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1.
Am J Surg Pathol ; 48(5): 511-520, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38567813

RESUMO

The diagnosis of solid pseudopapillary neoplasm of the pancreas (SPN) can be challenging due to potential confusion with other pancreatic neoplasms, particularly pancreatic neuroendocrine tumors (NETs), using current pathological diagnostic markers. We conducted a comprehensive analysis of bulk RNA sequencing data from SPNs, NETs, and normal pancreas, followed by experimental validation. This analysis revealed an increased accumulation of peroxisomes in SPNs. Moreover, we observed significant upregulation of the peroxisome marker ABCD1 in both primary and metastatic SPN samples compared with normal pancreas and NETs. To further investigate the potential utility of ABCD1 as a diagnostic marker for SPN via immunohistochemistry staining, we conducted verification in a large-scale patient cohort with pancreatic tumors, including 127 SPN (111 primary, 16 metastatic samples), 108 NET (98 nonfunctional pancreatic neuroendocrine tumor, NF-NET, and 10 functional pancreatic neuroendocrine tumor, F-NET), 9 acinar cell carcinoma (ACC), 3 pancreatoblastoma (PB), 54 pancreatic ductal adenocarcinoma (PDAC), 20 pancreatic serous cystadenoma (SCA), 19 pancreatic mucinous cystadenoma (MCA), 12 pancreatic ductal intraepithelial neoplasia (PanIN) and 5 intraductal papillary mucinous neoplasm (IPMN) samples. Our results indicate that ABCD1 holds promise as an easily applicable diagnostic marker with exceptional efficacy (AUC=0.999, sensitivity=99.10%, specificity=100%) for differentiating SPN from NET and other pancreatic neoplasms through immunohistochemical staining.


Assuntos
Carcinoma Ductal Pancreático , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia , Pâncreas/patologia , Carcinoma Ductal Pancreático/patologia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/genética , Tumores Neuroendócrinos/patologia , Ductos Pancreáticos/química , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análise , Membro 1 da Subfamília D de Transportadores de Cassetes de Ligação de ATP
2.
BMJ Case Rep ; 17(4)2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38594200

RESUMO

Hepatoid adenocarcinoma (HAC) of the mediastinum is a rare extrahepatic tumour that pathologically and morphologically resembles hepatocellular carcinoma. Extrahepatic HACs primarily occur in the stomach, ovaries, lung, gallbladder, pancreas and uterus. Patients with mediastinal HAC tend to be male smokers over forty years of age. Clinical symptoms of HAC are non-specific and varied in nature; therefore, diagnosis can be challenging and often delayed. Diagnostic investigations encompass haematological, radiological and histological assessment. Surgical resection is reserved for early-stage patients; however, since diagnosis may be delayed, most patients present with metastatic disease, for which the treatment of choice is platinum-based chemotherapy.


Assuntos
Adenocarcinoma , Carcinoma Hepatocelular , Neoplasias Hepáticas , Feminino , Humanos , Masculino , Mediastino/diagnóstico por imagem , Mediastino/patologia , Adenocarcinoma/diagnóstico , Carcinoma Hepatocelular/patologia , Pâncreas/patologia , Neoplasias Hepáticas/patologia
3.
Diagn Pathol ; 19(1): 59, 2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38622713

RESUMO

BACKGROUND: PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration. CASE PRESENTATION: A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa. CONCLUSIONS: A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.


Assuntos
Neoplasias Renais , Leucemia Mieloide Aguda , Neoplasias de Células Epitelioides Perivasculares , Masculino , Feminino , Humanos , Idoso , Biomarcadores Tumorais , Imuno-Histoquímica , Neoplasias de Células Epitelioides Perivasculares/cirurgia , Neoplasias de Células Epitelioides Perivasculares/patologia , Pâncreas/patologia
5.
Sci Rep ; 14(1): 9100, 2024 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-38643275

RESUMO

Diabetes constitutes a major public health problem, with dramatic consequences for patients. Both genetic and environmental factors were shown to contribute to the different forms of the disease. The monogenic forms, found both in humans and in animal models, specially help to decipher the role of key genes in the physiopathology of the disease. Here, we describe the phenotype of early diabetes in a colony of NOD mice, with spontaneous invalidation of Akt2, that we called HYP. The HYP mice were characterised by a strong and chronic hyperglycaemia, beginning around the age of one month, especially in male mice. The phenotype was not the consequence of the acceleration of the autoimmune response, inherent to the NOD background. Interestingly, in HYP mice, we observed hyperinsulinemia before hyperglycaemia occurred. We did not find any difference in the pancreas' architecture of the NOD and HYP mice (islets' size and staining for insulin and glucagon) but we detected a lower insulin content in the pancreas of HYP mice compared to NOD mice. These results give new insights about the role played by Akt2 in glucose homeostasis and argue for the ß cell failure being the primary event in the course of diabetes.


Assuntos
Diabetes Mellitus Tipo 1 , Diabetes Mellitus , Hiperglicemia , Ilhotas Pancreáticas , Humanos , Masculino , Camundongos , Animais , Camundongos Endogâmicos NOD , Ilhotas Pancreáticas/patologia , Diabetes Mellitus/patologia , Pâncreas/patologia , Insulina , Hiperglicemia/genética , Hiperglicemia/patologia , Diabetes Mellitus Tipo 1/patologia , Proteínas Proto-Oncogênicas c-akt/genética
6.
J Cardiothorac Surg ; 19(1): 184, 2024 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-38582893

RESUMO

The occurrence of ectopic pancreas in the mediastinum is rare. Herein, we report a 22-year-old female who presented with right shoulder pain, dysphagia, fever and headaches. Chest computer tomography revealed a mass in the posterior mediastinum with accompanying signs of acute mediastinitis. Needle biopsy and fine-needle aspiration revealed ectopic gastral tissue and ectopic pancreas tissue, respectively. Surgical resection was attempted due to recurring acute pancreatitis episodes. However, due to chronic-inflammatory adhesions of the mass to the tracheal wall, en-bloc resection was not possible without major tracheal resection. Since then, recurring pancreatitis episodes have been treated conservatively with antibiotics. We report this case due to its differing clinical and radiological findings in comparison to previous case reports, none of which pertained a case of ectopic pancreas tissue in the posterior mediastinum with recurring acute pancreatitis and mediastinitis.


Assuntos
Coristoma , Mediastinite , Pancreatite , Feminino , Humanos , Adulto Jovem , Adulto , Pancreatite/complicações , Pancreatite/diagnóstico , Mediastino/diagnóstico por imagem , Mediastino/patologia , Mediastinite/diagnóstico , Mediastinite/cirurgia , Mediastinite/complicações , Doença Aguda , Pâncreas/patologia , Coristoma/cirurgia , Coristoma/diagnóstico
7.
World J Gastroenterol ; 30(8): 817-832, 2024 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-38516247

RESUMO

Autoimmune pancreatitis (AIP) is an autoimmune subtype of chronic pancreatitis resulting from the aberrant immune response against the pancreas, leading to inflammation and fibrosis. Although AIP is rare, its incidence is increasing and is often misdiagnosed as other pancreatic diseases. AIP is commonly classified into two types. Type 1 AIP (AIP-1) is typically associated with elevated serum immunoglobulin G4 (IgG4) levels and systemic manifestations, while type 2 AIP is typically a more localized form of the disease, and may coexist with other autoimmune disorders, especially inflammatory bowel diseases. Additionally, there is emerging recognition of a third type (type 3 AIP), which refers to immunotherapy-triggered AIP, although this classification is still gaining acceptance in medical literature. The clinical manifestations of AIP mainly include painless jaundice and weight loss. Elevated serum IgG4 levels are particularly characteristic of AIP-1. Diagnosis relies on a combination of clinical, laboratory, radiological, and histological findings, given the similarity of AIP symptoms to other pancreatic disorders. The mainstay of treatment for AIP is steroid therapy, which is effective in most cases. Severe cases might require additional imm-unosuppressive agents. This review aims to summarize the current knowledge of AIP, encompassing its epidemiology, etiology, clinical presentation, diagnosis, and treatment options. We also address the challenges and controversies in diagnosing and treating AIP, such as distinguishing it from pancreatic cancer and managing long-term treatment, highlighting the need for increased awareness and knowledge of this complex disease.


Assuntos
Doenças Autoimunes , Pancreatite Autoimune , Humanos , Pancreatite Autoimune/diagnóstico , Pancreatite Autoimune/tratamento farmacológico , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Imunoglobulina G , Diagnóstico Diferencial , Pâncreas/patologia
8.
Endocr Relat Cancer ; 31(5)2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38552306

RESUMO

Insulinoma and glucagonoma are two rare functioning neoplasms of the neuroendocrine cells of the pancreas, respectively, characterized by an uncontrolled over-secretion of insulin or glucagon, responsible for the development of the hypoglycemic syndrome and the glucagonoma syndrome. They prevalently arise as sporadic tumors; only about 10% of cases develop in the context of rare inherited tumor syndromes, such as multiple endocrine neoplasia type 1 (MEN1), neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC), being the result of an autosomal-dominant germline heterozygous loss-of-function mutation in a tumor-suppressor gene. Here, we reviewed the main epidemiological and clinical aspects of insulinoma and glucagonoma in the context of genetic syndromes.


Assuntos
Glucagonoma , Insulinoma , Neoplasia Endócrina Múltipla Tipo 1 , Neoplasias Pancreáticas , Humanos , Insulinoma/genética , Insulinoma/patologia , Glucagonoma/genética , Glucagonoma/patologia , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia , Neoplasia Endócrina Múltipla Tipo 1/genética , Pâncreas/patologia
9.
Int J Mol Sci ; 25(6)2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38542259

RESUMO

Undifferentiated carcinoma with osteoclast-like giant cells (UCOGC) of the pancreas is a rare malignancy regarded as a subvariant of pancreatic ductal carcinoma (PDAC) characterized by variable prognosis. UCOGC shows a strikingly similar spectrum of oncogenic DNA mutations to PDAC. In the current work, we analyzed the landscape of somatic mutations in a set of 13 UCOGC cases via next-generation sequencing (NGS). We detected a spectrum of pathogenic or likely pathogenic mutations similar to those observed in PDAC following previously published results (10 KRAS, 9 TP53, 4 CDKN2A, and 1 SMAD4, CIC, GNAS, APC, ATM, NF1, FBXW7, ATR, and FGFR3). Our results support the theory that UCOGC is a variant of PDAC, despite its unique morphology; however, a UCOGC-specific genomic signature as well as predictive markers remain mainly unknown. Programmed death ligand 1 (PD-L1) status remains an important predictive marker based on previous studies.


Assuntos
Carcinoma Ductal Pancreático , Neoplasias Pancreáticas , Humanos , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia , Osteoclastos/patologia , Pâncreas/patologia , Carcinoma Ductal Pancreático/patologia , Células Gigantes/patologia , Mutação , Biologia Molecular
10.
PLoS One ; 19(3): e0300723, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38547077

RESUMO

Preclinical models of cancer are vital for assessing and predicting efficacies and toxicities of novel treatments prior to testing in human subjects. Current pancreatic tumor models exhibit variable growth rates, unpredictable tumor size after implantation in non-native tissues, or require surgical implantation. Surgical implantation in the pancreas may produce not only unpredictable tumor uptake but could also elicit additional inflammatory responses. In searching for a pancreatic carcinoma cell that can be introduced into a mouse via simple injection, we found that Pan02, a murine ductal pancreatic adenocarcinoma derived from a pancreatic lesion of a C57BL/6 mouse, inoculated peritoneally can consistently produce pancreatic tumors. This intraperitoneal, but not intravenous, introduction of Pan02 cells leads to the attachment and growth of Pan02 in the pancreas before spreading to other tissues. Time-course tissue analysis indicates that the Pan02 cells first find, infiltrate, and grow within the pancreas, producing a pancreatic tumor model. This model appears to mimic pancreatic cancer development in humans and is the first reported use of Pan02 cells to produce orthotopic pancreatic and metastatic neoplasms in a mouse model without the need for tumor implantation within matrices or survival surgeries. This orthotopic pancreatic tumor model, with consistent tumor uptake, synchronized tumor development and survival, and predictable outcomes may enable and accelerate the preclinical evaluation of treatment candidates for pancreatic cancer.


Assuntos
Adenocarcinoma , Carcinoma Ductal Pancreático , Neoplasias Pancreáticas , Humanos , Camundongos , Animais , Neoplasias Pancreáticas/patologia , Adenocarcinoma/patologia , Camundongos Endogâmicos C57BL , Carcinoma Ductal Pancreático/patologia , Pâncreas/patologia , Modelos Animais de Doenças , Linhagem Celular Tumoral
11.
Front Immunol ; 15: 1342816, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38515753

RESUMO

Salmonid alphavirus (SAV) causes pancreas disease (PD), which negatively impacts farmed Atlantic salmon. In this study, fish were vaccinated with a DNA-PD vaccine (DNA-PD) and an oil-adjuvanted, inactivated whole virus PD vaccine (Oil-PD). Controls were two non-PD vaccinated groups. Fish were kept in one tank and challenged by cohabitation with SAV genotype 2 in seawater. Protection against infection and mortality was assessed for 84 days (Efficacy study). Nineteen days post challenge (dpc), subgroups of fish from all treatment groups were transferred to separate tanks and cohabited with naïve fish (Transmission study 1) or fish vaccinated with a homologous vaccine (Transmission study 2), to evaluate virus transmission for 26 days (47 dpc). Viremia, heart RT-qPCR and histopathological scoring of key organs affected by PD were used to measure infection levels. RT-droplet digital PCR quantified shedding of SAV into water for transmission studies. The Efficacy study showed that PD associated growth-loss was significantly lower and clearance of SAV2 RNA significantly higher in the PD-DNA group compared to the other groups. The PD-DNA group had milder lesions in the heart and muscle. Cumulative mortality post challenge was low and not different between groups, but the DNA-PD group had delayed time-to-death. In Transmission study 1, the lowest water levels of SAV RNA were measured in the tanks containing the DNA-PD group at 21 and 34 dpc. Despite this, and irrespective of the treatment group, SAV2 was effectively transmitted to the naïve fish during 26-day cohabitation. At 47 dpc, the SAV RNA concentrations in the water were lower in all tanks compared to 34 dpc. In Transmission study 2, none of the DNA-PD immunized cohabitants residing with DNA-PD-vaccinated, pre-challenged fish got infected. In contrast, Oil-PD immunized cohabitants residing with Oil-PD-vaccinated, pre-challenged fish, showed infection levels similar to the naïve cohabitants in Transmission study 1. The results demonstrate that the DNA-PD vaccine may curb the spread of SAV infection as the DNA-PD vaccinated, SAV2 exposed fish, did not spread the infection to cohabiting DNA-PD vaccinated fish. This signifies that herd immunity may be achieved by the DNA-PD vaccine, a valuable tool to control the PD epizootic in farmed Atlantic salmon.


Assuntos
Alphavirus , Doenças dos Peixes , Pancreatopatias , Salmo salar , Vacinas de DNA , Vacinas Virais , Animais , Pancreatopatias/veterinária , Pancreatopatias/patologia , RNA/genética , Água , Pâncreas/patologia , DNA , Genótipo
13.
Cancer Med ; 13(5): e7026, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38477492

RESUMO

BACKGROUND: Differential diagnosis of pancreatic solid lesion (PSL) and prognosis of pancreatic cancer (PC) is a clinical challenge. We aimed to explore the differential diagnostic value of sound speed (SS) obtained from endoscopic ultrasonography (EUS) in PSL and the prognostic value of SS in PC. METHODS: Patients with PSL in The Third Xiangya Hospital of Central South University from March 2019 to October 2019 were prospectively enrolled, who obtained SS from PSL. Patients were divided into the PC group and the pancreatic benign lesion (PBL) group. SS1 is the SS of lesions and SS2 is the SS of normal tissues adjacent to lesions. Ratio1 is equal to SS1 divided by SS2 of PSL (ratio1 = SS1/SS2). RESULTS: Eighty patients were enrolled (24 PBL patients, 56 PC patients). SS1 and ratio1 in PC group were higher compared with PBL group (SS1:1568.00 vs. 1550.00, Z = -2.066, p = 0.039; ratio1: 1.0110 vs. 1.0051, Z = -3.391, p = 0.001). The SS1 in PC (Z = -6.503, p < 0.001) was higher compared to SS2. In the nonsurgical group of PC, low ratio1 predicted high overall survival (OS) (7.000 months vs. 4.000 months; p = 0.039). In the surgical group of PC, low SS1 was associated with low median OS (4.000 months vs. 12.000 months; p = 0.033). CONCLUSIONS: SS plays a vital role in distinguishing between PBL and PC. Higher SS1 and ratio1 obtained by EUS are more related to PC than PBL. In PC patients, high SS1 may predict pancreatic lesions. In the nonsurgical group of PC, low ratio1 may predict high OS. However, in the surgical group of PC, low SS1 may predict low OS.


Assuntos
Endossonografia , Neoplasias Pancreáticas , Humanos , Diagnóstico Diferencial , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Prognóstico
14.
Med Arch ; 78(1): 29-32, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38481591

RESUMO

Background: Radical surgical resection for pancreatic head carcinoma offers a chance for cure but unfortunately is only available to a limited number of patients. For a significant number of patients, palliative surgery remains the only option. The question of the most effective approach for patients with borderline resectable pancreatic head carcinoma (BRPHC) remains unresolved. Objective: The aim of the study was to compare the morbidity and mortality following R1 duodenocephalic pancreatectomy and double palliative bypass to explore the most optimal surgical treatment for patients with BRPHC. Methods: Our retrospective cohort study included 64 patients with BRPHC who underwent surgery from 2012 to 2019, with postoperative follow-up for three years. Morbidity and mortality parameters were examined based on the type of surgical treatment: R1 duodenocephalic pancreatectomy or palliative double bypass. Chi-square test, univariate regression, and Kaplan-Meier analysis were used as basic statistical methods in the analysis of the results. Results: Patients undergoing R1 duodenocephalic pancreatectomy had a 3.69 times higher risk of developing biliary leak (p=0.039; 95%CI:1.066, 1.181) and shorter survival compared to those undergoing palliative double bypass (p=0.022). No statistically significant association was found between the type of surgical procedure and other postoperative complications. Conclusion: Our study suggests that the double palliative bypass procedure may be a better option than R1 resection for patients with BRPHC.


Assuntos
Adenocarcinoma , Neoplasias Pancreáticas , Humanos , Estudos Retrospectivos , Neoplasias Pancreáticas/cirurgia , Pancreatectomia/efeitos adversos , Pâncreas/patologia , Adenocarcinoma/cirurgia , Morbidade , Cuidados Paliativos
15.
Gan To Kagaku Ryoho ; 51(3): 320-322, 2024 Mar.
Artigo em Japonês | MEDLINE | ID: mdl-38494818

RESUMO

Lymphoepithelial cyst(LEC)of the pancreas is a relatively rare benign cystic disease of the pancreas. In this report, we describe a case of LEC in which a malignant tumor could not be ruled out by preoperative diagnosis and surgery was performed. The patient was a 72-year-old man. A simple CT scan of the chest and abdomen performed as a follow-up for another disease incidentally revealed a mass in the pancreatic tail. Enhanced CT of the abdomen showed a tumor approximately 3 cm in size at the pancreatic tail with no contrast effect. MRCP showed moderate signal on T2WI, high signal on T1WI, and high signal on T2WI on some cysts inside the pancreas. PET-CT showed slight uptake of FDG. Both tumor markers CEA and CA19-9 were normal. Therefore, malignant disease such as pancreatic IPMC could not be ruled out, and laparoscopic distal pancreatectomy plus splenectomy was performed. The pathology results showed a diagnosis of pancreatic lymphoepithelial cyst with slight differentiation into sebaceous gland.


Assuntos
Cisto Epidérmico , Cisto Pancreático , Masculino , Humanos , Idoso , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Pâncreas/patologia , Cisto Pancreático/diagnóstico , Cisto Pancreático/cirurgia , Cisto Pancreático/patologia , Abdome/patologia , Cisto Epidérmico/diagnóstico , Cisto Epidérmico/cirurgia , Cisto Epidérmico/patologia
16.
Rev Med Liege ; 79(3): 168-174, 2024 Mar.
Artigo em Francês | MEDLINE | ID: mdl-38487911

RESUMO

Congenital hyperinsulinism is the most common cause of recurrent hypoglycemia in newborns and children. Early diagnosis and rapid management are essential to avoid hypoglycaemic brain injury and later neurological complications. Management of those patients involves biological evaluation, molecular genetics, imaging techniques and surgical advances. We report the case of a newborn with recurrent hypoglycemia due to congenital hyperinsulinism (CHI) caused by a new variant in the ABCC8 gene. Fluorine 18-L-3,4 Dihydroxyphenylalanine Positron Emission Tomography (18F-DOPA PET/CT scan) reported a focal lesion at the isthmus of the pancreas which has been removed by laparoscopic surgery with a complete recovery for the patient.


L'hyperinsulinisme congénital est la cause la plus fréquente d'hypoglycémies récidivantes chez le nouveau-né et l'enfant. Un diagnostic et une prise en charge précoces sont primordiaux pour éviter les conséquences potentielles sur le développement neurologique. Ces derniers reposent sur la conjonction d'éléments biologiques, génétiques et d'imagerie. Nous rapportons le cas d'un nouveau-né présentant des hypoglycémies récidivantes. La mise au point mettra en évidence un hyperinsulinisme congénital (CHI) lié à un variant non encore décrit au sein du gène ABCC8. L'imagerie par Fluorine 18-L-3,4 Dihydroxyphenylalanine Positron Emission Tomography/Computed Tomography-scanner (18F-DOPA PET/CT scan) a mis en évidence une forme focale de l'hyperinsulinisme justifiant une prise en charge chirurgicale amenant à une guérison complète et à l'arrêt de tout traitement médicamenteux.


Assuntos
Hiperinsulinismo Congênito , Laparoscopia , Criança , Humanos , Recém-Nascido , Lactente , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Hiperinsulinismo Congênito/diagnóstico por imagem , Hiperinsulinismo Congênito/genética , Hiperinsulinismo Congênito/patologia , Pâncreas/patologia , Pâncreas/cirurgia , Tomografia por Emissão de Pósitrons/métodos
17.
Anticancer Res ; 44(4): 1695-1702, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38537987

RESUMO

BACKGROUND/AIM: This study aimed to identify the risk factors for early recurrence (ER) after pancreatic ductal adenocarcinoma (PDAC) resection to create a novel scoring system for ER and analyze their effect on the recurrence pattern. PATIENTS AND METHODS: Sixty patients with PDAC who underwent pancreatectomy were included. The predicted risk factors for ER were analyzed. A new score defining ER was created and analyzed for recurrence pattern and prognosis. RESULTS: Independent predictors included high CA 19-9 (≥147 U/ml), high lymph node ratio (LNR of ≥0.1277), and no adjuvant chemotherapy (AC). The 5-year overall survival rates with a score of 0, 1, and 2 were 55.8%, 11.0%, and 0%, respectively. In the moderate- risk score group, prognosis was improved by induction of AC within 58 days. CONCLUSION: Preoperative high CA19-9, high LNR, and no AC could be ER predictors. Induction of postoperative chemotherapy within 58 days may improve prognosis.


Assuntos
Carcinoma Ductal Pancreático , Neoplasias Pancreáticas , Humanos , Pancreatectomia/efeitos adversos , Neoplasias Pancreáticas/patologia , Carcinoma Ductal Pancreático/patologia , Pâncreas/patologia , Prognóstico , Fatores de Risco , Recidiva Local de Neoplasia/patologia , Antígeno CA-19-9 , Estudos Retrospectivos
18.
Pancreas ; 53(4): e350-e356, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38518061

RESUMO

BACKGROUND: The International Cancer of the Pancreas Screening Consortium recommended annual imaging for individuals at increased risk for developing a pancreatic ductal adenocarcinoma (PDAC) who did not have concerning pancreatic findings or a cyst <3 cm without worrisome features. We aimed to determine if 3-cm cyst size accurately predicted advanced precursor lesions in high-risk individuals undergoing surveillance. METHODS: Imaging for high-risk individuals (HRIs) undergoing PDAC surveillance from 2007 to 2021 was reviewed and pancreatic abnormalities were recorded including dominant cyst size and number of cysts. Subjects were excluded if they had the following: (1) no follow-up imaging after baseline, (2) solid lesion at baseline, or (3) development of solid lesion without having cyst on prior imaging. RESULTS: Five of the 77 HRIs found to have a cystic lesion on surveillance developed a PDAC: 3 with cystic lesion >1 cm as compared with only 2 of 67 HRIs with cystic lesions <1 cm (P < 0.05). None of these cysts developed worrisome findings and 4/5 PDACs did not arise from visualized cystic precursor lesion. CONCLUSIONS: Patients with a cyst ≥1 cm were at increased risk for developing PDAC compared with patients with cyst <1 cm. Pancreatic ductal adenocarcinoma usually did not arise from a recognized cystic lesion.


Assuntos
Carcinoma Ductal Pancreático , Cisto Pancreático , Neoplasias Pancreáticas , Humanos , Imageamento por Ressonância Magnética/métodos , Neoplasias Pancreáticas/patologia , Cisto Pancreático/diagnóstico , Pâncreas/patologia , Carcinoma Ductal Pancreático/patologia , Estudos Retrospectivos
19.
Vet Med Sci ; 10(2): e1394, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38459816

RESUMO

BACKGROUND: Lovebird (Agapornis personatus) is a monotypic species of bird of the lovebird genus in the parrot family Psittaculidae and order Psittaciformes. OBJECTIVES: The present study was designed to investigate the histology and immunohistochemistry of the pancreas in the lovebird. METHODS: Totally, three adult birds were used. The pancreas was assessed using histological and immunofluorescent staining to detect insulin, glucagon, somatostatin, pancreatic polypeptide (PP) and neuropeptide Y (NY). RESULTS: The exocrine pancreas was composed of pyramidal acinar cells with zymogen granules at the apical cytoplasm. The endocrine pancreas was identified as large alpha, small beta and mixed islets of Langerhans. No intercalated duct was observed. Alpha cells with a density of 28.55% were the most numerous cell type, which were populated throughout the large islets, especially at the periphery. The beta cells with a density of 15.78% were accumulated mostly at the periphery of islets. The delta cells exhibited 17.81% intensity. Despite their lower density, the distribution of delta cells was like that of A cells throughout the islets. PP and NY cells were distinguished with densities of 14.69% and 20.63%, respectively. CONCLUSIONS: Although the arrangement of acinar cells, ductal systems and endocrine islets reflects patterns observed in various avian species, the absence of intercalated duct, the presence of three types of Langerhans islets as alpha, beta and mixed islets and the high expression of NY in the islets were some unique features observed in the current study. These findings contribute to the broader understanding of avian pancreas histology.


Assuntos
Agapornis , Ilhotas Pancreáticas , Animais , Pâncreas/metabolismo , Pâncreas/patologia , Ilhotas Pancreáticas/metabolismo , Glucagon/metabolismo , Insulina/metabolismo , Corantes
20.
Pancreatology ; 24(3): 463-488, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38480047

RESUMO

BACKGROUND: The management of branch-duct type intraductal papillary mucinous neoplasms (BD-IPMN) varies in existing guidelines. This study investigated the optimal surveillance protocol and safe discontinuation of surveillance considering natural history in non-resected IPMN, by systematically reviewing the published literature. METHODS: This review was guided by PRISMA. Research questions were framed in PICO format "CQ1-1: Is size criteria helpful to determine surveillance period? CQ1-2: How often should surveillance be carried out? CQ1-3: When should surveillance be discontinued? CQ1-4: Is nomogram predicting malignancy useful during surveillance?". PubMed was searched from January-April 2022. RESULTS: The search generated 2373 citations. After screening, 83 articles were included. Among them, 33 studies were identified for CQ1-1, 19 for CQ1-2, 26 for CQ1-3 and 12 for CQ1-4. Cysts <1.5 or 2 cm without worrisome features (WF) were described as more indolent, and most studies advised an initial period of surveillance. The median growth rate of cysts <2 cm ranged from 0.23 to 0.6 mm/year. Patients with cysts <2 cm showing no morphological changes and no WF after 5-years of surveillance have minimal malignancy risk of 0-2%. Two nomograms created with over 1000 patients had AUCs of around 0.8 and appear to be feasible in a real-world practice. CONCLUSIONS: For patients with suspected BD-IPMN <2 cm and no other WF, less frequent surveillance is recommended. Surveillance may be discontinued for cysts that remain stable during 5-year surveillance, with consideration of patient condition and life expectancy. With this updated surveillance strategy, patients with non-worrisome BD-IPMN should expect more streamlined management and decreased healthcare utilization.


Assuntos
Carcinoma Ductal Pancreático , Cistos , Neoplasias Intraductais Pancreáticas , Neoplasias Pancreáticas , Humanos , Neoplasias Intraductais Pancreáticas/patologia , Neoplasias Pancreáticas/patologia , Pâncreas/patologia , Cistos/patologia , Ductos Pancreáticos/patologia , Carcinoma Ductal Pancreático/patologia , Estudos Retrospectivos
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