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Revista Espanola de Enfermedades... Feb 2024We present the case of forty-four-year-old woman with autosomal dominant Polycystic liver disease (PLD) without kidney disease diagnosed for 18 years ago. She...
We present the case of forty-four-year-old woman with autosomal dominant Polycystic liver disease (PLD) without kidney disease diagnosed for 18 years ago. She experienced progressive abdominal distension and malnutrition over the previous twelve months. The patient had no other comorbidities. Physical examination revealed an 8 cm hepatomegaly in the right hypochondrium region. Liver function analysis was normal. CT scan showed multiple liver cysts with smooth and regular walls, predominantly in the right lobe which was considered as type III according to Gigot´s classification. Due to the significant hepatomegaly, conventional surgery was performed. Exploration showed multiple cysts of varying sizes on the liver surface, with diffuse involvement of the right hemiliver. Standard right lobe mobilization was technically not possible. Fenestration was performed in dominant superficial cysts, to allow access to the deeper cysts and liver parenchyma. A right hepatectomy was carried up using ultrasound devices and ultrasonic aspirator. And the biggest cysts located in the surface of left hepatic lobe were surgically unroofed. No surgical complications occurred, and the patient was discharged 5 days after the intervention.
PubMed: 38345523
DOI: 10.17235/reed.2024.10291/2024 -
Journal of Cystic Fibrosis : Official... Feb 2024Relieving gastrointestinal symptoms is a research priority in cystic fibrosis. Emerging evidence highlights effects of cystic fibrosis transmembrane conductance...
BACKGROUND
Relieving gastrointestinal symptoms is a research priority in cystic fibrosis. Emerging evidence highlights effects of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on gastrointestinal function, including pancreatic sufficiency. This study explores ivacaftor licensing and treatment on recorded pancreatic enzyme replacement therapy (PERT) prescription in the US and UK CF registries.
METHODS
Retrospective longitudinal registry study of recorded pancreatic PERT use between 2008 and 2017. Interrupted time series analysis in propensity-matched cohorts estimated annual change and step change according to ivacaftor eligibility before and after licensing year, 2012. Generalised estimating equations assessed adjusted risk of PERT use in individuals treated with ivacaftor after 2012 compared to untreated individuals.
RESULTS
In the US CF registry, the difference in annual change in prevalence of PERT use post-2012 between eligible cases and ineligible controls was -5.0 per 1000 people/year (95 %CI -7.6; -2.3, p = 0.001). The step change and annual change in prevalence of PERT use in eligible cases was not significantly different to controls in the UK CF registry. Relative to the relationship in 2013, ivacaftor treatment in the US CF registry was associated with a lower adjusted risk ratio of PERT use compared to untreated individuals by 2016 (0.97, 95 %CI 0.96; 0.99), which was not observed in the UK CF registry.
CONCLUSIONS
Licensing of ivacaftor was followed by a lower prevalence of PERT use in the eligible US population compared to pre-licensing period, as well as lower risk of PERT use in those who received treatment. Inconsistencies in US and UK CF registries were observed.
PubMed: 38342635
DOI: 10.1016/j.jcf.2024.01.011 -
Journal of Clinical Medicine Jan 2024Patients who undergo resection for non-invasive IPMN are at risk for long-term recurrence. Further evidence is needed to identify evidence-based surveillance strategies... (Review)
Review
Patients who undergo resection for non-invasive IPMN are at risk for long-term recurrence. Further evidence is needed to identify evidence-based surveillance strategies based on the risk of recurrence. We performed a systematic review of the current literature regarding recurrence patterns following resection of non-invasive IPMN to summarize evidence-based recommendations for surveillance. Among the 61 studies reviewed, a total of 8779 patients underwent resection for non-invasive IPMN. The pooled overall median follow-up time was 49.5 months (IQR: 38.5-57.7) and ranged between 14.1 months and 114 months. The overall median recurrence rate for patients with resected non-invasive IPMN was 8.8% (IQR: 5.0, 15.6) and ranged from 0% to 27.6%. Among the 33 studies reporting the time to recurrence, the overall median time to recurrence was 24 months (IQR: 17, 46). Existing literature on recurrence rates and post-resection surveillance strategies for patients with resected non-invasive IPMN varies greatly. Patients with resected non-invasive IPMN appear to be at risk for long-term recurrence and should undergo routine surveillance.
PubMed: 38337524
DOI: 10.3390/jcm13030830 -
Medicine Feb 2024Hemangioblastomas occur both sporadically and as an important component of von Hippel-Lindau (VHL) disease. The typical MRI features of hemangioblastoma are cysts with...
RATIONALE
Hemangioblastomas occur both sporadically and as an important component of von Hippel-Lindau (VHL) disease. The typical MRI features of hemangioblastoma are cysts with enhanced cystic wall nodules in the cerebellum or lesions with uniform enhancement on the surface or inside the spinal cord. If there is edema around hemangioblastoma, it is easy to be misdiagnosed as brain metastasis on MRI.
PATIENT CONCERNS
A 41-year-old male patient was found to have a lump in the pancreas during a health examination 3 months ago. Subsequently, the patient underwent surgical treatment. The postoperative pathology suggests that the pancreatic lesion is a neuroendocrine tumor. The patient subsequently came to our hospital for consultation on further treatment plans. Abnormal signals were found in the right cerebellum during pituitary magnetic resonance imaging (MRI) before the development of a treatment plan for neuroendocrine tumors. Subsequently, the patient underwent cerebellar mass resection surgery. The pathological result after the surgery was hemangioblastoma.
DIAGNOSIS
The patient underwent surgery to remove the tumor and was diagnosed with hemangioblastoma by pathological examination. Subsequently, the patient's genetic testing results confirmed the diagnosis of VHL syndrome.
INTERVENTIONS
The patient underwent cerebellar mass resection surgery.
OUTCOMES
The patient recovered after surgical resection.
LESSONS
In this report, we emphasize the atypical MRI manifestations of hemangioblastoma. For patients with VHL syndrome-related hemangioblastoma, genetic testing is necessary for the patient and their family members.
Topics: Adult; Humans; Male; Cerebellar Neoplasms; Hemangioblastoma; Magnetic Resonance Imaging; Neuroendocrine Tumors; Spinal Cord; von Hippel-Lindau Disease
PubMed: 38335432
DOI: 10.1097/MD.0000000000037162 -
Annals of Surgical Oncology Apr 2024The management of invasive intraductal papillary mucinous cystic neoplasm (I-IPMN) does not differ from de novo pancreatic ductal adenocarcinoma (PDAC); however, I-IPMNs...
BACKGROUND
The management of invasive intraductal papillary mucinous cystic neoplasm (I-IPMN) does not differ from de novo pancreatic ductal adenocarcinoma (PDAC); however, I-IPMNs are debated to have better prognosis. Despite being managed similarly to PDAC, no data are available on the response of I-IPMN to neoadjuvant chemotherapy.
METHODS
All patients undergoing pancreatic resection for a pancreatic adenocarcinoma from 2011 to 2022 were included. The PDAC and I-IPMN cohorts were compared to evaluate response to neoadjuvant therapy (NAT) and overall survival (OS).
RESULTS
This study included 1052 PDAC patients and 105 I-IPMN patients. NAT was performed in 25% of I-IPMN patients and 65% of PDAC patients. I-IPMN showed a similar pattern of pathological response to NAT compared with PDAC (p = 0.231). Furthermore, positron emission tomography (PET) response (71% vs. 61%; p = 0.447), CA19.9 normalization (85% vs. 76%, p = 0.290), and radiological response (32% vs. 37%, p = 0.628) were comparable between I-IPMN and PDAC. A significantly higher OS and disease-free survival (DFS) of I-IPMN was denoted by Kaplan-Meier analysis, with a p-value of < 0.001 in both plots. In a multivariate analysis, I-IPMN histology was independently associated with lower risk of recurrence and death.
CONCLUSIONS
I-IPMN patients have a longer OS and DFS after surgical treatment when compared with PDAC patients. The more favorable oncologic outcome of I-IPMNs does not seem to be related to early detection, as I-IPMN histological subclass is independently associated with a lower risk of disease recurrence. Moreover, neoadjuvant effect on I-IPMN was non-inferior to PDAC in terms of pathological, CA19.9, PET, and radiological response and thus can be considered in selected patients.
Topics: Humans; Pancreatic Neoplasms; Adenocarcinoma; Neoadjuvant Therapy; Pancreatic Intraductal Neoplasms; Adenocarcinoma, Mucinous; Neoplasm Recurrence, Local; Carcinoma, Pancreatic Ductal; Adenocarcinoma, Papillary; Retrospective Studies
PubMed: 38319513
DOI: 10.1245/s10434-023-14875-5 -
Cleveland Clinic Journal of Medicine Feb 2024Although most pancreatic cystic lesions do not progress to cancer, they create concern for patients and their primary care physicians. The lack of consensus guidelines... (Review)
Review
Although most pancreatic cystic lesions do not progress to cancer, they create concern for patients and their primary care physicians. The lack of consensus guidelines on diagnosis and surveillance of these lesions can lead to a management conundrum. We review current guidelines on diagnosis and management.
Topics: Humans; Pancreatic Cyst; Pancreatic Neoplasms; Pancreas
PubMed: 38307608
DOI: 10.3949/ccjm.91a.23019 -
JCEM Case Reports Feb 2024
PubMed: 38304008
DOI: 10.1210/jcemcr/luae002 -
International Journal of Surgery Case... Feb 2024Pancreatic serous cystic neoplasm (SCN) is usually benign and is often managed using imaging surveillance if asymptomatic. It has a higher incidence in females but is...
INTRODUCTION
Pancreatic serous cystic neoplasm (SCN) is usually benign and is often managed using imaging surveillance if asymptomatic. It has a higher incidence in females but is rare in younger age groups. Acute hemorrhagic complications associated with SCN are infrequent. Whether asymptomatic SCN can cause acute hemorrhage, especially in women of childbearing age, is not well-established.
PRESENTATION OF CASE
A 30-year-old Japanese female, who was six months postpartum and under surveillance for asymptomatic pancreatic SCN, presented to the emergency department with gradually worsening left lateral abdominal pain. Regular ultrasound revealed no change in SCN size; however, no imaging surveillance had been conducted over the past two years. She had pain in the entire abdomen, which intensified around the navel and elicited guarding. Abdominal contrast-enhanced computed tomography revealed a cystic mass in the pancreatic tail with a contrast blush within the cyst and an adjacent retroperitoneal hematoma. Endovascular embolization was performed to control the hemorrhage. The patient had an uneventful medical recovery and was discharged five days after embolization. Five months after discharge, she underwent laparoscopic distal pancreatectomy and splenectomy as an elective surgery and was discharged uneventfully.
DISCUSSION
Even with periodic imaging surveillance, pancreatic SCN can suddenly cause spontaneous hemorrhage. Clinicians should be aware that pancreatic SCN can potentially cause life-threatening complications, including spontaneous hemorrhage.
CONCLUSION
We report a case of an unexpected complication with spontaneous hemorrhage in a young woman who was under imaging surveillance for pancreatic SCN. The patient was successfully treated with angioembolization and planned laparoscopic surgery.
PubMed: 38286083
DOI: 10.1016/j.ijscr.2024.109309 -
Children (Basel, Switzerland) Jan 2024Blunt pancreatic injury (BPI) is relatively uncommon in children, and is associated with relatively high morbidity and mortality, especially if diagnosis is delayed. The... (Review)
Review
Blunt pancreatic injury (BPI) is relatively uncommon in children, and is associated with relatively high morbidity and mortality, especially if diagnosis is delayed. The aim of this report is to review the literature regarding controversial questions in the early diagnosis and management of pediatric BPI. A representative case of blunt pancreatic trauma in a six-year-old girl with delayed diagnosis and intraoperative and postoperative complications was described. A systematic search of databases and the grey literature in Scopus and Web of Science using relevant keywords was conducted. A total of 26 relevant articles published in last 5 years were found in PubMed. Although early CT performance is considered part of initial pancreatic trauma workup, the sensitivity of CT for detecting main pancreatic duct injuries in children is relatively low. MRCP and ERCP (if available) are useful for assessing ductal injury and should be performed when the status of the pancreatic duct is unclear on the CT. Most patients with low-grade pancreatic damage may be treated conservatively. Although surgery involving distal pancreatectomy remains the preferred approach for most children with high-grade pancreatic injury, there is growing evidence to suggest that non-operative management (NOM) is safe and effective. Most pancreatic pseudo cysts following NOM had relatively mild complications, and most resolved spontaneously. For those children who do require surgery, a conservative operative approach with the least risk is advocated. In conclusion, the optimal management for pediatric pancreatic trauma is controversial. Further clinical trials are required to generate clinical practice guidelines on pancreatic trauma in a child population.
PubMed: 38275445
DOI: 10.3390/children11010135 -
Machine Learning in Medical Imaging.... Oct 2023Intraductal Papillary Mucinous Neoplasm (IPMN) cysts are pre-malignant pancreas lesions, and they can progress into pancreatic cancer. Therefore, detecting and...
Intraductal Papillary Mucinous Neoplasm (IPMN) cysts are pre-malignant pancreas lesions, and they can progress into pancreatic cancer. Therefore, detecting and stratifying their risk level is of ultimate importance for effective treatment planning and disease control. However, this is a highly challenging task because of the diverse and irregular shape, texture, and size of the IPMN cysts as well as the pancreas. In this study, we propose a novel computer-aided diagnosis pipeline for IPMN risk classification from multi-contrast MRI scans. Our proposed analysis framework includes an efficient volumetric self-adapting segmentation strategy for pancreas delineation, followed by a newly designed deep learning-based classification scheme with a radiomics-based predictive approach. We test our proposed decision-fusion model in multi-center data sets of 246 multi-contrast MRI scans and obtain superior performance to the state of the art (SOTA) in this field. Our ablation studies demonstrate the significance of both radiomics and deep learning modules for achieving the new SOTA performance compared to international guidelines and published studies (81.9% vs 61.3% in accuracy). Our findings have important implications for clinical decision-making. In a series of rigorous experiments on multi-center data sets (246 MRI scans from five centers), we achieved unprecedented performance (81.9% accuracy). The code is available upon publication.
PubMed: 38274402
DOI: 10.1007/978-3-031-45676-3_14