|
1
|
Gong XH, Xu JR, Qian LJ. Atypical and uncommon CT and MR imaging presentations of pancreatic ductal adenocarcinoma. Abdom Radiol (NY) 2021; 46:4226-4237. [PMID: 33914139 DOI: 10.1007/s00261-021-03089-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Revised: 04/04/2021] [Accepted: 04/10/2021] [Indexed: 10/21/2022]
Abstract
Pancreatic ductal adenocarcinomas (PDACs) occasionally have atypical and uncommon imaging presentations that can present a diagnostic dilemma and result in false interpretation. This article aimed to illustrate these CT and MR imaging findings, including isoattenuating PDAC, coexisting acute pancreatitis, PDAC with a cystic feature, groove PDAC, diffuse PDAC, hypointensity on diffusion-weighted imaging (DWI), multifocal PDAC, intratumoral calcification, and extrapancreatic invasion with a barely discernable mass. A subset of PDACs with atypical features are occasionally encountered during routine clinical practice. Knowledge of and attention to these atypical and uncommon variable imaging features may allow radiologists to avoid misinterpretation and a delayed diagnosis.
Collapse
|
|
2
|
Gao J, Han F, Jin Y, Wang X, Zhang J. A Radiomics Nomogram for the Preoperative Prediction of Lymph Node Metastasis in Pancreatic Ductal Adenocarcinoma. Front Oncol 2020; 10:1654. [PMID: 32974205 PMCID: PMC7482654 DOI: 10.3389/fonc.2020.01654] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2020] [Accepted: 07/28/2020] [Indexed: 12/17/2022] Open
Abstract
PURPOSE To construct and verify a CT-based multidimensional nomogram for the evaluation of lymph node (LN) status in pancreatic ductal adenocarcinoma (PDAC). MATERIALS AND METHODS We retrospectively assessed data from 172 patients with clinicopathologically confirmed PDAC surgically resected between February 2014 and November 2016. Patients were assigned to either a training cohort (n = 121) or a validation cohort (n = 51). We acquired radiomics features from the preoperative venous phase (VP) CT images. The maximum relevance-minimum redundancy (mRMR) algorithm and the least absolute shrinkage and selection operator (LASSO) methods were used to select the optimal features. We used multivariable logistic regression to construct a combined radiomics model for visualization in the form of a nomogram. Performance of the nomogram was evaluated by the receiver operating characteristic (ROC) curve approach, calibration testing, and analysis of clinical usefulness. RESULTS A Rad score consisting of 10 LN status-related radiomics features was found to be significantly associated with the actual LN status (P < 0.01). A nomogram that consisted of Rad scores, CT-reported parenchymal atrophy, and CT-reported LN status performed well in terms of predictive power in the training cohort (area under the curve, 0.92), and this was confirmed in the validation cohort (area under the curve, 0.95). The nomogram also performed well in the calibration test and decision curve analysis, demonstrating its potential clinical value. CONCLUSION A multidimensional radiomics nomogram consisting of Rad scores, CT-reported parenchymal atrophy, and CT-reported LN status may contribute to the non-invasive evaluation of LN status in PDAC patients.
Collapse
Affiliation(s)
| | | | | | | | - Jiawen Zhang
- Department of Radiology, Huashan Hospital, Fudan University, Shanghai, China
| |
Collapse
|
|
3
|
Lorenzo D, Rebours V, Maire F, Palazzo M, Gonzalez JM, Vullierme MP, Aubert A, Hammel P, Lévy P, Mestier LD. Role of endoscopic ultrasound in the screening and follow-up of high-risk individuals for familial pancreatic cancer. World J Gastroenterol 2019; 25:5082-5096. [PMID: 31558858 PMCID: PMC6747297 DOI: 10.3748/wjg.v25.i34.5082] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2019] [Revised: 08/04/2019] [Accepted: 08/24/2019] [Indexed: 02/06/2023] Open
Abstract
Managing familial pancreatic cancer (FPC) is challenging for gastroenterologists, surgeons and oncologists. High-risk individuals (HRI) for pancreatic cancer (PC) (FPC or with germline mutations) are a heterogeneous group of subjects with a theoretical lifetime cumulative risk of PC over 5%. Screening is mainly based on annual magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS). The goal of screening is to identify early-stage operable cancers or high-risk precancerous lesions (pancreatic intraepithelial neoplasia or intraductal papillary mucinous neoplasms with high-grade dysplasia). In the literature, target lesions are identified in 2%-5% of HRI who undergo screening. EUS appears to provide better identification of small solid lesions (0%-46% of HRI) and chronic-pancreatitis-like parenchymal changes (14%-77% of HRI), while MRI is probably the best modality to identify small cystic lesions (13%-49% of HRI). There are no specific studies in HRI on the use of contrast-enhanced harmonic EUS. EUS can also be used to obtain tissue samples. Nevertheless, there is still limited evidence on the accuracy of imaging procedures used for screening or agreement on which patients to treat. The cost-effectiveness of screening is also unclear. Certain new EUS-related techniques, such as searching for DNA abnormalities or protein markers in pancreatic fluid, appear to be promising.
Collapse
Affiliation(s)
- Diane Lorenzo
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
| | - Vinciane Rebours
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
- INSERM, UMR1149, Paris 92110, France
| | - Frédérique Maire
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
| | - Maxime Palazzo
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
| | - Jean-Michel Gonzalez
- Departement of Gastroenterology, Aix Marseille university - APHM - Hôpital Nord, Marseille 13000, France
| | - Marie-Pierre Vullierme
- Radiology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 92110, France
| | - Alain Aubert
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
| | - Pascal Hammel
- Oncology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 92110, France
| | - Philippe Lévy
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
| | - Louis de Mestier
- Pancreatology Department, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Clichy, and Paris Diderot University, Paris 75013, France
- INSERM, UMR1149, Paris 92110, France
| |
Collapse
|
|
4
|
Matsuda Y. Age-related morphological changes in the pancreas and their association with pancreatic carcinogenesis. Pathol Int 2019; 69:450-462. [PMID: 31339204 DOI: 10.1111/pin.12837] [Citation(s) in RCA: 26] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2019] [Accepted: 06/26/2019] [Indexed: 12/14/2022]
Abstract
Age-related pathological changes in the pancreas have been unclear because they are often minor and nonspecific. However, recent studies have shown that they are closely related to various pathological conditions such as pancreatic cancer and diabetes mellitus. Knowledge of age-related changes is important to determine appropriate prevention, detection, and treatment strategies for various diseases observed in elderly patients. We present a review of the pathological age-related non-neoplastic changes in the exocrine pancreas such as pancreatic fatty replacement, lobulocentric pancreatic atrophy, pancreatic duct ectasia, and metaplasia of exocrine pancreas, as well as changes in islet cells. We have discussed common pancreatic neoplasms in elderly patients, such as pancreatic intraepithelial neoplasia (PanIN), intraductal papillary mucinous neoplasms (IPMNs), and pancreatic ductal adenocarcinoma (PDAC). Age-related pathological changes play a key role in pancreatic carcinogenesis via telomere dysfunction. Further studies are warranted to clarify molecular mechanisms of pancreatic carcinogenesis in elderly patients.
Collapse
Affiliation(s)
- Yoko Matsuda
- Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kagawa, Japan
| |
Collapse
|
|
5
|
Konings IC, Cahen DL, Harinck F, Fockens P, van Hooft JE, Poley JW, Bruno MJ. Evolution of features of chronic pancreatitis during endoscopic ultrasound-based surveillance of individuals at high risk for pancreatic cancer. Endosc Int Open 2018; 6:E541-E548. [PMID: 29713680 PMCID: PMC5909773 DOI: 10.1055/a-0574-2396] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/22/2017] [Accepted: 10/25/2017] [Indexed: 12/20/2022] Open
Abstract
BACKGROUND AND STUDY AIMS During endoscopic ultrasound (EUS)-based pancreatic ductal adenocarcinoma (PDAC)-surveillance in asymptomatic individuals, features of chronic pancreatitis (CP) are often detected. Little is known about the prevalence and progression of these features. The aim of this study was to quantify these features, assess the interobserver agreement, assess possible associated factors, and assess the natural course during 3 years of follow-up. PATIENTS AND METHODS Two experienced endosonographers reviewed anonymized sequential EUS videos of participants in PDAC surveillance that were obtained in 2012 and 2015 for features of CP. Descriptives, agreement analyses, univariate and multivariate analyses for possible risk factors, and repeated measures analyses to assess intra-individual changes over time were performed. RESULTS A total of 42 EUS videos of 21 participants were reviewed. Any feature of CP was present in 86 % (2012) and 81 % (2015) of participants, with a mean of 2.5 features per individual. The overall interobserver agreement was almost perfect at 83 %. No baseline factors were significantly associated with features of CP. Features did not change over time, except for hyperechoic foci without shadowing, which decreased intra-individually (β = - 1.6, P = 0.005). CONCLUSIONS This blinded study shows features of CP to be highly prevalent in individuals at high risk of developing pancreatic cancer. No baseline factors were associated with presence of these features. CP features did not increase intra-individually over a 3-year period. Longer follow-up and pathological examination of pancreatic resection specimens will be essential to learn whether EUS detection and follow-up of these CP features bear clinical relevance.
Collapse
Affiliation(s)
- Ingrid C.A.W. Konings
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands,Corresponding author Ingrid C.A.W. Konings Erasmus MC, University Medical Center RotterdamP.O. Box 20403000 CA RotterdamThe Netherlands+31-10-70-30331
| | - Djuna L. Cahen
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
| | - Femme Harinck
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
| | - Paul Fockens
- Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands
| | - Jeanin E. van Hooft
- Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands
| | - Jan-Werner Poley
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
| | - Marco J. Bruno
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
| |
Collapse
|
|
6
|
The Prevalence and Clinicopathological Characteristics of High-Grade Pancreatic Intraepithelial Neoplasia: Autopsy Study Evaluating the Entire Pancreatic Parenchyma. Pancreas 2017; 46:658-664. [PMID: 28196020 DOI: 10.1097/mpa.0000000000000786] [Citation(s) in RCA: 51] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
OBJECTIVE We sought to identify clinicopathological characteristics of high-grade pancreatic intraepithelial neoplasia (PanIN)/carcinoma in situ to facilitate screening for pancreatic ductal adenocarcinoma. METHODS We evaluated PanIN lesions in 173 consecutive autopsy cases with no evidence of pancreatic ductal adenocarcinoma and/or intraductal papillary mucinous neoplasm (mean age, 80.5 years) by submitting the entire pancreas for microscopic examination. RESULTS PanIN-3 was found in 4% of examined cases, whereas PanIN-1 and PanIN-2 were present in 77% and 28%, respectively. PanIN-3 was more frequently identified in patients with diabetes mellitus and/or older age. PanIN-3 lesions were always multifocal, and the number of PanIN-3 foci was positively associated with those of PanIN-1 or PanIN-2. PanIN-3 was located more frequently in the pancreatic body and tail than in the head and predominantly involved small interlobular/intralobular ducts rather than the main duct. Notably, 71% of pancreata with PanIN-3 showed cystic changes in PanIN-3 and lower grade PanIN lesions. PanIN-3 was also accompanied by higher grade extralobular fibrosis. CONCLUSIONS We found that 4% of the examined pancreata harbored PanIN-3 lesions that were associated with several unique clinicopathological features. The cystic change along with fibrotic pancreatic parenchyma may be detected by imaging studies such as endoscopic ultrasound.
Collapse
|
|
7
|
Fukumoto T, Watanabe T, Hirai I, Kimura W. Pancreatic volume is one of the independent prognostic factors for resectable pancreatic ductal adenocarcinomas. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2016; 23:472-9. [PMID: 27246905 DOI: 10.1002/jhbp.365] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/06/2016] [Accepted: 05/27/2016] [Indexed: 12/16/2022]
Abstract
BACKGROUND It is well known that pancreatic ductal adenocarcinoma (PDAC) is accompanied with pancreatic atrophy and fibrosis. We previously reported the correlation between pancreatic volume and body surface area (BSA) and significant reduction of BSA-adjusted pancreatic volume in pancreatic cancer patients. We evaluated potential correlation between BSA-adjusted pancreatic volume and PDAC prognosis. METHODS The study subjects were 48 pancreatic cancer patients received pancreatectomy at our department from June 2006 to September 2012. Pancreatic volumetry was retrospectively performed using the images obtained from multidetector computed tomography before the surgery. BSA-adjusted pancreatic volumes were calculated and analyzed for potential correlation with the prognosis. RESULTS Average BSA-adjusted pancreatic volume among 48 cases was 35.4 ± 11.9 ml/m(2) . Types of surgery included 24 cases with pancreaticoduodenectomy and 24 cases with distal pancreatectomy. The cases with BSA-adjusted pancreatic volume less than 40 ml/m(2) had significantly poorer prognosis compared to the cases of 40 ml/m(2) and greater (3-year survival rate: 32.4% vs. 64.3%). Statistical analysis identified four prognosis factors, i.e. BSA-adjusted pancreatic volume less than 40 ml/m(2) , postoperative adjuvant chemotherapy, lymph node metastasis and lymphatic invasion. CONCLUSIONS This study demonstrated BSA-adjusted pancreatic volume as a prognosis factor for PDAC and the volume of 40 ml/m(2) is considered to be the cutoff value.
Collapse
Affiliation(s)
- Tsuyoshi Fukumoto
- First Department of Surgery, Yamagata University Faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata City, Yamagata, 990-9585, Japan
| | - Toshihiro Watanabe
- First Department of Surgery, Yamagata University Faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata City, Yamagata, 990-9585, Japan
| | - Ichiro Hirai
- First Department of Surgery, Yamagata University Faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata City, Yamagata, 990-9585, Japan
| | - Wataru Kimura
- First Department of Surgery, Yamagata University Faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata City, Yamagata, 990-9585, Japan.
| |
Collapse
|
|
8
|
Esposito I, Segler A, Steiger K, Klöppel G. Pathology, genetics and precursors of human and experimental pancreatic neoplasms: An update. Pancreatology 2015; 15:598-610. [PMID: 26365060 DOI: 10.1016/j.pan.2015.08.007] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2015] [Revised: 08/02/2015] [Accepted: 08/12/2015] [Indexed: 12/11/2022]
Abstract
Over the past decade, there have been substantial improvements in our knowledge of pancreatic neoplasms and their precursor lesions. Extensive genetic analyses, recently using high-throughput molecular techniques and next-generation sequencing methodologies, and the development of sophisticated genetically engineered mouse models closely recapitulating human disease, have improved our understanding of the genetic basis of pancreatic neoplasms. These advances are paving the way for refined, molecular-based classifications of pancreatic neoplasms with the potential to better predict prognosis and, possibly, response to therapy. Another major development resides in the identification of subsets of pancreatic exocrine and endocrine neoplasms which occur in the context of hereditary syndromes and whose genetic basis and tumor development have been at least partially defined. However, despite all molecular progress, correct and careful morphological characterization of tissue specimens both in the context of experimental and routine diagnostic pathology represents the basis for any further genetic investigation or clinical decision. This review focuses on the current and new concepts of classification and on the current models of tumor development, both in the field of exocrine and endocrine neoplasms, and underscores the importance of applying standardized terminology to allow adequate data interpretation and promote scientific exchange in the field of pancreas research.
Collapse
Affiliation(s)
- Irene Esposito
- Institute of Pathology, Heinrich-Heine-University of Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany.
| | - Angela Segler
- Institute of Pathology, Technische Universität München, Ismaningerstr. 22, 81675, Munich, Germany
| | - Katja Steiger
- Institute of Pathology, Technische Universität München, Ismaningerstr. 22, 81675, Munich, Germany
| | - Günter Klöppel
- Institute of Pathology, Technische Universität München, Ismaningerstr. 22, 81675, Munich, Germany
| |
Collapse
|
|
9
|
Lami G, Biagini MR, Galli A. Endoscopic ultrasonography for surveillance of individuals at high risk for pancreatic cancer. World J Gastrointest Endosc 2014; 6:272-85. [PMID: 25031786 PMCID: PMC4094985 DOI: 10.4253/wjge.v6.i7.272] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2014] [Revised: 06/10/2014] [Accepted: 06/20/2014] [Indexed: 02/05/2023] Open
Abstract
Pancreatic cancer is a highly lethal disease with a genetic susceptibility and familial aggregation found in 3%-16% of patients. Early diagnosis remains the only hope for curative treatment and improvement of prognosis. This can be reached by the implementation of an intensive screening program, actually recommended for individuals at high-risk for pancreatic cancer development. The aim of this strategy is to identify pre-malignant precursors or asymptomatic pancreatic cancer lesions, curable by surgery. Endoscopic ultrasound (EUS) with or without fine needle aspiration (FNA) seems to be the most promising technique for early detection of pancreatic cancer. It has been described as a highly sensitive and accurate tool, especially for small and cystic lesions. Pancreatic intraepithelial neoplasia, a precursor lesion which is highly represented in high-risk individuals, seems to have characteristics chronic pancreatitis-like changes well detected by EUS. Many screening protocols have demonstrated high diagnostic yields for pancreatic pre-malignant lesions, allowing prophylactic pancreatectomies. However, it shows a high interobserver variety even among experienced endosonographers and a low sensitivity in case of chronic pancreatitis. Some new techniques such as contrast-enhanced harmonic EUS, computer-aided diagnostic techniques, confocal laser endomicroscopy miniprobe and the detection of DNA abnormalities or protein markers by FNA, promise improvement of the diagnostic yield of EUS. As the resolution of imaging improves and as our knowledge of precursor lesions grows, we believe that EUS could become the most suitable method to detect curable pancreatic neoplasms in correctly identified asymptomatic at-risk patients.
Collapse
|
|
10
|
Abstract
Over the past few years there have been substantial advances in our knowledge of premalignant lesions of the pancreas. Given the dismal prognosis of untreated pancreatic cancer, and the small proportion of patients who are operative candidates, an understanding of these premalignant lesions is essential for the development of strategies for early diagnosis and prevention. The 2010 WHO classification has added new entities, including intraductal tubular papillary neoplasms (ITPNs), and clarified the nomenclature and grading of previously recognised precursor lesions of pancreatic adenocarcinoma, such as intraductal papillary mucinous neoplasms (IPMNs), mucinous cystic neoplasms (MCNs) and pancreatic intraepithelial neoplasia (PanIN). In particular, there has been an upsurge of interest in the natural history of IPMN, driven partly by improvements in imaging modalities and the consequent apparent increase in their incidence, and partly by recognition that subtypes based on location or histological appearance define groups with significantly different behaviours. In mid 2012 revised international guidelines for the classification and management of IPMNs and MCNs were published, although in several respects these guidelines represent a consensus view rather than being evidence-based. In recent years major advances in molecular technologies, including whole-exome sequencing, have significantly enhanced our knowledge of pancreatic premalignancy and have identified potentially highly specific diagnostic biomarkers such as mutations in GNAS and RNF43 that could be used to pre-operatively assess pancreatic cysts.
Collapse
|
|
11
|
Abstract
This article reviews the genetics and incipient pathology of familial pancreatic cancer and the screening modalities in current use, and summarizes the outcomes of reported screening programs.
Collapse
Affiliation(s)
- Adam W Templeton
- Department of Gastroenterology, Digestive Diseases Center, University of Washington, Box Number 356424, 1959 Northeast Pacific Street, Seattle, WA 98195, USA.
| | | |
Collapse
|
|
12
|
Segura PP, Ponce CG, Ramón Y Cajal T, Blanch RS, Aranda E. Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management: a guideline of the TTD group. Clin Transl Oncol 2012; 14:553-63. [PMID: 22855135 DOI: 10.1007/s12094-012-0840-0] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2012] [Accepted: 04/19/2012] [Indexed: 12/16/2022]
Abstract
Pancreatic carcinoma (PC) represents the fourth leading cause of cancer death in Spain with a death rate of 2,400 males and 2,000 females per year. Poor outcome related to its silent nature and the lack of reliable secondary prevention measures translate into advanced-stage diagnosis, 75 % of deaths within the first year of diagnosis and 5-year survival rate of <5 %. Family history was first recognized as a risk factor for PC. Further population-based and case-control studies subsequently found that 7.8 % of patients with PC have a family history of the same tumor and individuals with a first-degree relative with PC have a 3.2-fold increased risk of developing PC. Overall, it is estimated that up to 10 % of PC have a familial component. However, known genetic syndromes account for <20 % of the observed familial aggregation of PC. We review the most important aspects in epidemiology, molecular biology and clinical management of familial PC.
Collapse
Affiliation(s)
- P Pérez Segura
- Medical Oncology, Clinical Hospital San Carlos, Madrid, Spain.
| | | | | | | | | |
Collapse
|
|
13
|
Precursor lesions of early onset pancreatic cancer. Virchows Arch 2011; 458:439-51. [PMID: 21369801 PMCID: PMC3062030 DOI: 10.1007/s00428-011-1056-3] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2010] [Revised: 02/03/2011] [Accepted: 02/07/2011] [Indexed: 02/07/2023]
Abstract
Early onset pancreatic cancer (EOPC) constitutes less than 5% of all newly diagnosed cases of pancreatic cancer (PC). Although histopathological characteristics of EOPC have been described, no detailed reports on precursor lesions of EOPC are available. In the present study, we aimed to describe histopathological picture of extratumoral parenchyma in 23 cases of EOPCs (definition based on the threshold value of 45 years of age) with particular emphasis on two types of precursor lesions of PC: pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasms (IPMNs). The types, grades, and densities of precursor lesions of PC were compared in patients with EOPCs, in young patients with neuroendocrine neoplasms (NENs), and in older (at the age of 46 or more) patients with PC. PanINs were found in 95.6% of cases of EOPCs. PanINs-3 were found in 39.1% of EOPC cases. Densities of all PanIN grades in EOPC cases were larger than in young patients with NENs. Density of PanINs-1A in EOPC cases was larger than in older patients with PC, but densities of PanINs of other grades were comparable. IPMN was found only in a single patient with EOPC but in 20% of older patients with PC. PanINs are the most prevalent precursor lesions of EOPC. IPMNs are rarely precursor lesions of EOPC. Relatively high density of low-grade PanINs-1 in extratumoral parenchyma of patients with EOPC may result from unknown multifocal genetic alterations in pancreatic tissue in patients with EOPCs.
Collapse
|
|
14
|
Affiliation(s)
- Ralph H Hruban
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, 401 North Broadway, Weinberg 2242, Baltimore, MD 21231, USA.
| | | | | | | | | |
Collapse
|
|
15
|
Fujimori N, Nakamura T, Oono T, Igarashi H, Takahata S, Nakamura M, Tanaka M, Hayashi A, Aishima S, Ishigami K, Ogoshi K, Ito T, Takayanagi R. Adenocarcinoma involving the whole pancreas with multiple pancreatic masses. Intern Med 2010; 49:1527-32. [PMID: 20686284 DOI: 10.2169/internalmedicine.49.3347] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 77-year-old man was referred to our hospital for further investigation of pancreatic masses. Imaging studies revealed hypovascular masses in the pancreatic head and body. Total pancreatectomy was performed under the diagnosis of double primary pancreatic carcinomas. Macroscopic examination revealed 3 nodules: one each in the pancreatic head, body, and tail. Microscopically, all 3 lesions showed similar carcinoma cells, which communicated with each other via the intraductal component, indicating a single large tumor. Incidentally, we also identified an adenocarcinoma of the common bile duct (CBD). The final diagnosis was synchronous double cancer involving the whole pancreas and the CBD.
Collapse
Affiliation(s)
- Nao Fujimori
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka
| | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
16
|
Resection of four synchronous invasive ductal carcinomas in the pancreas head and body associated with pancreatic intraepithelial neoplasia: report of a case. Surg Today 2009; 39:1091-7. [PMID: 19997809 DOI: 10.1007/s00595-009-3990-0] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2008] [Accepted: 03/09/2009] [Indexed: 10/20/2022]
Abstract
This report describes a very rare case of four synchronous invasive ductal carcinomas (IDCs) in the pancreas head and body with possible multicentricity. The patient was a 75-year-old woman. Abdominal dynamic computed tomography showed four low-density masses (25 mm, 20 mm, 10 mm, and 10 mm in diameter) in the pancreas head and body. The patient underwent a pylorus-preserving subtotal pancreatoduodenectomy. Histologically, the discontinuity between the four tumors was confirmed; one tumor (20 mm) was moderately differentiated tubular adenocarcinoma, and the others (25 mm, 10 mm, and 10 mm) were papillary adenocarcinomas. Two smaller papillary adenocarcinomas were composed of abundant fibrosis, pancreatic intraepithelial neoplasia (PanIN) 2-3, and IDC with stromal invasion. PanIN-1-2 lesions proximal to the four IDCs were evident. The immunohistochemical staining by CK20, MUC1, and Ki-67 revealed apparently different features for 2 IDCs (25 mm and 20 mm) and somewhat differential features for three papillary adenocarcinomas. Therefore, the progression of PanIN to IDC and multicentric occurrences of these four IDCs were possible. In this report, we show that immunohistochemistry and the confirmation of the presence of PanINs in IDC were useful to some extent for the study of multiple pancreatic cancers.
Collapse
|
|
17
|
Shi C, Klein AP, Goggins M, Maitra A, Canto M, Ali S, Schulick R, Palmisano E, Hruban RH. Increased Prevalence of Precursor Lesions in Familial Pancreatic Cancer Patients. Clin Cancer Res 2009; 15:7737-7743. [PMID: 19996207 DOI: 10.1158/1078-0432.ccr-09-0004] [Citation(s) in RCA: 162] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
PURPOSE: Histologic findings in 51 pancreata resected from patients with a strong family history of pancreatic cancer were compared with the findings in 40 pancreata resected from patients with sporadic pancreatic cancer. None of the patients in the familial group had a known inherited syndrome other than familial pancreatic cancer. EXPERIMENTAL DESIGN: Precursor lesions, including pancreatic intraepithelial neoplasia (PanIN), intraductal papillary mucinous neoplasm (IPMN), and incipient IPMN, were quantified. Invasive cancers were classified using established histologic criteria. RESULTS: The individual precursor lesions identified in both groups were histologically similar. Precursor lesions were more common in the familial cases than in the sporadic cases. The relative rate of PanINs per square centimeter was 2.75-fold higher (95% confidence interval, 2.05-3.70; adjusted for age) in familial compared with sporadic cases. PanIN-3 lesions were more common in familial versus sporadic pancreatic cancer patients (relative rate, 4.20; 95% confidence interval, 2.22-7.93; adjusted for age). High-grade incipient IPMNs were only observed in the familial cases. Nine of the 51 (18%) familial pancreatic cancers and 4 of the 40 (10%) sporadic cancers arose in association with an IPMN. No significant differences were found in the types of invasive cancers. CONCLUSIONS: Noninvasive precursor lesions are more common in patients with a strong family history of pancreatic cancer than in patients with sporadic disease, and precursor lesions are of a higher grade in patients with a strong family history of pancreatic cancer. These findings can form a basis for the design of screening tests for the early detection of pancreatic neoplasia. (Clin Cancer Res 2009;15(24):7737-43).
Collapse
Affiliation(s)
- Chanjuan Shi
- Authors' Affiliations: The Sol Goldman Pancreatic Cancer Research Center and Departments of Pathology, Oncology, Medicine, and Surgery, The Johns Hopkins Medical Institutions; and Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland
| | | | | | | | | | | | | | | | | |
Collapse
|