics ◽

Emission Tomography ◽

Extensive Literature ◽

Intraductal Papillary Mucinous Neoplasms ◽

International Consensus ◽

Additional Tool ◽

Positron Emission ◽

Mucinous Neoplasms

Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas represent a heterogeneous group of tumors, increasingly diagnosed in clinical practice. An early differential diagnosis between malignant and benign lesions is crucial to patient management and the choice of surgery or observation. The therapeutic approach is currently based on a patient’s clinical, biochemical, and morphological characteristics. The latest published International Consensus Guidelines (ICG) make no mention of the role of metabolic assessments of IPMNs. The aim of this study was to review the current literature, examining the role of 18-fluorodeoxyglucose (FDG) positron emission tomography (PET) in IPMN management. An extensive literature review was conducted according to the 2009 Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and 10 articles were analyzed in detail, focusing on the value of PET as opposed to other standard imaging criteria. Data were retrieved on 419 patients. The 18-FDG-PET proved more sensitive, specific, and accurate than the ICG criteria in detecting malignant IPMNs (reaching 80%, 95%, and 87% vs. 67%, 58%, and 63%, respectively). Metabolic assessments may be used as an additional tool for the appropriate management of patients with doubtful imaging findings.

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Ductal Dilatation of ≥5 mm in Intraductal Papillary Mucinous Neoplasm Should Trigger the Consideration for Pancreatectomy: A Meta-Analysis and Systematic Review of Resected Cases

Cancers ◽

10.3390/cancers13092031 ◽

2021 ◽

Vol 13 (9) ◽

pp. 2031

Author(s):

Y.H. Andrew Wu ◽

Atsushi Oba ◽

Laurel Beaty ◽

Kathryn L. Colborn ◽

Salvador Rodriguez Franco ◽

...

Keyword(s):

Retrospective Studies ◽

Invasive Carcinoma ◽

Meta Analysis ◽

Diagnostic Value ◽

Published Data ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms ◽

Sensitivity Specificity

Intraductal papillary mucinous neoplasms (IPMN) are common but difficult to manage since accurate tools for diagnosing malignancy are unavailable. This study tests the diagnostic value of the main pancreatic duct (MPD) diameter for detecting IPMN malignancy using a meta-analysis of published data of resected IPMNs. Collected from a comprehensive literature search, the articles included in this analysis must report malignancy cases (high-grade dysplasia (HGD) and invasive carcinoma (IC)) and MPD diameter so that two MPD cut-offs could be created. The sensitivity, specificity, and odds ratios of the two cutoffs for predicting malignancy were calculated. A review of 1493 articles yielded 20 retrospective studies with 3982 resected cases. A cutoff of ≥5 mm is more sensitive than the ≥10 mm cutoff and has pooled sensitivity of 72.20% and 75.60% for classification of HGD and IC, respectively. Both MPD cutoffs of ≥5 mm and ≥10 mm were associated with malignancy (OR = 4.36 (95% CI: 2.82, 6.75) vs. OR = 3.18 (95% CI: 2.25, 4.49), respectively). The odds of HGD and IC for patients with MPD ≥5 mm were 5.66 (95% CI: 3.02, 10.62) and 7.40 (95% CI: 4.95, 11.06), respectively. OR of HGD and IC for MPD ≥10 mm cutoff were 4.36 (95% CI: 3.20, 5.93) and 4.75 (95% CI: 2.39, 9.45), respectively. IPMN with MPD of >5 mm could very likely be malignant. In selected IPMN patients, pancreatectomy should be considered when MPD is >5 mm.

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Intraductal Papillary Mucinous Neoplasm

Journal of Insurance Medicine ◽

10.17849/0743-6661-46.2.71 ◽

2016 ◽

Vol 46 (2) ◽

pp. 71-74

Author(s):

David S. Williams

Keyword(s):

Intraductal Papillary Mucinous Neoplasm ◽

Invasive Cancer ◽

Pancreatic Ducts ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms

Intraductal papillary mucinous neoplasms are cystic growths often noted incidentally in the pancreatic ducts. Left untreated, some may progress to invasive cancer.

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Successful management of life-threatening bleeding of intraductal papillary mucinous neoplasms in the pancreatic head

SAGE Open Medical Case Reports ◽

10.1177/2050313x17741014 ◽

2017 ◽

Vol 5 ◽

pp. 2050313X1774101 ◽

Cited By ~ 2

Author(s):

Hiroyuki Tokue ◽

Hideo Morita ◽

Azusa Tokue ◽

Yoshito Tsushima

Keyword(s):

Intraductal Papillary Mucinous Neoplasm ◽

Pancreatic Head ◽

Endovascular Embolization ◽

Fistula Formation ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms ◽

Risk Of Rupture ◽

Abdominal Hemorrhage

Complications associated with intraductal papillary mucinous neoplasms, such as acute pancreatitis, perforation, and fistula formation, have been documented. Intraductal papillary mucinous neoplasm with intratumoral hemorrhage is rare. To the best of our knowledge, there have been no previous reports of intraductal papillary mucinous neoplasm rupture and bleeding with intra-abdominal hemorrhage. A 74-year-old woman complained of acute upper right abdominal pain. She was under follow-up for an intraductal papillary mucinous neoplasm in the pancreatic head. Contrast-enhanced computed tomography revealed intraductal papillary mucinous neoplasm rupture and bleeding with intra-abdominal hemorrhage. The bleeding was treated with selective endovascular embolization of a branch of the gastroduodenal artery. Follow-up examinations are recommended even for intraductal papillary mucinous neoplasm patients without malignant findings because of the potential risk of rupture and bleeding with intra-abdominal hemorrhage. Clinicians should be aware of this possibility to ensure that patients are appropriately treated.

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Intrahepatic Biliary Intraductal Papillary Mucinous Neoplasm With Intermittent Jaundice: Case Report

International Surgery ◽

10.9738/intsurg-d-15-00142.1 ◽

2017 ◽

Author(s):

Omer Cennet ◽

Ahmet Bulent Dogrul ◽

Mehmet Bulent Tırnaksz ◽

Halit Uner ◽

Aytekin Akyol ◽

...

Keyword(s):

Case Report ◽

Intraductal Papillary Mucinous Neoplasm ◽

Invasive Carcinoma ◽

Malignant Potential ◽

Clinical Entity ◽

Intraductal Papillary Mucinous Neoplasms ◽

Ductal System ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms

Abstract Intraductal papillary mucinous neoplasms (IPMNs) represent a relatively new clinical entity. IPMNs have malignant potential which exhibit a broad histological spectrum, ranging from adenoma to invasive carcinoma. Although IPMNs are commonly seen within pancreas, they may develop anywhere within the biliary ductal system and produce mucin which may cause intermittent jaundice. This report details the case of 50 year-old woman presenting with intermittent jaundice who was diagnosed as hepatic IPMN.

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Intraductal Papillary Mucinous Neoplasms of The Pancreas: A Nationwide Registry-Based Study

Scandinavian Journal of Surgery ◽

10.1177/1457496918766727 ◽

2018 ◽

Vol 107 (4) ◽

pp. 302-307 ◽

Cited By ~ 2

Author(s):

L. Aronsson ◽

B. Andersson ◽

R. Andersson ◽

B. Tingstedt ◽

S. O. Bratlie ◽

...

Keyword(s):

Intraductal Papillary Mucinous Neoplasm ◽

Biliary Obstruction ◽

Invasive Disease ◽

National Registry ◽

Periampullary Cancer ◽

High Grade ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Pancreatic Resections ◽

Mucinous Neoplasms

Background and Aims: To investigate the paraclinical and pathological features of surgically resected intraductal papillary mucinous neoplasms in Sweden. Materials and Methods: A review of prospectively collected data on patients undergoing pancreatic resection for a histopathologically verified intraductal papillary mucinous neoplasm between 2010 and 2016 was performed using the Swedish National Registry for Pancreatic and Periampullary Cancer. Results: A total of 3038 pancreatic resections were performed during the study period, of which 251 (8.3%) were due to intraductal papillary mucinous neoplasms. The intraductal papillary mucinous neoplasm cases comprised 227 noninvasive and 24 invasive lesions. There was an annual increase in the number of resected intraductal papillary mucinous neoplasms, from 13 in 2010 to 56 in 2016, and an increase in the proportion of intraductal papillary mucinous neoplasm to the total number of pancreatic resections (4.7%–11%). Biliary obstruction was the only independent predictor of invasive disease, with odds ratio 3.106 (p = 0.030). There was no difference in survival between low-, intermediate-, and high-grade dysplastic lesions (p = 0.417). However, once invasive, the prognosis was severely impacted (p < 0.001). Three-year survival was 90% for noninvasive intraductal papillary mucinous neoplasm and 39% for invasive intraductal papillary mucinous neoplasm. Survival was better in lymph node negative invasive intraductal papillary mucinous neoplasm (p = 0.021), but still dismal compared to noninvasive lesions (p < 0.001). Conclusion: The number of surgically resected intraductal papillary mucinous neoplasms is increasing in Sweden. Biliary obstruction is associated with invasive disease. Low-to-high-grade dysplastic intraductal papillary mucinous neoplasm has an excellent prognosis, while invasive intraductal papillary mucinous neoplasm has a poor survival rate.

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Risk for Colorectal Adenomas Among Patients with Pancreatic Intraductal Papillary Mucinous Neoplasms: a Prospective Case- Control Study

Journal of Gastrointestinal and Liver Diseases ◽

10.15403/jgld.2014.1121.244.rsk ◽

2015 ◽

Vol 24 (4) ◽

pp. 445-450

Author(s):

Nikola Panic ◽

Gabriele Capurso ◽

Fabia Attili ◽

Giovanna Vitale ◽

Serena Stigliano ◽

...

Keyword(s):

Colorectal Cancer ◽

Intraductal Papillary Mucinous Neoplasm ◽

Colorectal Polyps ◽

Colorectal Adenomas ◽

Intraductal Papillary Mucinous Neoplasms ◽

Mucinous Neoplasm ◽

Increased Risk ◽

Mucinous Neoplasms ◽

First Time ◽

Control Study

Background & Aims: It has been reported that patients with intraductal papillary mucinous neoplasms of the pancreas are at an increased risk of colorectal cancer. The aim of our study was to investigate whether patients with intraductal papillary mucinous neoplasms are at a higher risk of colorectal adenomas with respect to the general population, as this condition represents the precursor of sporadic colorectal cancer. Methods: A case–control study was conducted at the Catholic University and University Sapienza, Rome, Italy. The cases were patients with intraductal papillary mucinous neoplasms without history of colorectal cancer, who had underwent screening colonoscopy for the first time. The controls were individuals who had underwent first time colonoscopy for screening or evaluation of non-specific abdominal symptoms. Chi-square and Fisher tests were used to compare the distributions of categorical variables. Results: We enrolled 122 cases and 246 controls. Colorectal polyps were found in 52 cases (42.6%) and 79 controls (32.1%) (p<0.05). In 29 cases (23.8%) and 57 controls (23.2%) histological examination disclosed adenomatous polyps (p=0.90). There was no difference between the groups in relation to the presence of polyps with low-grade (19.7% vs. 19.8%, p=0.98) and high-grade dysplasia (4.9% vs. 4.5%, p=0.85). Conclusion: Patients with intraductal papillary mucinous neoplasms of the pancreas are not at an increased risk for the development of adenomatous colorectal polyps. Abbreviations: BD-IPMN: branch duct intraductal papillary mucinous neoplasm; CRC: colorectal cancer; FAP: familiar adenomatous polyposis; FNA: fine needle aspiration; FOBT: fecal occult blood test; HNPCC: hereditary non-polyposis colorectal cancer; IPMN: intraductal papillary mucinous neoplasm; M-IPMN: mixed intraductal papillary mucinous neoplasm; MD-IPMNs: main duct intraductal papillary mucinous neoplasm; PDAC: pancreatic ductal adenocarcinoma; S-MRCP: magnetic resonance cholangiopancreatography with secretin stimulation.

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