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1.
G Ital Nefrol ; 41(4)2024 Aug 26.
Article in Italian | MEDLINE | ID: mdl-39243410

ABSTRACT

This article constitutes a review of the existing literature on the potential correlation between autosomal dominant polycystic kidney disease (ADPKD) and intraductal papillary mucinous neoplasms (IPMN) of the pancreas. Additionally, it presents a clinical case where familiarity for both pathologies was observed, derived from the direct experience of our clinic, reinforcing the hypothesis of a possible common pathogenetic pathway. The review focuses on the potential genetic correlation between these two pathologies within the realm of ciliopathies, emphasizing the importance of targeted screening and monitoring strategies to detect pancreatic complications early in patients with ADPKD. Furthermore, it highlights the complexity in the clinical management of these rare conditions and underscores the importance of early diagnosis in optimizing clinical outcomes.


Subject(s)
Adenocarcinoma, Mucinous , Carcinoma, Papillary , Pancreatic Intraductal Neoplasms , Polycystic Kidney, Autosomal Dominant , Humans , Adenocarcinoma, Mucinous/complications , Adenocarcinoma, Mucinous/diagnosis , Adenocarcinoma, Mucinous/pathology , Carcinoma, Papillary/complications , Carcinoma, Papillary/diagnosis , Carcinoma, Papillary/pathology , Pancreatic Intraductal Neoplasms/complications , Pancreatic Intraductal Neoplasms/diagnosis , Pancreatic Intraductal Neoplasms/pathology , Polycystic Kidney, Autosomal Dominant/complications , Polycystic Kidney, Autosomal Dominant/genetics
2.
Cancer Control ; 31: 10732748241263644, 2024.
Article in English | MEDLINE | ID: mdl-39293798

ABSTRACT

Intraductal papillary mucinous neoplasms (IPMNs) are a very common incidental finding during patient radiological assessment. These lesions may progress from low-grade dysplasia (LGD) to high-grade dysplasia (HGD) and even pancreatic cancer. The IPMN progression risk grows with time, so discontinuation of surveillance is not recommended. It is very important to identify imaging features that suggest LGD of IPMNs, and thus, distinguish lesions that only require careful surveillance from those that need surgical resection. It is important to know the management guidelines and especially the indications for surgery, to be able to point out in the report the findings that suggest malignant degeneration. The imaging tools employed for diagnosis and risk assessment are Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) with contrast medium. According to the latest European guidelines, MRI is the method of choice for the diagnosis and follow-up of patients with IPMN since this tool has a highest sensitivity in detecting mural nodules and intra-cystic septa. It plays a key role in the diagnosis of worrisome features and high-risk stigmata, which are associated with IPMNs malignant degeneration. Nowadays, the main limit of diagnostic tools is the ability to identify the precursor of pancreatic cancer. In this context, increasing attention is being given to artificial intelligence (AI) and radiomics analysis. However, these tools remain in an exploratory phase, considering the limitations of currently published studies. Key limits include noncompliance with AI best practices, radiomics workflow standardization, and clear reporting of study methodology, including segmentation and data balancing. In the radiological report it is useful to note the type of IPMN so as the morphological features, size, rate growth, wall, septa and mural nodules, on which the indications for surveillance and surgery are based. These features should be reported so as the surveillance time should be suggested according to guidelines.


Subject(s)
Magnetic Resonance Imaging , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Humans , Magnetic Resonance Imaging/methods , Risk Assessment/methods , Pancreatic Intraductal Neoplasms/diagnostic imaging , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Neoplasms/diagnostic imaging , Pancreatic Neoplasms/pathology , Carcinoma, Pancreatic Ductal/diagnostic imaging , Carcinoma, Pancreatic Ductal/pathology , Adenocarcinoma, Mucinous/diagnostic imaging , Adenocarcinoma, Mucinous/pathology , Radiomics
3.
Medicine (Baltimore) ; 103(33): e39323, 2024 Aug 16.
Article in English | MEDLINE | ID: mdl-39151506

ABSTRACT

The aim of this study was to evaluate the cutoff value for identifying malignance in main pancreatic duct (MPD)-involved intraductal papillary mucinous neoplasm (IPMN) with an MPD diameter ranging from 5 to 10 mm. Clinical-radiological characteristics of 142 patients, including MPD-involved IPMNs (n = 53) and branch-duct (BD)-IPMNs (n = 89) were analyzed. Logistic regression analysis was used to determine the risk factors of malignant IPMNs and invasive carcinoma. ROC curves were used to identify different cutoffs in terms of preoperative MPD values to predict the presence of invasive carcinoma as well as malignant IPMNs, and the prediction performance was evaluated. For MPD-involved IPMNs (5 mm < MPD < 10 mm), MPD diameter of 7.5 mm for discriminating malignant IPMNs (area under curve [AUC] = 0.67) and 7.7 mm for discriminating invasive IPMNs (AUC = 0.56) were found to be the optimal cutoff values at receiver operating characteristic curve (ROC) analysis. MPD > 7.5 mm and carbohydrate antigen19-9 (Ca19-9) > 37 U/ml were found to be predictors of malignant IPMNs at univariate, and MPD > 7.5 mm was a predictor in multivariate analysis in MPD-involved IPMNs. The AUC of the ROC curve of MPD (7.5 mm) combined with Ca19-9 in identifying malignant IPMNs was 0.73 in MPD-involved IPMNs. MPD (7.5 mm) combined with Ca19-9 performed well in identifying malignant IPMNs in MPD-involved IPMNs.


Subject(s)
Carcinoma, Pancreatic Ductal , Pancreatic Ducts , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Humans , Male , Female , Middle Aged , Pancreatic Ducts/pathology , Pancreatic Ducts/diagnostic imaging , Aged , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/diagnosis , Risk Factors , Pancreatic Intraductal Neoplasms/pathology , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/diagnosis , ROC Curve , Retrospective Studies , CA-19-9 Antigen/blood , Adult , Adenocarcinoma, Mucinous/pathology , Aged, 80 and over
4.
BMJ Case Rep ; 17(8)2024 Aug 29.
Article in English | MEDLINE | ID: mdl-39209745

ABSTRACT

Acinar cystic transformation (ACT) of the pancreas is a rare non-neoplastic cystic lesion. It is difficult to distinguish from more concerning pathology, particularly a side-branch duct intraductal papillary mucinous neoplasm (IPMN). All reported cases of ACT have been clinically benign with no evidence of recurrence or malignant transformation. The Fukuoka guidelines is a classification system designed to help guide the management of IPMNs and mucinous cystic neoplasms. We report a case of ACT that was preoperatively diagnosed as a suspected side-branch IPMN. Through the application of current guidelines, the patient underwent a Whipple's procedure. We highlight the difficulties in obtaining an accurate preoperative diagnosis in cases of ACT and the current radiological and cytological methods available to clinicians.


Subject(s)
Pancreatic Cyst , Pancreatic Intraductal Neoplasms , Humans , Diagnosis, Differential , Pancreas/pathology , Pancreas/diagnostic imaging , Pancreatic Cyst/diagnosis , Pancreatic Cyst/pathology , Pancreatic Intraductal Neoplasms/diagnosis , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/surgery , Tomography, X-Ray Computed
5.
Cesk Patol ; 60(2): 81-89, 2024.
Article in English | MEDLINE | ID: mdl-39138010

ABSTRACT

The current WHO classification of digestive system tumours (2019) has presented the concept of diagnostics of intraductal and cystic neoplasms of the pancreas mostly based on integrated molecular data and evaluations of their malignant potential. Intraductal pancreatic neoplasms with ductal phenotype include microscopic precursor lesions of pancreatic ductal adenocarcinoma - the pancreatic intraepithelial neoplasia and macroscopic precursor lesions of pancreatic cancer, where intraductal papillary mucinous neoplasm represents the most common neoplasm of the pancreas with cystic appearance. Both intraductal oncocytic papillary neoplasm and intraductal tubulopapillary neoplasm are now classified as separate entities associated with less aggressive subtypes of pancreatic carcinoma and better prognosis. Clinical significance of microscopic pancreatic intraepithelial neoplasias is limited, in contrast to other intraductal neoplasms, which are presented as cystic and/or solid tumours by imaging methods with important consequences for further treatment and indication of surgical therapy (resection versus "watch and wait" strategies). Neoplasms of nonductal origin, such as acinar cell carcinomas and neuroendocrine neoplasms, can uncommonly display an intraductal growth and their correct classification has a great clinical importance. Moreover, differential diagnostics of cystic pancreatic lesions include not only cystic and pseudocystically transformed neoplasms, but also a large spectrum of reactive, inflammatory and dysontogenetic cystic lesions.


Subject(s)
Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Humans , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/diagnosis , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/diagnosis , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/diagnosis , Diagnosis, Differential
7.
Am J Case Rep ; 25: e944405, 2024 Aug 08.
Article in English | MEDLINE | ID: mdl-39113281

ABSTRACT

BACKGROUND Minimally invasive pancreatectomy has become the standard practice for the management of benign and malignant pancreatic tumors. Techniques such as robotic and laparoscopic approaches are known to reduce morbidity by offering benefits such as less blood loss, reduced pain, shorter hospital stays, and quicker recovery times. The indication for repeated minimally invasive pancreatectomy for recurrent or de novo pancreatic neoplasm after primary pancreatic surgery remains debated. CASE REPORT A 50-year-old woman was admitted to our hospital with a diagnosis of an intraductal papillary mucinous neoplasm in the pancreatic head. In 2010, she underwent laparoscopic single-branch resection for a branch-type tumor in the pancreatic uncinate process. During a 5-year follow-up, a de novo intraductal papillary mucinous neoplasm was detected, showing gradual growth and the presence of a mural nodule over the next 7 years. The patient's CEA level was elevated to 7.0 ng/mL. Considering the tumor's progression and the appearance of a mural nodule, we recommended a robot-assisted Whipple procedure. The operation began with laparoscopic adhesiolysis. After detachment of the adhesions and remobilization of the duodenum using the Kocher maneuver, the operation continued with the Da Vinci surgical system. The postoperative period was uneventful, and the patient was discharged on postoperative day 20. Pathological examination revealed intraductal papillary mucinous carcinoma in situ with negative resection margins. CONCLUSIONS This case verifies the safety and feasibility of performing a robotic Whipple procedure for a newly diagnosed pancreatic neoplasm in patients who have previously undergone minimally invasive pancreatic surgery.


Subject(s)
Pancreatectomy , Pancreatic Neoplasms , Humans , Female , Middle Aged , Pancreatectomy/methods , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/pathology , Laparoscopy , Reoperation , Pancreatic Intraductal Neoplasms/surgery , Robotic Surgical Procedures , Adenocarcinoma, Mucinous/surgery , Adenocarcinoma, Mucinous/pathology , Carcinoma, Pancreatic Ductal/surgery
8.
Mod Pathol ; 37(9): 100554, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38950698

ABSTRACT

Intraductal oncocytic papillary neoplasm (IOPN) of the pancreas is a recently recognized pancreatic tumor. Here, we aimed to determine its most essential features with the systematic review tool. PubMed, Scopus, and Embase were searched for studies reporting data on pancreatic IOPN. The clinicopathologic, immunohistochemical, and molecular data were extracted and summarized. Then, a comparative analysis of the molecular alterations of IOPN with those of pancreatic ductal adenocarcinoma and intraductal papillary mucinous neoplasm from reference cohorts (including The Cancer Genome Atlas) was conducted. The key findings from 414 IOPNs were as follows: 1) The male-to-female ratio was 1.5:1. Pancreatic head was the most common site (131/237; 55.3%), but a diffuse tumor extension involving more than one pancreatic segment was described in about 1 out of 5 cases (49/237; 20.6%). The mean size was 45.5 mm. An associated invasive carcinoma was present in 50% of cases (168/336). In those cases, most tumors were pT1 or pT2 and pN0 (>80%), and vascular invasion was uncommon (20.6%). Regarding survival, more than 90% of patients were alive after surgical resection. 2) Immunohistochemical and molecular features were as follows. The most commonly expressed mucins were MUC5AC (110/112; 98.2%) and MUC6 (78/84; 92.8%). Compared with pancreatic ductal adenocarcinoma and intraductal papillary mucinous neoplasm, the classic pancreatic drivers KRAS, TP53, CDKN2A, SMAD4, and GNAS were less altered in IOPN (P < .01). Moreover, fusions involving PRKACA or PRKACB gene were detected in all of the 68 cases examined, with PRKACB::ATP1B1 being the most common (27/68 cases; 39.7%). These genomic events emerged as an entity-defining molecular alteration of IOPN (P < .01). Thus, such fusions represent a promising biomarker for diagnostic purposes. Recent evidence also suggests their role in influencing the acquisition of oncocytic morphology. IOPN is a distinct pancreatic neoplasm with specific clinicopathologic and molecular features. Considering the clinical or prognostic implications, its recognition is essential for pathologists and, ultimately, patients' management.


Subject(s)
Carcinoma, Pancreatic Ductal , Pancreatic Intraductal Neoplasms , Humans , Carcinoma, Pancreatic Ductal/genetics , Carcinoma, Pancreatic Ductal/pathology , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/genetics , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/genetics , Male , Biomarkers, Tumor/genetics , Biomarkers, Tumor/analysis , Female , Carcinoma, Papillary/pathology , Carcinoma, Papillary/genetics , Middle Aged
9.
Ann Surg Oncol ; 31(10): 7023-7032, 2024 Oct.
Article in English | MEDLINE | ID: mdl-38961040

ABSTRACT

BACKGROUND: The clinico-oncological outcomes of precursor epithelial subtypes of adenocarcinoma arising from intraductal papillary mucinous neoplasms (A-IPMN) are limited to small cohort studies. Differences in recurrence patterns and response to adjuvant chemotherapy between A-IPMN subtypes are unknown. METHODS: Clincopathological features, recurrence patterns and long-term outcomes of patients undergoing pancreatic resection (2010-2020) for A-IPMN were reported from 18 academic pancreatic centres worldwide. Precursor epithelial subtype groups were compared using uni- and multivariate analysis. RESULTS: In total, 297 patients were included (median age, 70 years; male, 78.9%), including 54 (18.2%) gastric, 111 (37.3%) pancreatobiliary, 80 (26.9%) intestinal and 52 (17.5%) mixed subtypes. Gastric, pancreaticobiliary and mixed subtypes had comparable clinicopathological features, yet the outcomes were significantly less favourable than the intestinal subtype. The median time to recurrence in gastric, pancreatobiliary, intestinal and mixed subtypes were 32, 30, 61 and 33 months. Gastric and pancreatobiliary subtypes had worse overall recurrence (p = 0.048 and p = 0.049, respectively) compared with the intestinal subtype but gastric and pancreatobiliary subtypes had comparable outcomes. Adjuvant chemotherapy was associated with improved survival in the pancreatobiliary subtype (p = 0.049) but not gastric (p = 0.992), intestinal (p = 0.852) or mixed subtypes (p = 0.723). In multivariate survival analysis, adjuvant chemotherapy was associated with a lower likelihood of death in pancreatobiliary subtype, albeit with borderline significance [hazard ratio (HR) 0.56; 95% confidence interval (CI) 0.31-1.01; p = 0.058]. CONCLUSIONS: Gastric, pancreatobiliary and mixed subtypes have comparable recurrence and survival outcomes, which are inferior to the more indolent intestinal subtype. Pancreatobiliary subtype may respond to adjuvant chemotherapy and further research is warranted to determine the most appropriate adjuvant chemotherapy regimens for each subtype.


Subject(s)
Adenocarcinoma, Mucinous , Neoplasm Recurrence, Local , Pancreatic Neoplasms , Humans , Male , Female , Aged , Neoplasm Recurrence, Local/pathology , Chemotherapy, Adjuvant , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/drug therapy , Survival Rate , Follow-Up Studies , Middle Aged , Prognosis , Adenocarcinoma, Mucinous/pathology , Adenocarcinoma, Mucinous/drug therapy , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/drug therapy , Carcinoma, Pancreatic Ductal/surgery , Pancreatic Intraductal Neoplasms/pathology , Pancreatectomy , Adenocarcinoma/pathology , Adenocarcinoma/drug therapy , Retrospective Studies , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Aged, 80 and over , Stomach Neoplasms/pathology , Stomach Neoplasms/drug therapy
11.
Gan To Kagaku Ryoho ; 51(6): 655-657, 2024 Jun.
Article in Japanese | MEDLINE | ID: mdl-39009525

ABSTRACT

An 87-year-old woman presented to the emergency department with left thigh pain, and sciatic nerve pain was diagnosed. A chest CT scan showed bronchiectasis and tree-in buds and an acid-fast stain test of gastric juice was positive; further, M. avium-PCR of sputum and culture results were positive leading to a diagnosis of pulmonary nontuberculous mycobacterial infection(NTM). Abdominal CT showed dilatation of the main pancreatic duct and a multifocal cystic tumor in the pancreatic tail, which was found to be complicated with an intraductal papillary mucinous tumor(IPMN).


Subject(s)
Mycobacterium Infections, Nontuberculous , Pancreatic Neoplasms , Humans , Female , Aged, 80 and over , Pancreatic Neoplasms/complications , Mycobacterium Infections, Nontuberculous/complications , Mycobacterium Infections, Nontuberculous/diagnosis , Mycobacterium Infections, Nontuberculous/microbiology , Adenocarcinoma, Mucinous/complications , Adenocarcinoma, Mucinous/diagnosis , Pancreatic Intraductal Neoplasms/complications
12.
Dig Surg ; 41(3): 111-121, 2024.
Article in English | MEDLINE | ID: mdl-38981458

ABSTRACT

INTRODUCTION: Intraductal papillary mucinous neoplasm (IPMN) is an important precursor lesion of pancreatic cancer. Systemic inflammatory parameters are widely used in the prognosis prediction of cancer; however, their prognostic implications in IPMN with associated invasive carcinoma (IPMN-INV) are unclear. This study aims to explore the prognostic value of systemic inflammatory parameters in patients with IPMN-INV. METHODS: From 2015 to 2021, patients with pathologically confirmed IPMN who underwent surgical resection at Peking Union Medical College Hospital were enrolled. The clinical, radiological, and pathological data of the enrolled patients were collected and analyzed. Preoperative systemic inflammatory parameters were calculated as previously reported. RESULTS: Eighty-six patients with IPMN-INV met the inclusion criteria. The lymphocyte-to-monocyte ratio (LMR) was the only systemic inflammatory parameter independently associated with the cancer-specific survival (CSS). An LMR higher than 3.5 was significantly associated with a favorable CSS in univariate (hazard ratio [HR] 0.305, p = 0.003) and multivariate analyses (HR 0.221, p = 0.001). Other independently prognostic factors included the presence of clinical symptoms, cyst size, N stage, and tumor differentiation. Additionally, a model including LMR was established for the prognosis prediction of IPMN-INV and had a C-index of 0.809. CONCLUSIONS: Preoperative LMR could serve as a feasible prognostic biomarker for IPMN-INV. A decreased LMR (cutoff value of 3.5) was an independent predictor of poor survival for IPMN-INV.


Subject(s)
Lymphocytes , Monocytes , Pancreatic Neoplasms , Humans , Male , Female , Aged , Middle Aged , Pancreatic Neoplasms/mortality , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/blood , Pancreatic Neoplasms/pathology , Prognosis , Retrospective Studies , Carcinoma, Pancreatic Ductal/mortality , Carcinoma, Pancreatic Ductal/surgery , Carcinoma, Pancreatic Ductal/blood , Carcinoma, Pancreatic Ductal/pathology , Pancreatic Intraductal Neoplasms/mortality , Pancreatic Intraductal Neoplasms/surgery , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/blood , Neoplasm Invasiveness , Survival Rate , Hospitals, High-Volume , Adenocarcinoma, Mucinous/mortality , Adenocarcinoma, Mucinous/surgery , Adenocarcinoma, Mucinous/blood , Adenocarcinoma, Mucinous/pathology , Lymphocyte Count , Leukocyte Count
13.
Sci Rep ; 14(1): 17218, 2024 07 26.
Article in English | MEDLINE | ID: mdl-39060387

ABSTRACT

The primary aim of this investigation was to leverage radiomics features derived from contrast-enhanced abdominal computed tomography (CT) scans to devise a predictive model to discern the benign and malignant nature of intraductal papillary mucinous neoplasms (IPMNs). Radiomic signatures were meticulously crafted to delineate benign from malignant IPMNs by extracting pertinent features from contrast-enhanced CT images within a designated training cohort (n = 84). Subsequent validation was conducted with data from an independent test cohort (n = 37). The discriminative ability of the model was quantitatively evaluated through receiver operating characteristic (ROC) curve analysis, with the integration of carefully selected clinical features to improve the comparative analysis. Arterial-phase images were utilized to construct a model comprising 8 features for distinguishing between benign and malignant cases. The model achieved an accuracy of 0.891 [95% confidence interval (95% CI), 0.816-0.996] in the cross-validation set and 0.553 (95% CI 0.360-0.745) in the test set. Conversely, employing 9 features from the venous-phase resulted in a model with a cross-validation accuracy of 0.862 (95%CI 0.777-0.946) and a test set accuracy of 0.801 (95% CI 0.653-0.950).Integrating the identified clinical features with imaging features yielded a model with a cross-validation accuracy of 0.934 (95% CI 0.879-0.990) and a test set accuracy of 0.904 (95% CI 0.808-0.999), thereby further improving its discriminatory ability. Our findings distinctly illustrate that venous-phase radiomics features eclipse arterial-phase radiomic features in terms of predictive accuracy regarding the nature of IPMNs. Furthermore, the synthesis and meticulous screening of clinical features with radiomic data significantly increased the diagnostic efficacy of our model, underscoring the pivotal importance of a comprehensive and integrated approach for accurate risk stratification in IPMN management.


Subject(s)
Contrast Media , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Tomography, X-Ray Computed , Humans , Female , Tomography, X-Ray Computed/methods , Male , Middle Aged , Aged , Pancreatic Intraductal Neoplasms/diagnostic imaging , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Neoplasms/diagnostic imaging , Pancreatic Neoplasms/pathology , Diagnosis, Differential , ROC Curve , Carcinoma, Pancreatic Ductal/diagnostic imaging , Carcinoma, Pancreatic Ductal/pathology , Adenocarcinoma, Mucinous/diagnostic imaging , Adenocarcinoma, Mucinous/pathology , Retrospective Studies , Radiomics
15.
Ann Surg ; 280(4): 683-692, 2024 Oct 01.
Article in English | MEDLINE | ID: mdl-38842190

ABSTRACT

OBJECTIVE: To quantify the rate of progression in surveilled cysts and assess what factors should indicate delayed resection. BACKGROUND: Side-branch intraductal papillary mucinous neoplasms (SB-IPMNs) are increasingly discovered, making it challenging to identify which patients require resection, thus avoiding inappropriate treatment. Most incidental lesions are surveyed, yet the consequences of that decision remain uncertain. METHODS: A prospectively maintained database of pancreatic cystic neoplasms was queried for patients with SB-IPMN. Patients with ≥2 imaging studies >6 months apart were included. Clinically relevant progression (CR-progression) was defined by symptoms, worrisome/high-risk stigmata, or invasive cancer (IC). Growth ≥5 mm in 2 years is considered CR-progression; size ≥3 cm alone is not. RESULTS: Between 1997 and 2023, 1337 patients were diagnosed with SB-IPMN. Thirty-seven (2.7%) underwent up-front surgery; 1000 (75.0%) had >6 months of surveillance.The rate of CR-progression was 15.3% (n = 153) based on size increase (n = 63, 6.3%), main-duct involvement (n = 48, 4.8%), symptoms (n = 8, 5.0%), or other criteria (n = 34, 3.4%). At a median follow-up of 6.6 years (interquartile range: 3.0-10.26), 17 patients (1.7%) developed IC. Those with CR-progression developed IC in 11.1% (n = 17) and high-grade dysplasia (HGD) in 6.5% (n = 10). Nearly half of the cancers were not contiguous with the surveyed SB-IPMN.Size ≥3 cm was not associated with HGD/IC ( P = 0.232). HGD/IC was least common in CR-progression determined by size growth (6.3%) versus main-duct involvement (24%) or other (43%, P < 0.001)Patients with CR-progression demonstrated improved survival (overall survival) with resection on time-to-event ( P < 0.001) and multivariate Cox regression (hazard ratio = 0.205, 0.096-0.439, P < 0.001) analyses. Overall survival was not improved with resection in all patients ( P = 0.244). CONCLUSIONS: CR-progression for SB-IPMNs is uncommon, with the development of cancer anywhere in the pancreas being rare. Initial size should not drive resection. Long-term and consistent nonoperative surveillance is warranted, with surgery currently reserved for CR-progression, knowing that the majority of these still harbor low-grade pathology.


Subject(s)
Disease Progression , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Humans , Male , Female , Aged , Middle Aged , Follow-Up Studies , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/mortality , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/surgery , Pancreatic Cyst/pathology , Pancreatic Cyst/surgery , Carcinoma, Pancreatic Ductal/surgery , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/mortality , Pancreatectomy/methods , Adenocarcinoma, Mucinous/pathology , Adenocarcinoma, Mucinous/surgery , Adult
17.
Hum Pathol ; 150: 36-41, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38914167

ABSTRACT

Intraductal tubulopapillary neoplasms (ITPNs) are rare pancreatic tumors with distinct histological and molecular features. Distinction of ITPN from other pancreatic neoplasms is crucial given the known favorable prognosis and the high frequency and diversity of potentially targetable fusions in ITPN. While the histological features of ITPN are well documented, there are few reports on the cytological features, and molecular characterization of ITPN. The authors reported three cases diagnosed in their laboratory between 2016 and 2021. Clinical data, cytomorphological and histological features, with immunophenotypic and molecular characterizations of these cases are described and compared with those reported in the literature. All 3 cases were diagnosed as ITPN based on the microscopic presence of intraductal nodules composed of tightly packed small tubular glands lined by cuboidal cells lacking apparent mucin. On molecular profiling KRAS and TP53 variants were found in Case 1, FGFR2-INA fusion in Case 2, and STARD3NL-BRAF fusion was detected in Case 3. Immunohistochemistry (IHC) revealed that the neoplastic cells in Case 1 were MUC2 positive and MUC6 negative, but in Cases 2 and 3, were negative for MUC2 and positive for MUC6. These results demonstrate the immunophenotypic and molecular variabilities of histologically similar pancreatic neoplasms. The absence of alterations characteristic of more common pancreatic neoplasms should prompt the consideration of fusion studies in morphologically relevant cases. The combination of morphological, IHC, and molecular analyses is important for reliable identification of ITPN given its potential clinical management implications.


Subject(s)
Biomarkers, Tumor , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Humans , Male , Middle Aged , Biomarkers, Tumor/genetics , Biomarkers, Tumor/analysis , Female , Aged , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/genetics , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/genetics , Carcinoma, Pancreatic Ductal/genetics , Carcinoma, Pancreatic Ductal/pathology , Immunohistochemistry
18.
Am J Surg Pathol ; 48(8): 1032-1040, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-38841868

ABSTRACT

Intraductal oncocytic papillary neoplasms (IOPNs) of the pancreatobiliary tract are considered a separate entity from intraductal papillary mucinous neoplasms (IPMNs), especially because of the distinct molecular alterations represented by PRKACA or PRKACB fusion. However, IOPNs display a spectrum of cytoarchitectural features. Typically, an IOPN is composed of arborizing papillae lined by layers of cells with oncocytic cytoplasm, prominent nucleoli, and intraepithelial lumina, while a significant subset shows atypical morphology: lack of the characteristic cytoarchitectural features such as arborizing papillae and prominent nucleoli, or mixture with nononcocytic IPMN-like components within a single lesion. To elucidate the tumorigenesis and morphologic spectrum of IOPNs, we analyzed 22 IOPNs, including those with atypical morphology for PRKACA/PRKACB fusions in each different component separately using fluorescence in situ hybridization. In total, 18 of 22 (82%) cases harbored PRKACA/PRKACB fusions, including 3 of 3 (100%) purely typical IOPNs and 15 of 19 (79%) IOPNs with atypical morphology. In the latter, PRKACA/PRKACB fusions were noted in atypical components as well as typical IOPN components. Notably, gastric-type IPMN-like components in the fusion-positive cases were usually low grade and had scattered neoplastic cells with eosinophilic cytoplasm, a morphologic feature suggestive of an early lesion of IOPN. In summary, most IOPNs with atypical morphology either lack characteristic cytoarchitectural features or exhibit a mixture with nononcocytic IPMN-like components, harbored PRKACA/PRKACB fusion as did typical IOPN components. Our observations expanded the morphologic spectrum of IOPNs. They are expected to be useful for correct diagnosis of this neoplasm.


Subject(s)
Biomarkers, Tumor , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits , Gene Fusion , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Bile Duct Neoplasms/genetics , Bile Duct Neoplasms/pathology , Biomarkers, Tumor/genetics , Carcinoma, Pancreatic Ductal/genetics , Carcinoma, Pancreatic Ductal/pathology , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/genetics , In Situ Hybridization, Fluorescence , Pancreatic Intraductal Neoplasms/genetics , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Neoplasms/genetics , Pancreatic Neoplasms/pathology , Phenotype
19.
BMC Gastroenterol ; 24(1): 201, 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38886637

ABSTRACT

BACKGROUND: Dilatation of common bile duct (CBD) is mostly pathological and mainly occurs secondary to mechanical causes. We aimed to explore the prevalence of CBD dilatation in Intraductal Papillary Mucinous Neoplasms of the pancreas (IPMN) among patients referred to EUS. METHODS: A retrospective study of all patients who had an EUS diagnosis of IPMN from 2011 to 2019 at Galilee Medical Center were extracted. Control group including patients with other types of pancreatic cysts. RESULTS: Overall, 2400 patients were included in the study, of them 158 patients were diagnosed with pancreatic cysts, 117 patients (74%) diagnosed with IPMN (group A), and 41 patients (26%) diagnosed with other pancreatic cysts (group B). Univariate analysis showed significant association of IPMN (OR 3.8, 95% CI 1.3-11.5), resected gallbladder (GB) (OR 7.75, 95% CI 3.19-18.84), and age (OR 1, 95% CI 1.01-1.08) with CBD dilatation. Classifying IPMN to sub-groups using adjusted multivariate logistic regression analysis, only main duct-IPMN (MD-IPMN) significantly correlated with CBD dilatation compared to branch duct (BD)-IPMN and mixed type-IPMN (OR 19.6, 95% CI 4.57-83.33, OR 16.3, 95% CI 3.02-88.08). CONCLUSION: MD-IPMN was significantly correlated with dilated CBD. Assessment of the pancreas is warranted in encountered cases of dilated CBD without obvious mechanical cause.


Subject(s)
Common Bile Duct , Humans , Male , Female , Retrospective Studies , Middle Aged , Dilatation, Pathologic/diagnostic imaging , Aged , Common Bile Duct/diagnostic imaging , Common Bile Duct/pathology , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/diagnostic imaging , Endosonography , Pancreatic Cyst/pathology , Pancreatic Cyst/diagnostic imaging , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/diagnostic imaging , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/diagnostic imaging , Adenocarcinoma, Mucinous/pathology , Adenocarcinoma, Mucinous/diagnostic imaging
20.
Pancreas ; 53(6): e521-e527, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38888840

ABSTRACT

OBJECTIVES: Endoscopic ultrasound (EUS) is an excellent diagnostic tool that provides high-resolution images of pancreatic cystic lesions. However, its role in the diagnosis of malignant intraductal papillary mucinous neoplasm (IPMN) remains limited and unclear. We aimed to determine the usefulness of this modality for such diagnosis. METHODS: Overall, 246 patients who underwent EUS for IPMN after computed tomography (CT)/magnetic resonance imaging (MRI) from April 2018 to June 2021 were followed up until March 2022. We assessed the added value of performing EUS after CT or MRI for diagnosing malignant IPMN, using receiver operating characteristic curve analysis. Walls as thick as 2 mm were considered thickened in this study if they were highly uneven. RESULTS: EUS clearly enhanced accuracy in identifying enhancing nodules and thickened walls. The areas under the receiver operating characteristic curve and corresponding 95% confidence intervals were 0.655 (0.549-0.760) and 0.566 (0.478-0.654) upon CT/MRI but 0.853 (0.763-0.942) and 0.725 (0.634-0.817) when observed using EUS. The combination of nodule size, thickened wall, and main duct size yielded the highest area under the receiver operating characteristic curve (0.944 [0.915-0.973]). CONCLUSIONS: EUS more accurately detects malignant IPMN, as uneven wall thickening and certain nodules cannot be identified with CT/MRI.


Subject(s)
Endosonography , Magnetic Resonance Imaging , Pancreatic Intraductal Neoplasms , Pancreatic Neoplasms , ROC Curve , Tomography, X-Ray Computed , Humans , Endosonography/methods , Male , Female , Aged , Middle Aged , Magnetic Resonance Imaging/methods , Tomography, X-Ray Computed/methods , Pancreatic Neoplasms/diagnostic imaging , Pancreatic Neoplasms/pathology , Pancreatic Intraductal Neoplasms/diagnostic imaging , Pancreatic Intraductal Neoplasms/pathology , Carcinoma, Pancreatic Ductal/diagnostic imaging , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/diagnosis , Adenocarcinoma, Mucinous/diagnostic imaging , Adenocarcinoma, Mucinous/pathology , Aged, 80 and over , Retrospective Studies , Adult , Reproducibility of Results
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