RESUMO
ABSTRACT: We present the imaging findings of a 48-year-old woman that metastasized to multiple bones with a history of duodenal gangliocytic paraganglioma. 68Ga-DOTATATE PET/CT showed multiple osteolytic bone destruction with intense uptake. Multiple bone metastases originating from duodenal gangliocytic paraganglioma confirmed histopathological results of a biopsy on the chest-back bone.
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Neoplasias Ósseas , Neoplasias Duodenais , Compostos Organometálicos , Paraganglioma , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Feminino , Pessoa de Meia-Idade , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Neoplasias Duodenais/diagnóstico por imagem , Neoplasias Duodenais/patologia , Neoplasias Duodenais/secundário , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/secundário , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Duodenal papillary adenoma, a potentially malignant benign tumor is primarily treated with endoscopic papillectomy. Despite its efficacy, endoscopic papillectomy has a high complication rate. This study investigates whether pancreatic duct and common bile duct stent placement can mitigate these complications. METHODS: In a retrospective observational analysis, 79 patients with duodenal papillary adenoma, treated with endoscopic papillectomy at our center, were studied. The cohort included patients who underwent endoscopic papillectomy with no stents placement, common bile duct stent placement alone, pancreatic duct stent placement alone, or stents placement in both ducts. We assessed the outcomes of endoscopic papillectomy, including complete resection rate and recurrence rate as the primary and secondary outcomes respectively. In the meantime, the incidence of complications were also analysed as the safety outcomes. RESULTS: Complete resection rates did not significantly differ between patients with or without stent placement (85.7% P group vs. 89.2% N-P group, P = 0.64). Early complication rates were similar across groups. However, significant reduction in common bile duct stenosis was observed in the stenting group (0% B group vs. 10.5% N-B group, P = 0.03). Furthermore, stent placement correlated with lower adenoma recurrence rates during follow-up (2.4% P group vs. 16.2% N-P group, P = 0.03; 2.4% B group vs. 15.8% N-B group, P = 0.04). CONCLUSIONS: Pancreatic duct and common bile duct stent placement in endoscopic papillectomy may decrease late complications, particularly common bile duct stenosis, and reduce the recurrence of duodenal papillary adenoma. TRIAL REGISTRATION: This study received approval from the Institutional Review Board and Ethics Committee of Beijing Friendship Hospital (Approval No. BFHHZS20230203), and retrospectively registered in www. CLINICALTRIALS: gov (NCT06301048, Initial Release date: 02/18/2024, Last Public Release date: 03/03/2024).
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Adenoma , Neoplasias Duodenais , Ductos Pancreáticos , Stents , Humanos , Masculino , Estudos Retrospectivos , Feminino , Pessoa de Meia-Idade , Adenoma/cirurgia , Adenoma/patologia , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Ductos Pancreáticos/cirurgia , Ductos Pancreáticos/patologia , Idoso , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia , Resultado do Tratamento , Recidiva Local de Neoplasia , Esfinterotomia Endoscópica/métodos , Esfinterotomia Endoscópica/efeitos adversos , Adulto , Ampola Hepatopancreática/cirurgia , Ducto Colédoco/cirurgiaRESUMO
BACKGROUND: Complete endoscopic resection of superficial non-ampullary duodenal epithelial tumors (SNADETs) is technically difficult, especially with an extremely high risk of adverse event (AE), although various endoscopic resection methods including endoscopic mucosal resection (EMR), underwater EMR (UEMR), and endoscopic submucosal dissection (ESD) have been tried for SNADETs. Accordingly, a novel simple resection method that can completely resect tumors with a low risk of AEs should be developed. AIMS: A resection method of Noninjecting Resection using Bipolar Soft coagulation mode (NIRBS) which has been reported to be effective and safe for colorectal lesions is adapted for SNADETs. In this study we evaluated its effectiveness, safety, and simplicity for SNADETs measuring ≤ 20 mm. RESULTS: This study included 13 patients with resected lesions with a mean size of 7.8 (range: 3-15) mm. The pathological distributions of the lesions were as follows: adenomas, 77% (n = 10) and benign and non-adenomatous lesions, 23% (n = 3). The en bloc and R0 resection rate was 100% (n = 13). The median procedure duration was 68 s (32-105). None of the patients presented with major AEs including bleeding and perforation. CONCLUSIONS: Large studies such as prospective, randomized, and controlled trials should be conducted for the purpose of validating effectiveness, safety, and simplicity of the NIRBS for SNADETs measuring ≤ 20 mm suggested in this study.
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Neoplasias Duodenais , Ressecção Endoscópica de Mucosa , Humanos , Projetos Piloto , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Pessoa de Meia-Idade , Feminino , Masculino , Idoso , Ressecção Endoscópica de Mucosa/métodos , Ressecção Endoscópica de Mucosa/efeitos adversos , Adenoma/cirurgia , Adenoma/patologia , Idoso de 80 Anos ou mais , Eletrocoagulação/métodos , AdultoRESUMO
PURPOSE OF REVIEW: This review aims to detail the characteristics and outcomes of duodenal-type follicular lymphoma (DTFL), a rare lymphoma variant. It focuses on integrating recent reports in treatment modalities and highlights emerging insights into the unique biological features of the disease. RECENT FINDING: Recent studies confirm the indolent nature of DTFL, with extended follow-up periods showing favorable outcomes under watchful waiting strategies and a notable proportion of patients experiencing spontaneous remission. Additionally, advancements in understanding the disease's biology revealed that the tumor microenvironment is marked by specific genomic expressions indicative of chronic inflammation. SUMMARY: The observations of spontaneous resolution and the generally favorable progression of DTFL call for a conservative approach in initiating treatment. Clinical management should judiciously consider the disease's typically benign course against the potential risks of intervention, promoting customized treatment protocols tailored for cases with clinical necessity. Additionally, the discovery of an inflammatory tumor microenvironment and molecular evidence suggesting an antigen-driven process highlight critical areas for future research.
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Linfoma Folicular , Humanos , Linfoma Folicular/terapia , Linfoma Folicular/patologia , Linfoma Folicular/genética , Neoplasias Duodenais/patologia , Neoplasias Duodenais/terapia , Neoplasias Duodenais/genética , Microambiente TumoralRESUMO
OBJECTIVE: This study was conducted to investigate the imaging information, laboratory data, and clinical characteristics of duodenal papillary malignancies, aiming to contribute to the early diagnosis of these diseases. METHODS: The clinical characteristics, laboratory data, and computed tomography (CT) findings of 17 patients with adenoma of the major duodenal papilla (the adenoma group) and 58 patients with cancer of the major duodenal papilla (the cancer group) were retrospectively analyzed. The measurement data were analyzed using t test and expressed as mean ± standard deviation. The counting data were analyzed using the χ2 test and expressed in n (%). Pearson correlation analysis was also conducted, and a scatter plot was drawn. RESULTS: There were significant differences in the diameter, shape, margin, and target sign of the major duodenal papilla, pancreatic duct diameter, common bile duct diameter, enhancement uniformity, fever, direct bilirubin, total bilirubin, carcinoembryonic antigen, carbohydrate antigen 19-9, and jaundice between the adenoma group and the cancer group (P < .01). The enhancement magnitude of the duodenal papilla was correlated with the lesion size, and the venous phase CT value of the enhanced scan was correlated with the duodenal papilla diameter (P < .05). Additionally, 12 patients in the cancer group suffered from malignant transformation of adenomas. CONCLUSION: Firstly, CT is of high value in the diagnosis of duodenal papilla diseases. Secondly, the enhancement magnitude of the duodenal papilla is correlated with the lesion size. Thirdly, patients with duodenal papilla adenomas have a risk of progression into adenocarcinoma, thereby requiring close follow-up.
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Neoplasias Duodenais , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X/métodos , Neoplasias Duodenais/diagnóstico por imagem , Neoplasias Duodenais/sangue , Neoplasias Duodenais/patologia , Idoso , Adulto , Ampola Hepatopancreática/patologia , Ampola Hepatopancreática/diagnóstico por imagem , Adenoma/diagnóstico por imagem , Adenoma/sangue , Adenoma/patologiaRESUMO
INTRODUCTION: Recommendations for resection technique of duodenal neuroendocrine neoplasms (D-NEN) with a size between 10 and 20 mm are lacking. The primary aim was to compare overall survival (OS) and progression-free survival (PFS) after endoscopic resection (ER) with surgical resection (SR). The secondary aim was to assess the incidence and clinical variables correlated with OS. METHODS: Data of patients with D-NENs between 2008 and 2018 were extracted from the Netherlands Cancer Registry and the Dutch Nationwide Pathology Databank. RESULTS: A total of 259 patients were identified, of which 138 were included: 98 (68 %) underwent ER and 44 patients (32 %) underwent SR. Of these, 38 patients had D-NENs sized between 10 and 20 mm. ER Patients were more frequently male and had a lower T-stage and tumour size than SR patients (all P < 0.05). Positive resection margins were observed more frequently after ER compared to SR (71 % vs 15 %, P < 0.005). No patients with tumours between 10 and 20 mm died after ER or SR (median follow-up 71.8 vs. 52.0 months). PFS rates were not significantly different after ER compared to SR (P = 0.672). Recurrence rates were 13 % for ER and 7 % for SR (P = 0.604). CONCLUSION: Between 2008 and 2018, the incidence increased from 0.06 to 0.11 per 100,000 patients per year. OS after ER or SR did not differ for D-NEN between 10 and 20 mm. Recurrence and PFS rates were not significantly different. These results suggest that D-NENs sized between 10 and 20 mm could potentially be treated first with ER. Future studies are needed to confirm this hypothesis.
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Neoplasias Duodenais , Tumores Neuroendócrinos , Humanos , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Masculino , Feminino , Tumores Neuroendócrinos/cirurgia , Tumores Neuroendócrinos/patologia , Estudos Retrospectivos , Pessoa de Meia-Idade , Idoso , Países Baixos/epidemiologia , Margens de Excisão , Taxa de Sobrevida , Carga Tumoral , Estadiamento de Neoplasias , Intervalo Livre de Progressão , Adulto , Sistema de RegistrosRESUMO
BACKGROUND: Multiple endocrine neoplasias (MENs) are a group of hereditary diseases involving multiple endocrine glands, and their prevalence is low. MEN type 1 (MEN1) has diverse clinical manifestations, mainly involving the parathyroid glands, gastrointestinal tract, pancreas and pituitary gland, making it easy to miss the clinical diagnosis. CASE SUMMARY: We present the case of a patient in whom MEN1 was detected early. A middle-aged male with recurrent abdominal pain and diarrhea was admitted to the hospital. Blood tests at admission revealed hypercalcemia and hypophosphatemia, and emission computed tomography of the parathyroid glands revealed a hyperfunctioning parathyroid lesion. Gastroscopy findings suggested a duodenal bulge and ulceration. Ultrasound endoscopy revealed a hypoechoic lesion in the duodenal bulb. Further blood tests revealed elevated levels of serum gastrin. Surgery was performed, and pathological analysis of the surgical specimens revealed a parathyroid adenoma after parathyroidectomy and a neuroendocrine tumor after duodenal bulbectomy. The time from onset to the definitive diagnosis of MEN1 was only approximately 1 year. CONCLUSION: For patients who present with gastrointestinal symptoms accompanied by hypercalcemia and hypophosphatemia, clinicians need to be alert to the possibility of MEN1.
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Hipercalcemia , Neoplasia Endócrina Múltipla Tipo 1 , Neoplasias das Paratireoides , Paratireoidectomia , Humanos , Neoplasia Endócrina Múltipla Tipo 1/cirurgia , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/patologia , Masculino , Neoplasias das Paratireoides/cirurgia , Neoplasias das Paratireoides/diagnóstico , Neoplasias das Paratireoides/patologia , Neoplasias das Paratireoides/complicações , Pessoa de Meia-Idade , Hipercalcemia/diagnóstico , Hipercalcemia/etiologia , Hipercalcemia/sangue , Adenoma/cirurgia , Adenoma/diagnóstico , Adenoma/patologia , Adenoma/sangue , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/diagnóstico , Neoplasias Duodenais/patologia , Hipofosfatemia/etiologia , Hipofosfatemia/diagnóstico , Dor Abdominal/etiologia , Dor Abdominal/diagnóstico , Tumores Neuroendócrinos/cirurgia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/sangue , Tumores Neuroendócrinos/patologia , Diarreia/etiologia , Diarreia/diagnóstico , Detecção Precoce de Câncer/métodos , Gastroscopia , Resultado do TratamentoRESUMO
Paragangliomas (PGLs) are rare and encapsulated neuroendocrine tumors (NET), located in the adrenal gland or the extra-adrenal paraganglia. Extra-adrenal PGLs may develop a gangliocytic component with ganglion cells which are called gangliocytic paragangliomas (GPs). The most common location is the duodenum, and they appear with digestive symptoms or as an incidental finding. We described a 43 years old patient, with epigastric pain, nausea and vomiting. The CT-scan reveals a nodular image in the duodenum. An ultrasound-guided FNA was performed and the pathological report revealed neuroendocrine cell groups and neural tissue. Surgery was the chosen treatment. As the patient did not present lymphatic or pancreatic parenchyma invasion, radiotherapy (RT) was not administered. The management of GPs is not well established and multidisciplinary team approach is recommended to lead to therapeutic options. Surgical resection is still key in the treatment, and adjuvant RT may be considered in cases of lymph node invasion.
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Neoplasias Duodenais , Adulto , Feminino , Humanos , Masculino , Neoplasias Duodenais/patologia , Neoplasias Duodenais/cirurgia , Paraganglioma/radioterapia , Paraganglioma/cirurgia , Paraganglioma/patologia , Paraganglioma/diagnóstico , Paraganglioma/diagnóstico por imagem , Paraganglioma/terapiaRESUMO
BACKGROUND: Familial adenomatous polyposis (FAP) predisposes individuals to duodenal adenomas. This study describes the histopathological features of endoscopic and surgical specimens from the duodenum, as well as genotype-phenotype associations. METHODS: All known FAP patients were included from the Danish Polyposis Register. FAP patients were defined as having more than 100 cumulative colorectal adenomas and/or having a known germline pathogenic variant in the APC gene. Endoscopic procedures, histopathology, and genetics were evaluated. RESULTS: Of 500 FAP patients, 70.6% underwent esophagogastroduodenoscopy (EGD) at least once. Of these, 59.2% presented with detectable duodenal adenomas. The most severe morphology was tubular in 62.7% patients, tubulovillous in 25.4%, and villous in 12.0%, while the most severe dysplasia was low-grade in 67.5% patients, high-grade in 25.4%, and 6.7% had adenocarcinoma. In 6.2% of FAP patients, duodenal resection was recommended, including 29% with duodenal adenocarcinoma. The risk of duodenal surgery was 1.31 per 1,000 person-years (median age: 53 years). The predominant reason for surgery was extensive polyposis (67.7%). Of the patients who underwent duodenal resection, a median of six (IQR: 4-8) EGDs were performed within five years prior to surgery, but 67.6% and 83.9% never underwent a duodenal polypectomy or endoscopic mucosa resection, respectively. Of note, seventeen of 500 patients (3.4%) developed duodenal adenocarcinoma, of which 47% were advanced at diagnosis. Genetic evaluations revealed various pathogenic variants in the APC gene, with no strong genotype-phenotype association. CONCLUSIONS: The prevalence of duodenal adenomas and cancer in FAP warrants vigilant endoscopic surveillance. Nevertheless, the need for duodenal surgery persists and should together with endoscopic practice be monitored in national registers.
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Polipose Adenomatosa do Colo , Neoplasias Duodenais , Humanos , Polipose Adenomatosa do Colo/cirurgia , Polipose Adenomatosa do Colo/genética , Polipose Adenomatosa do Colo/patologia , Masculino , Feminino , Dinamarca , Pessoa de Meia-Idade , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/genética , Neoplasias Duodenais/patologia , Adulto , Seguimentos , Idoso , Endoscopia do Sistema Digestório , Estudos de Coortes , Adulto Jovem , Adenoma/cirurgia , Adenoma/genética , Adenoma/patologia , Adolescente , Sistema de Registros , Adenocarcinoma/cirurgia , Adenocarcinoma/genética , Adenocarcinoma/patologia , Duodeno/cirurgia , Duodeno/patologia , Estudos de Associação GenéticaRESUMO
PURPOSE: This study aimed to assess the safety and efficacy of endoscopic submucosal dissection (ESD) and pre-cutting endoscopic mucosal resection (pEMR) in treating non-ampullary duodenal subepithelial lesions (NADSELs) and to evaluate the clinical utility of endoscopic ultrasound (EUS) before endoscopic resection (ER). METHODS: In this retrospective single-centre cohort study, we compared the clinical outcomes of patients with NADSELs who underwent ESD or pEMR between January 2014 and June 2023. The accuracies of EUS in determining the pathological type and origin of the lesions were evaluated using postoperative histopathology as the gold standard. RESULTS: Overall, 56 patients with NADSELs underwent ER in this study, including 16 and 40 treated with pEMR and ESD, respectively. There were no significant differences between the two groups in terms of en bloc resection rate, complete (R0) resection rate, perioperative complication rate, and postoperative hospital length of stay (P > 0.05). However, the pEMR group had significantly shorter median operational (13.0 min vs. 30.5 min, P < 0.001) and mean fasting (1.9 days vs. 2.8 days, P = 0.006) time and lower median hospital costs (¥12,388 vs. ¥19,579, P = 0.002). The accuracies of EUS in determining the pathological type and origin of the lesions were 76.8% and 94.6%, respectively, compared with histopathological evaluation. CONCLUSIONS: EUS can accurately predict the origin of NADSELs. Suitable lesions determined to originate from the submucosa or more superficial layers using EUS can be treated using pEMR as it shortens the operational and recovery time, reduces hospitalisation costs, and achieves an R0 resection rate similar to ESD.
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Ressecção Endoscópica de Mucosa , Humanos , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , Ressecção Endoscópica de Mucosa/métodos , Idoso , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Neoplasias Duodenais/diagnóstico por imagem , Resultado do Tratamento , Endossonografia , Adulto , Tempo de InternaçãoRESUMO
Gastrointestinal stromal tumors (GISTs) are uncommon mesenchymal neoplasms primarily found in the gastrointestinal (GI) tract. While they typically occur in older adults, GISTs can manifest in individuals of any age. This publication paper presents a case study of a rare occurrence: a spontaneously ruptured duodenal GIST in a young female. Through an in-depth review of the literature, we aim to provide insights into the clinical presentation, diagnostic challenges, management strategies, and long-term outcomes associated with this unique clinical scenario.
RésuméLes tumeurs stromales gastro-intestinales (GIST) sont des néoplasmes mésenchymateux peu communs principalement trouvés dans le tractus gastro-intestinal (GI). Bien qu'elles surviennent généralement chez les adultes plus âgés, les GIST peuvent se manifester chez des individus de tout âge. Cet article présente une étude de cas d'une occurrence rare: une GIST duodénale spontanément rompue chez une jeune femme. À travers une revue approfondie de la littérature, nous visons à fournir des éclairages sur la présentation clinique, les défis diagnostiques, les stratégies de prise en charge et les résultats à long terme associés à ce scénario clinique unique.
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Neoplasias Duodenais , Tumores do Estroma Gastrointestinal , Humanos , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Neoplasias Duodenais/patologia , Neoplasias Duodenais/diagnóstico , Neoplasias Duodenais/diagnóstico por imagem , Adulto , Ruptura Espontânea , Resultado do Tratamento , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND/AIMS: There is limited knowledge regarding the management of duodenal subepithelial lesions (SELs) owing to a lack of understanding of their natural course. This study aimed to assess the natural course of asymptomatic duodenal SELs and provide management recommendations. METHODS: Patients diagnosed with duodenal SELs and followed up for a minimum of 6 months were retrospectively investigated. RESULTS: Among the 443,533 patients who underwent esophagogastroduodenoscopy between 2008 and 2020, duodenal SELs were identified in 0.39% (1,713 patients). Among them, 396 duodenal SELs were monitored for a median period of 72.5 months (interquartile range, 37.7-111.3 mo). Of them, 16 SELs (4.0%) showed substantial changes in size or morphology at a median follow-up of 35.1 months (interquartile range, 21.7-51.4 mo). Of these SELs with substantial changes, tissues of two SELs were acquired using endoscopic ultrasound-guided fine needle aspiration biopsy: one was a lipoma and the other was non-diagnostic. Three SELs were surgically or endoscopically removed; two were diagnosed as gastrointestinal stromal tumors, and one was a lipoma. An initial size of 20 mm or larger was associated with substantial changes during follow-up (p = 0.016). CONCLUSION: While the majority of duodenal SELs may not exhibit substantial interval changes, regular follow-up with endoscopy may be necessary for cases with an initial size of 20 mm or larger, considering a possibility of malignancy.
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Doenças Assintomáticas , Neoplasias Duodenais , Endoscopia do Sistema Digestório , Humanos , Estudos Retrospectivos , Feminino , Masculino , Pessoa de Meia-Idade , Neoplasias Duodenais/patologia , Neoplasias Duodenais/cirurgia , Idoso , Adulto , Lipoma/patologia , Lipoma/cirurgia , Lipoma/diagnóstico por imagem , Progressão da Doença , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Fatores de Tempo , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Duodenopatias/patologia , Duodenopatias/cirurgiaRESUMO
BACKGROUND: Due to rarity of duodenal GISTs, clinicians have few information about its clinical features, diagnosis, management and prognosis. CASE REPORT: We report a case of promptly diagnosed duodenal GIST in a 61-year-old Egyptian man presented shocked with severe attack of hematemesis and melena. Upper gastroduodenal endoscopy was done and revealed a large ulcerating bleeding mass at first part of duodenum 4 hemo-clips were applied with good hemostasis. An exploratory laparotomy and distal gastrectomy, duodenectomy and gastrojejunostomy were performed. The morphology of the mass combined with immunohistochemistry was consistent with duodenal gastrointestinal stromal tumours (GISTs) of high risk type. The patient is on amatinib one tablet daily and he was well with no evidence of tumor recurrence. CONCLUSION: despite being rare, emergency presentation with sudden severe, life-threatening hemorrhagic shock duodenal GISTs might be a cause of potentially lethal massive combined upper and lower gastrointestinal bleeding which is the key feature of this rare and challenging tumor.
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Neoplasias Duodenais , Hemorragia Gastrointestinal , Tumores do Estroma Gastrointestinal , Choque Hemorrágico , Humanos , Tumores do Estroma Gastrointestinal/complicações , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/cirurgia , Masculino , Pessoa de Meia-Idade , Hemorragia Gastrointestinal/etiologia , Neoplasias Duodenais/complicações , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Choque Hemorrágico/etiologia , Melena/etiologia , Hematemese/etiologia , GastrectomiaRESUMO
Duodenal type follicular lymphoma (DFL), a rare entity of follicular lymphoma (FL), is clinically indolent and is characterized by a low histological grade compared with nodal follicular lymphoma (NFL). Our previous reports revealed that DFL shares characteristics of both NFL and mucosa-associated lymphoid tissue (MALT) lymphoma in terms of clinical and biological aspects, suggesting its pathogenesis may involve antigenic stimulation. In contrast to NFL, the genomic methylation status of DFL is still challenging. Here, we determined the methylation profiles of DNAs from patients with DFL (n = 12), NFL (n = 10), duodenal reactive lymphoid hyperplasia (D-RLH) (n = 7), nodal reactive lymphoid hyperplasia (N-RLH) (n = 5), and duodenal samples from normal subjects (NDU) (n = 5) using methylation specific PCR of targets previously identified in MALT lymphoma (CDKN2B/P15, CDKN2A/P16, CDKN2C/P18, MGMT, hMLH-1, TP73, DAPK, HCAD). DAPK1 was frequently methylated in DFL (9/12; 75%), NFL (9/10; 90%), and D-RLH (5/7; 71%). CDKN2B/P15 sequences were methylated in six DFL samples and in only one NFL sample. Immunohistochemical analysis showed that p15 expression inversely correlated with methylation status. Genes encoding other cyclin-dependent kinase inhibitors (CDKN2A/P16, CDKN2C/P18) were not methylated in DFL samples. Methylation of the genes of interest was not detected in DNAs from D-RLH, except for DAPK1, and the difference in the extent of methylation between NDU and D-RLH was statistically significant (P = 0.013). Our results suggest that D-RLH serves as a reservoir for the development of DFL and that methylation of CDKN2B/P15 plays an important role in this process.
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Inibidor de Quinase Dependente de Ciclina p15 , Metilação de DNA , Proteínas Quinases Associadas com Morte Celular , Linfoma Folicular , Pseudolinfoma , Humanos , Linfoma Folicular/genética , Linfoma Folicular/patologia , Linfoma Folicular/metabolismo , Proteínas Quinases Associadas com Morte Celular/genética , Masculino , Pseudolinfoma/genética , Pseudolinfoma/patologia , Feminino , Pessoa de Meia-Idade , Inibidor de Quinase Dependente de Ciclina p15/genética , Inibidor de Quinase Dependente de Ciclina p15/metabolismo , Idoso , Neoplasias Duodenais/genética , Neoplasias Duodenais/patologia , Neoplasias Duodenais/metabolismo , AdultoRESUMO
BACKGROUND: Pancreatoduodenectomy is the only cure for cancers of the pancreas and the periampullary region but has considerable operative complications and uncertain prognosis. Our goal was to analyse temporal improvements and provide contemporary population-based benchmarks for outcomes following pancreatoduodenectomy. METHODS: We empanelled a cohort comprising all patients in Sweden with pancreatic or periampullary cancer treated with pancreatoduodenectomy from 1964 to 2016 and achieved complete follow-up through 2016. We analysed postoperative deaths and disease-specific net survival. RESULTS: We analysed 5923 patients with cancer of the pancreas (3876), duodenum (444), bile duct (504), or duodenal papilla (963) who underwent classic (3332) or modified (1652) Whipple's procedure or total pancreatectomy (803). Postoperative deaths declined from 17.2% in the 1960s to 1.6% in the contemporary time period (2010-2016). For all four cancer types, median, 1-year and 5-year survival improved substantially over time. Among patients operated between 2010 and 2016, 5-year survival was 29.0% (95% confidence interval (CI): 25.5, 33.0) for pancreatic cancer, 71.2% (95% CI: 62.9, 80.5) for duodenal cancer, 30.8% (95% CI: 23.0, 41.3) for bile duct cancer, and 62.7% (95% CI: 55.5, 70.8) for duodenal papilla cancer. CONCLUSION: There is a continuous and substantial improvement in the benefit-harm ratio after pancreatoduodenectomy for cancer.
Assuntos
Ampola Hepatopancreática , Neoplasias Duodenais , Neoplasias Pancreáticas , Pancreaticoduodenectomia , Humanos , Pancreaticoduodenectomia/métodos , Pancreaticoduodenectomia/mortalidade , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/mortalidade , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Suécia/epidemiologia , Ampola Hepatopancreática/cirurgia , Ampola Hepatopancreática/patologia , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/mortalidade , Neoplasias Duodenais/patologia , Resultado do Tratamento , Neoplasias do Ducto Colédoco/cirurgia , Neoplasias do Ducto Colédoco/mortalidade , Neoplasias do Ducto Colédoco/patologia , Idoso de 80 Anos ou mais , Adulto , Neoplasias dos Ductos Biliares/cirurgia , Neoplasias dos Ductos Biliares/mortalidadeRESUMO
BACKGROUND: Cancer arising in the periampullary region can be anatomically classified in pancreatic ductal adenocarcinoma (PDAC), distal cholangiocarcinoma (dCCA), duodenal adenocarcinoma (DAC), and ampullary carcinoma. Based on histopathology, ampullary carcinoma is currently subdivided in intestinal (AmpIT), pancreatobiliary (AmpPB), and mixed subtypes. Despite close anatomical resemblance, it is unclear how ampullary subtypes relate to the remaining periampullary cancers in tumor characteristics and behavior. METHODS: This international cohort study included patients after curative intent resection for periampullary cancer retrieved from 44 centers (from Europe, United States, Asia, Australia, and Canada) between 2010 and 2021. Preoperative CA19-9, pathology outcomes and 8-year overall survival were compared between DAC, AmpIT, AmpPB, dCCA, and PDAC. RESULTS: Overall, 3809 patients were analyzed, including 348 DAC, 774 AmpIT, 848 AmpPB, 1,036 dCCA, and 803 PDAC. The highest 8-year overall survival was found in patients with AmpIT and DAC (49.8% and 47.9%), followed by AmpPB (34.9%, P < 0.001), dCCA (26.4%, P = 0.020), and finally PDAC (12.9%, P < 0.001). A better survival was correlated with lower CA19-9 levels but not with tumor size, as DAC lesions showed the largest size. CONCLUSIONS: Despite close anatomic relations of the five periampullary cancers, this study revealed differences in preoperative blood markers, pathology, and long-term survival. More tumor characteristics are shared between DAC and AmpIT and between AmpPB and dCCA than between the two ampullary subtypes. Instead of using collective definitions for "periampullary cancers" or anatomical classification, this study emphasizes the importance of individual evaluation of each histopathological subtype with the ampullary subtypes as individual entities in future studies.
Assuntos
Ampola Hepatopancreática , Carcinoma Ductal Pancreático , Colangiocarcinoma , Neoplasias do Ducto Colédoco , Neoplasias Duodenais , Neoplasias Pancreáticas , Humanos , Masculino , Feminino , Ampola Hepatopancreática/patologia , Ampola Hepatopancreática/cirurgia , Taxa de Sobrevida , Neoplasias Duodenais/patologia , Neoplasias Duodenais/cirurgia , Idoso , Colangiocarcinoma/patologia , Colangiocarcinoma/cirurgia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Pessoa de Meia-Idade , Carcinoma Ductal Pancreático/patologia , Carcinoma Ductal Pancreático/cirurgia , Neoplasias do Ducto Colédoco/patologia , Neoplasias do Ducto Colédoco/cirurgia , Neoplasias do Ducto Colédoco/mortalidade , Seguimentos , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Prognóstico , Estudos de Coortes , Estudos RetrospectivosRESUMO
The guidelines recently recognized the intra-ampullary papillary tubular neoplasm (IAPN) as a distinct tumor entity. However, the data on IAPN and its distinction from other ampullary tumors remain limited. A detailed clinicopathologic analysis of 72 previously unpublished IAPNs was performed. The patients were: male/female=1.8; mean age=67 years (range: 42 to 86 y); mean size=2.3 cm. Gross-microscopic correlation was crucial. From the duodenal perspective, the ampulla was typically raised symmetrically, with a patulous orifice, and was otherwise covered by stretched normal duodenal mucosa. However, in 6 cases, the protrusion of the intra-ampullary tumor to the duodenal surface gave the impression of an "ampullary-duodenal tumor," with the accurate diagnosis of IAPN established only by microscopic correlation illustrating the abrupt ending of the lesion at the edge of the ampulla. Microscopically, the preinvasive component often revealed mixed phenotypes (44.4% predominantly nonintestinal). The invasion was common (94%), typically small (mean=1.2 cm), primarily pancreatobiliary-type (75%), and showed aggressive features (lymphovascular invasion in 66%, perineural invasion in 41%, high budding in 30%). In 6 cases, the preinvasive component was pure intestinal, but the invasive component was pancreatobiliary. LN metastasis was identified in 42% (32% in those with ≤1 cm invasion). The prognosis was significantly better than ampullary-ductal carcinomas (median: 69 vs. 41 months; 3-year: 68% vs. 55%; and 5-year: 51% vs. 35%, P =0.047). In conclusion, unlike ampullary-duodenal carcinomas, IAPNs are often (44.4%) predominantly nonintestinal and commonly (94%) invasive, displaying aggressive features and LN metastasis even when minimally invasive, all of which render them less amenable to ampullectomy. However, their prognosis is still better than that of the "ampullary-ductal" carcinomas, with which IAPNs are currently grouped in CAP protocols (while IAPNs are kindreds of intraductal tumors of the pancreatobiliary tract, the latter represents the ampullary counterpart of pancreatic adenocarcinoma/cholangiocarcinoma).
Assuntos
Ampola Hepatopancreática , Neoplasias do Ducto Colédoco , Humanos , Masculino , Feminino , Ampola Hepatopancreática/patologia , Idoso , Pessoa de Meia-Idade , Adulto , Idoso de 80 Anos ou mais , Neoplasias do Ducto Colédoco/patologia , Neoplasias do Ducto Colédoco/mortalidade , Neoplasias do Ducto Colédoco/cirurgia , Invasividade Neoplásica , Neoplasias Duodenais/patologia , Carcinoma Papilar/patologia , Carcinoma Papilar/cirurgiaRESUMO
INTRODUCTION: Superficial non-ampullary duodenal epithelial tumors (SNADETs) include low-grade adenoma (LGA) and high-grade adenoma or carcinoma (HGA/Ca) and are classified into two different epithelial subtypes, gastric-type (G-type) and intestinal-type (I-type). We attempted to distinguish them by endoscopic characteristics including magnifying endoscopy with narrow-band imaging (M-NBI). METHODS: Various endoscopic and M-NBI findings of 286 SNADETs were retrospectively reviewed and compared between G- and I-types and histological grades. M-NBI findings were divided into four patterns based on the following vascular patterns; absent, network, intrastructural vascular (ISV), and unclassified. Lesions displaying a single pattern were classified as mono-pattern and those displaying multiple patterns as mixed-pattern. Lesions showing CDX2 positivity were categorized as I-types and those showing MUC5AC or MUC6 positivity were categorized as G-types based on immunohistochemistry. RESULTS: Among 286 lesions, 23 (8%) were G-type and 243 (85%) were I-type. More G-type lesions were located oral to papilla (91.3 vs. 45.6%, p < 0.001), and had protruding morphology compared to those of I-types (65.2 vs. 14.4%, p < 0.001). The major M-NBI pattern was ISV in G-type (78.2 vs. 26.3%, p < 0.001), and absent for I-type (0 vs. 34.5%, p = 0.003). Three endoscopic characteristics; location oral to papilla, protruding morphology, and major M-NBI pattern (ISV) were independent predictors for G-type. Mixed-pattern was more common in HGA/Ca than LGA for I-type (77.0 vs. 58.8%, p = 0.01); however, there was no difference for those in G-type. CONCLUSION: Endoscopic findings including M-NBI are useful to differentiate epithelial subtypes.