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Revista Espanola de Enfermedades... Mar 2022We present the case of a 37-year-old female with abdominal pain in the epigastrium radiating to the right flank of a month of evolution. On physical examination, the...
We present the case of a 37-year-old female with abdominal pain in the epigastrium radiating to the right flank of a month of evolution. On physical examination, the epigastric mass was palpated, firm and painless. Computed tomography (CT) showed a cystic tumor in the body and tail of the pancreas with solid areas and defined borders (12 x 10 cm), which displaced structures. Endoscopic ultrasound (EUS)-guided fine needle biopsy was performed, with a cytology consistent with solid pseudopapillary tumor (SPT). Subsequently, a distal pancreatectomy with tumor resection and nodal dissection were performed. Cytology reported discohesive cells, some arranged around capillaries, with small nuclei with clefts, CK7 negative and β-catenin positive. After four years of follow-up, there is no evidence of recurrence.
Topics: Abdominal Pain; Adult; Biopsy, Fine-Needle; Endosonography; Female; Humans; Pancreas; Pancreatectomy; Pancreatic Neoplasms
PubMed: 34689567
DOI: 10.17235/reed.2021.8345/2021 -
World Journal of Gastroenterology Aug 2021The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are... (Review)
Review
The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Many pediatric cancers may present pancreatic metastases, such as renal cell carcinoma, which may evolve with pancreatic metastatic disease even after two or more decades. Several childhood diseases may create a predisposition for the development of pancreatic cancer during adulthood; hence, there is a need for extensive screening strategies and complex programs to facilitate the transition from pediatric to adult healthcare. Nevertheless, genetic studies highlight the fact the specific gene mutations and family aggregations may be correlated with a special predisposition towards pancreatic cancer. This review aims to report the main pancreatic cancers diagnosed during childhood, the most important childhood diseases predisposing to the development of pancreatic malignancies, and the gene mutations associates with pancreatic malignant tumors.
Topics: Adult; Child; Humans; Insulinoma; Pancreas; Pancreatic Neoplasms; Survival Rate
PubMed: 34539135
DOI: 10.3748/wjg.v27.i32.5322 -
Cells Jun 2021Compared to pancreatic adenocarcinoma (PDAC), pancreatic neuroendocrine tumors (PanNET) represent a rare and heterogeneous tumor entity. In addition to surgical...
Compared to pancreatic adenocarcinoma (PDAC), pancreatic neuroendocrine tumors (PanNET) represent a rare and heterogeneous tumor entity. In addition to surgical resection, several therapeutic approaches, including biotherapy, targeted therapy or chemotherapy are applicable. However, primary or secondary resistance to current therapies is still challenging. Recent genome-wide sequencing efforts in PanNET identified a large number of mutations in pathways involved in epigenetic modulation, including acetylation. Therefore, targeting epigenetic modulators in neuroendocrine cells could represent a new therapeutic avenue. Detailed information on functional effects and affected signaling pathways upon epigenetic targeting in PanNETs, however, is missing. The primary human PanNET cells NT-3 and NT-18 as well as the murine insulinoma cell lines beta-TC-6 (mouse) and RIN-T3 (rat) were treated with the non-selective histone-deacetylase (HDAC) inhibitor panobinostat (PB) and analyzed for functional effects and affected signaling pathways by performing Western blot, FACS and qPCR analyses. Additionally, NanoString analysis of more than 500 potentially affected targets was performed. In vivo immunohistochemistry (IHC) analyses on tumor samples from xenografts and the transgenic neuroendocrine Rip1Tag2-mouse model were investigated. PB dose dependently induced cell cycle arrest and apoptosis in neuroendocrine cells in human and murine species. HDAC inhibition stimulated redifferentiation of human primary PanNET cells by increasing mRNA-expression of somatostatin receptors (SSTRs) and insulin production. In addition to hyperacetylation of known targets, PB mediated pleitropic effects via targeting genes involved in the cell cycle and modulation of the JAK2/STAT3 axis. The HDAC subtypes are expressed ubiquitously in the existing cell models and in human samples of metastatic PanNET. Our results uncover epigenetic HDAC modulation using PB as a promising new therapeutic avenue in PanNET, linking cell-cycle modulation and pathways such as JAK2/STAT3 to epigenetic targeting. Based on our data demonstrating a significant impact of HDAC inhibition in clinical relevant in vitro models, further validation in vivo is warranted.
Topics: Animals; Cell Line, Tumor; Histone Deacetylase Inhibitors; Histone Deacetylases; Humans; Mice; Neoplasm Proteins; Neuroectodermal Tumors; Pancreatic Neoplasms; Panobinostat; Rats
PubMed: 34204116
DOI: 10.3390/cells10061408 -
Frontiers in Cellular and Infection... 2022The intestinal microenvironment is composed of normal gut microbiota and the environment in which it lives. The largest microecosystem in the human body is the gut... (Review)
Review
The intestinal microenvironment is composed of normal gut microbiota and the environment in which it lives. The largest microecosystem in the human body is the gut microbiota, which is closely related to various diseases of the human body. Pancreatic cancer (PC) is a common malignancy of the digestive system worldwide, and it has a 5-year survival rate of only 5%. Early diagnosis of pancreatic cancer is difficult, so most patients have missed their best opportunity for surgery at the time of diagnosis. However, the etiology is not entirely clear, but there are certain associations between PC and diet, lifestyle, obesity, diabetes and chronic pancreatitis. Many studies have shown that the translocation of the gut microbiota, microbiota dysbiosis, imbalance of the oral microbiota, the interference of normal metabolism function and toxic metabolite products are closely associated with the incidence of PC and influence its prognosis. Therefore, understanding the correlation between the gut microbiota and PC could aid the diagnosis and treatment of PC. Here, we review the correlation between the gut microbiota and PC and the research progresses for the gut microbiota in the diagnosis and treatment of PC.
Topics: Carcinogenesis; Dysbiosis; Gastrointestinal Microbiome; Humans; Pancreas; Pancreatic Neoplasms; Tumor Microenvironment
PubMed: 35463649
DOI: 10.3389/fcimb.2022.872019 -
Cancer Reports (Hoboken, N.J.) Oct 2021Pancreatic adenocarcinoma (PDAC) is highly lethal. Surgery offers the only chance of cure, but 5-year overall survival (OS) after surgical resection and adjuvant therapy...
BACKGROUND
Pancreatic adenocarcinoma (PDAC) is highly lethal. Surgery offers the only chance of cure, but 5-year overall survival (OS) after surgical resection and adjuvant therapy remains dismal. Adjuvant trials were mostly conducted in the West enrolling fit patients. Applicability to a general population, especially Asia has not been described adequately.
AIM
We aimed to evaluate the clinical outcomes, prognostic factors of survival, pattern, and timing of recurrence after curative resection in an Asian institution.
METHODS AND RESULTS
The clinicopathologic and survival outcomes of 165 PDAC patients who underwent curative resection between 1998 and 2013 were reviewed retrospectively. Median age at surgery was 62.0 years. 55.2% were male, and 73.3% had tumors involving the head of pancreas. The median OS of the entire cohort was 19.7 months. Median OS of patients who received adjuvant chemotherapy was 23.8 months. Negative predictors of survival include lymph node ratio (LNR) of >0.3 (HR = 3.36, P = .001), tumor site involving the body or tail of pancreas (HR = 1.59, P = .046), presence of perineural invasion (PNI) (HR = 2.36, P = .018) and poorly differentiated/undifferentiated tumor grade (HR = 1.86, P = .058). The median time to recurrence was 8.87 months, with 66.1% and 81.2% of patients developing recurrence at 12 months and 24 months respectively. The most common site of recurrence was the liver.
CONCLUSION
The survival of Asian patients with resected PDAC who received adjuvant chemotherapy is comparable to reported randomized trials. Clinical characteristics seem similar to Western patients. Hence, geographical locations may not be a necessary stratification factor in RCTs. Conversely, lymph node ratio and status of PNI ought to be incorporated.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Pancreatic Ductal; Chemotherapy, Adjuvant; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Pancreatectomy; Pancreatic Neoplasms; Prognosis; Retrospective Studies; Singapore; Survival Rate
PubMed: 33939335
DOI: 10.1002/cnr2.1393 -
The Journal of International Medical... Aug 2021Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of...
Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of other tumours, making preoperative diagnosis a considerable challenge. We report correlative ultrasound, contrast-enhanced ultrasonography, contrast-enhanced magnetic resonance imaging, and positron emission tomography-computed tomography findings in a 60-year-old woman with PB. PB often presents with uncommon imaging features and should be considered in the differential diagnosis of pancreatic masses. It is important for clinicians to be aware of these differences to provide effective treatment.
Topics: Adult; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Pancreatic Neoplasms; Ultrasonography
PubMed: 34461770
DOI: 10.1177/03000605211039565 -
Diagnostic and Interventional Radiology... Mar 2022PURPOSE The purpose of this paper was to distinguish solid pseudopapillary neoplasms (SPNs) and nonfunctional neuroendocrine tumors (nf-NETs) of pancreas using...
PURPOSE The purpose of this paper was to distinguish solid pseudopapillary neoplasms (SPNs) and nonfunctional neuroendocrine tumors (nf-NETs) of pancreas using univariate analysis and clinical-CT logistic regression model. METHODS Twenty-eight patients with SPNs and 46 patients with nf-NETs underwent enhanced CT examinations. Clinical data (sex, age), categorical (location, cystic degeneration, calcification, hemorrhage, and enhancement pattern), and numeric CT features (lesion long diameter, long/ short diameter ratio, tumor attenuation values and tumor/pancreas attenuation ratios at unenhanced phase [UP], arterial phase [AP], and venous phase [VP]) were recorded. The logistic regression model was constructed by stepwise forward method of binary logistic regression after univariate analysis. The corresponding operating characteristic curve (ROC) and nomogram were delineated. The area under the curve (AUC), sensitivity, and specificity of ROC were calculated. RESULTS The SPNs were observed more often in relatively young (P < .001), female (P < .001) patients. After the univariate analysis, the categorical CT features of location (P = .048), hemorrhage (P = .003), and enhancement pattern (P = .004) and the numeric CT features of lesion long diameter (P = .005), tumor/pancreasUP (P = .002), tumorAP (P < .001), and tumor/pancreasAP (P < .001) had statistical significance. The AUC (95% CI), sensitivity, and specificity of a logistic regression model composed of age, tumor/pancreasUP, and tumor/pancreasAP were 0.933 (95% CI, 0.850-0.978), 84.78%, and 92.86%. CONCLUSION The SPNs often occurred in 20- to 40-year-old female patients, were located in the body or tail of pancreas, showed hemorrhagic degeneration, heterogeneous enhancement, and were relatively larger in size compared with nf-NETs. Tumor/pancreasUP, tumorAP, and tumor/pancreasAP values of SPNs were smaller than those of nf-NETs. The clinical-CT logistic regression model and nomogram consisting of age, tumor/pancreasUP, and tumor/pancreasAP parameters helped to differentiate SPNs from nf-NETs.
Topics: Adult; Diagnosis, Differential; Female; Humans; Neuroendocrine Tumors; Nomograms; Pancreas; Pancreatic Neoplasms; Retrospective Studies; Tomography, X-Ray Computed; Young Adult
PubMed: 35548896
DOI: 10.5152/dir.2022.20926 -
International Journal of Surgery... Dec 2023Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant pancreatic tumor with a highly favorable prognosis. Most SPN patients are young and...
Local resection for solid pseudopapillary neoplasms of the pancreas shows improved postoperative gastrointestinal function and reduced mental stress: a multiquestionnaire survey from a large cohort.
BACKGROUND
Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant pancreatic tumor with a highly favorable prognosis. Most SPN patients are young and middle-aged women. The main controversial topic for SPN is local resection (LR) versus radical resection (RR). Theoretically, LR could lead to better gastrointestinal function (GIF) and less mental stress. However, no data is available to support this hypothesis.
METHODS
All SPN patients undergoing surgical treatment in Peking Union Medical College Hospital from 2001 to 2021 were included in the study. A cross-sectional online multiquestionnaire survey containing 110 questions was sent to them (Clinicaltrial.org, NCT05604716). This online multiquestionnaire survey focused on GIF and mental stress and consisted of eight questionnaires. Multiple linear regression analysis was conducted to identify independent factors impacting GIF and mental stress.
RESULTS
A total of 183 cases provided valid results. Among them, 46 patients (25.1%) underwent LR, and 137 (74.9%) underwent RR. Ninety-four cases (51.4%) underwent minimally invasive surgery (MIS), while 89 (48.6%) underwent open surgery. The average GSRS score of the patients was 1.9±0.7, indicating that most suffered from mild gastrointestinal dysfunction. The scores of PHQ-9 and GAD-7 in 16 patients (8.7%) and 27 (14.8%) patients, respectively, were beyond 10.0, which indicated clinical depression and anxiety. Additionally, 19 (10.4%) patients reported poor ability to work, and 31(16.9%) patients had significant body image concerns. Compared to other clinicopathological characteristics, LR (LR vs. RR: PHQ-9 score, P =0.018; WAI average score, P =0.010; EORTC QLQ-C30, nine subdomains, P <0.05; GSRS average score, P =0.006) and MIS (MIS vs. open surgery: EORTC QLQ-C30, three subdomains, P <0.05; GSRS average score, P =0.006) were the most significant factors predicting improved GIF and reduced mental stress.
CONCLUSIONS
This study systematically presents postoperative GIF and mental stress of SPN patients using validated multiquestionnaires for the first time. It provides solid evidence that LR and MIS can improve GIF and reduce mental stress after surgery for SPN patients, which could be helpful for the surgeons to make more personalized surgical plans for their patients.
Topics: Middle Aged; Humans; Female; Pancreatectomy; Cross-Sectional Studies; Pancreatic Neoplasms; Pancreaticoduodenectomy; Neoplasms, Glandular and Epithelial; Surveys and Questionnaires; Pancreas
PubMed: 37830944
DOI: 10.1097/JS9.0000000000000702 -
Cancer Metastasis Reviews Sep 2021The ever-growing perception of cancer stem cells (CSCs) as a plastic state rather than a hardwired defined entity has evolved our understanding of the functional and... (Review)
Review
The ever-growing perception of cancer stem cells (CSCs) as a plastic state rather than a hardwired defined entity has evolved our understanding of the functional and biological plasticity of these elusive components in malignancies. Pancreatic cancer (PC), based on its biological features and clinical evolution, is a prototypical example of a CSC-driven disease. Since the discovery of pancreatic CSCs (PCSCs) in 2007, evidence has unraveled their control over many facets of the natural history of PC, including primary tumor growth, metastatic progression, disease recurrence, and acquired drug resistance. Consequently, the current near-ubiquitous treatment regimens for PC using aggressive cytotoxic agents, aimed at ''tumor debulking'' rather than eradication of CSCs, have proven ineffective in providing clinically convincing improvements in patients with this dreadful disease. Herein, we review the key hallmarks as well as the intrinsic and extrinsic resistance mechanisms of CSCs that mediate treatment failure in PC and enlist the potential CSC-targeting 'natural agents' that are gaining popularity in recent years. A better understanding of the molecular and functional landscape of PCSC-intrinsic evasion of chemotherapeutic drugs offers a facile opportunity for treating PC, an intractable cancer with a grim prognosis and in dire need of effective therapeutic advances.
Topics: Drug Resistance, Neoplasm; Humans; Neoplastic Stem Cells; Pancreas; Pancreatic Neoplasms; Prognosis
PubMed: 34453639
DOI: 10.1007/s10555-021-09979-x -
Genes Jan 2020Next-generation sequencing has led to the recent discovery of several novel pancreatic cancer susceptibility genes. These genes include ataxia telangiectasia mutated (),... (Review)
Review
Next-generation sequencing has led to the recent discovery of several novel pancreatic cancer susceptibility genes. These genes include ataxia telangiectasia mutated (), a serine/threonine kinase that is an integral component of DNA repair. Pathogenic germline variants are frequently identified in patients with pancreatic ductal adenocarcinoma (PDAC) with and without a family history of the disease. Loss of is also a frequent somatic event in the development of PDAC. These discoveries have advanced our understanding of the genetic basis of pancreatic cancer risk and will impact patient care through appropriate patient-risk stratification; personalized screening and early detection efforts; and, for some, targeted therapy.
Topics: Animals; Ataxia Telangiectasia Mutated Proteins; Carcinoma, Pancreatic Ductal; DNA Repair; Germ-Line Mutation; Humans; Neoplasm Proteins; Pancreas; Pancreatic Neoplasms; Risk Factors
PubMed: 31963441
DOI: 10.3390/genes11010108