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1.
Khirurgiia (Mosk) ; (1): 14-24, 2020.
Artigo em Russo | MEDLINE | ID: mdl-31994495

RESUMO

OBJECTIVE: Adjustment of diagnostics and management of the surgical treatment of patients with intraductal papillary mucinous tumor of pancreas. MATERIAL AND METHODS: From 2012 to 2018, 45 patients with intraductal papillary mucinous tumor were observed. During the observation the ultrasound examination, contrast-enhanced computed tomography and magnetic resonance tomography with contrast were used. In 29 cases radical surgery was performed, nonradical in 1 case; case follow-up is chosen for 15 patients. RESULTS: Intraductal papillary mucinous tumor was diagnosed and the definition of the type of tumor was made on the base of 2 types of imaging methods. Intraductal papillary mucinous tumor type 1 was founded in 5 (11%), whereas intraductal papillary mucinous tumor type 2 was founded in 20 (44,5%) and intraductal papillary mucinous tumor type 3 was observed in 20 (44,5%) cases. Intraductal papillary mucinous tumor associated with carcinoma was observed in 16 cases. Pancreaticoduodenal resection was performed in 20, distal exsection of pancreas was performed in 4 cases (2 in open manner access, 2 in robot-assisted manner), pancreatic head resection was performed in 3 cases (1 in open manner access, 2 in laparoscopic access) and in 2 cases the duodenopancreatectomy was performed. Explorative laparotomy was performed in case of intraductal papillary mucinous tumor type 2 associated with mucilaginous carcinoma and miliary metastasis in the liver. Early postoperative complications were observed in 5 cases (16, 6%): biliary fistula (n=2), postoperative wound infection (n=2), arrosive hemorrhage type B in ISGPS (n=1, was treated in an X-ray endovascular manner). Case follow-up was chosen in 15 cases of intraductal papillary mucinous tumor over the course of 6 to 74 months and disease progression was not observed. CONCLUSION: Intraductal papillary mucinous tumor is a condition associated with high risk of malignant change and demands early disease detection. The treatment should be provided in medical centers that specialize in the pancreas deceases, where a full patient examination as well as a clear-eyed understanding of diagnostic information with the execution of desirable type of surgical intervention with the guaranty of achievement R0 condition can be offered.


Assuntos
Neoplasias Intraductais Pancreáticas/diagnóstico , Neoplasias Intraductais Pancreáticas/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Humanos , Pâncreas/patologia , Pâncreas/cirurgia , Pancreatectomia/efeitos adversos , Neoplasias Intraductais Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/diagnóstico por imagem , Pancreaticoduodenectomia/efeitos adversos
2.
Zhonghua Wai Ke Za Zhi ; 58(1): 22-26, 2020 Jan 01.
Artigo em Chinês | MEDLINE | ID: mdl-31902165

RESUMO

The incidence of pancreatic cancer (PC) has continuously shown an upward trend all over the world. It remains one of the most challenging malignant tumors in clinical practice and is characterized by difficult diagnosis in early stages, low surgical resection rate and poor prognosis. Due to its significant genetic heterogeneity, there are notable individual differences in disease progression, clinical efficacy, sensitivity to chemoradiotherapy, and prognosis among PC patients. In-depth study is needed to reveal the molecular biological characteristics of different PC subtypes and their correlation with clinical manifestations and chemoradiotherapy sensitivity, which could contribute to develop corresponding targeted therapeutic strategies.It is not only the fundamental basis for the innovation of PC morphological classification to molecular subtyping, but also a prerequisite for achieving a shift in treatment mode from "standard therapeutic strategy for different diseases" to "treat the same disease with different strategies" .This article reviews several hot issues on the comprehensive diagnosis and treatment of PC in the era of targeted therapy and prospects its future development.


Assuntos
Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Quimiorradioterapia , Progressão da Doença , Humanos , Terapia de Alvo Molecular , Prognóstico , Radioterapia , Resultado do Tratamento
3.
Oncology ; 98(1): 53-60, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31578019

RESUMO

INTRODUCTION: The present study evaluated the potential benefit of adding cetuximab to neoadjuvant, adjuvant, or palliative standard therapy for pancreatic cancer. METHODS: A systematic literature search was performed in MEDLINE, Web of Science, and Cochrane Central Register of Controlled Trials (CENTRAL). Only randomised controlled trials (RCTs) investigating the effect of adding cetuximab to standard chemotherapy in pancreatic cancer were included. Evaluated outcomes were overall survival, progression-free survival, objective response, and toxicity. For overall survival and progression-free survival, hazard ratios (HR) with 95% confidence intervals (CI) were chosen as effect measure. For objective response, odds ratios (OR) with 95% CI were used. Analysis was based on a random effects model. RESULTS: After screening 568 publications, a total of 4 RCTs with 924 patients were included. In all trials, patients were adequately randomised with balanced intervention and control groups. There was no significant difference in overall survival (HR 1.04; 95% CI: 0.90-1.19; p = 0.60), progression-free survival (HR 1.06; 95% CI: 0.93-1.22; p = 0.36), or objective response (OR 0.99; 95% CI: 0.66 -1.49; p = 0.96) when adding cetuximab to a standard therapy. Toxicity was the same or higher in each of the included trials. According to GRADE, the certainty of the evidence is high. Therefore, adding cetuximab to pancreatic cancer therapy has no clinically relevant benefit. CONCLUSION: In the presence of no survival benefit, increased toxicity, and higher costs, a decreased cost-benefit ratio compared to the standard care must be suggested. Conducting further RCTs in unselected pancreatic cancer populations is unlikely to change this conclusion.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Cetuximab/uso terapêutico , Neoplasias Pancreáticas/tratamento farmacológico , Antineoplásicos Imunológicos/administração & dosagem , Antineoplásicos Imunológicos/efeitos adversos , Cetuximab/administração & dosagem , Cetuximab/efeitos adversos , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/mortalidade , Prognóstico , Resultado do Tratamento
4.
Acta Cytol ; 64(1-2): 103-123, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-30970350

RESUMO

Advanced methods of molecular characterization have elucidated the genetic, epigenetic, and proteomic alterations associated with the broad spectrum of pancreatic disease, particularly neoplasia. Next-generation sequencing, in particular, has revealed the genomic diversity among pancreatic ductal adenocarcinoma, neuroendocrine and acinar tumors, solid pseudopapillary neoplasm, and other pancreatico-biliary neoplasms. Differentiating these entities from one another by morphologic analysis alone may be challenging, especially when examining the small quantities of diagnostic material inherent to cytologic specimens. In order to enhance the sensitivity and specificity of pancreatic cytomorphology, multiple diagnostic, prognostic, and predictive ancillary tests have been and continue to be developed. Although a great number of such tests have been developed for evaluation of specimens collected from cystic lesions and strictures, ancillary techniques also play a significant role in the evaluation of cytologic specimens obtained from solid lesions of the pancreas. Furthermore, while some tests have been developed to differentiate diagnostic entities from one another, others have been developed to simply identify dysplasia and malignancy. Ancillary studies are particularly important in the subset of cases for which cytomorphologic analysis provides a result that is equivocal or insufficient to guide clinical management. Selection of appropriate ancillary testing modalities requires familiarity with both their methodology and the molecular basis of the pancreatic diseases for which testing is being performed.


Assuntos
Carcinoma Ductal Pancreático/diagnóstico , Citodiagnóstico/métodos , Pâncreas/patologia , Neoplasias Pancreáticas/diagnóstico , Carcinoma Ductal Pancreático/genética , Carcinoma Ductal Pancreático/metabolismo , Regulação Neoplásica da Expressão Gênica , Humanos , Imuno-Histoquímica , MicroRNAs/genética , Pâncreas/metabolismo , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/metabolismo , Sensibilidade e Especificidade , Proteína Smad4/genética , Proteína Smad4/metabolismo
5.
Acta Cytol ; 64(1-2): 124-135, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31509835

RESUMO

Pancreatic cysts are increasingly detected on imaging studies. Accurate determination of the cyst type is important to provide appropriate care for the patients. It is also very clear that not one single modality can provide adequate diagnostic information. A multidisciplinary approach is the key to the diagnosis of pancreatic cysts. In this setting, the role of ancillary testing, which includes biochemical testing (carcinoembryonic antigen and amylase levels in the cyst), molecular testing (e.g., KRAS, GNAS, VHL, and CTNB1), and/or immunohistochemical tests are very important to obtain an accurate diagnosis. This review will discuss helpful ancillary tests in common pancreatic cyst neoplasms and how to approach the diagnosis of pancreatic cysts.


Assuntos
Carcinoma Ductal Pancreático/diagnóstico , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Cisto Pancreático/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Antígeno Carcinoembrionário/metabolismo , Carcinoma Ductal Pancreático/genética , Carcinoma Ductal Pancreático/metabolismo , Análise Mutacional de DNA/métodos , Humanos , Imuno-Histoquímica/métodos , Mutação , Cisto Pancreático/genética , Cisto Pancreático/metabolismo , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/metabolismo , Proteínas Proto-Oncogênicas p21(ras)/genética
6.
Gut ; 69(1): 7-17, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31672839

RESUMO

BACKGROUND AND AIM: The International Cancer of the Pancreas Screening Consortium met in 2018 to update its consensus recommendations for the management of individuals with increased risk of pancreatic cancer based on family history or germline mutation status (high-risk individuals). METHODS: A modified Delphi approach was employed to reach consensus among a multidisciplinary group of experts who voted on consensus statements. Consensus was considered reached if ≥75% agreed or disagreed. RESULTS: Consensus was reached on 55 statements. The main goals of surveillance (to identify high-grade dysplastic precursor lesions and T1N0M0 pancreatic cancer) remained unchanged. Experts agreed that for those with familial risk, surveillance should start no earlier than age 50 or 10 years earlier than the youngest relative with pancreatic cancer, but were split on whether to start at age 50 or 55. Germline ATM mutation carriers with one affected first-degree relative are now considered eligible for surveillance. Experts agreed that preferred surveillance tests are endoscopic ultrasound and MRI/magnetic retrograde cholangiopancreatography, but no consensus was reached on how to alternate these modalities. Annual surveillance is recommended in the absence of concerning lesions. Main areas of disagreement included if and how surveillance should be performed for hereditary pancreatitis, and the management of indeterminate lesions. CONCLUSIONS: Pancreatic surveillance is recommended for selected high-risk individuals to detect early pancreatic cancer and its high-grade precursors, but should be performed in a research setting by multidisciplinary teams in centres with appropriate expertise. Until more evidence supporting these recommendations is available, the benefits, risks and costs of surveillance of pancreatic surveillance need additional evaluation.


Assuntos
Carcinoma/diagnóstico , Detecção Precoce de Câncer/métodos , Neoplasias Pancreáticas/diagnóstico , Fatores Etários , Pesquisa Biomédica/métodos , Carcinoma/genética , Predisposição Genética para Doença , Mutação em Linhagem Germinativa , Humanos , Programas de Rastreamento/métodos , Neoplasias Pancreáticas/genética , Vigilância da População/métodos , Fatores de Risco
7.
Zhonghua Wai Ke Za Zhi ; 57(12): 912-916, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31826595

RESUMO

Objective: To evaluate the role of multidisciplinary team (MDT) clinic in the diagnosis of pancreatic diseases and patient compliance with MDT advice in the current medical system. Methods: The study included 512 patients that had visited the pancreas-oriented MDT clinic of Zhongshan Hospital between May 2015 and May 2019.The clinical and pathological data were collected and analyzed retrospectively. Diseases were classified in accordance with 2010 WHO classification of tumors of the digestive system and usual clinical practices. Compliance was determined according to whether a patient received corresponding therapies or undergoing further checks or follow-ups. Results: Among the 512 patients that had visited the MDT clinic, 121 patients were referred due to undetermined diagnosis. Classified according to the final diagnosis, the rate of undetermined diagnosis in different disease categories from high to low in order was inflammatory diseases of the pancreas (75.0%, 24/32), other lesions of the pancreas (56.1%,23/41), pancreatic cystic lesions (19.1%,17/89), pancreatic carcinomas (18.3%,48/262) and pancreatic neuroendocrine neoplasms (pNEN)(10.2%,9/88). The MDT clinic made diagnosis to 68 patients directly with an accuracy of 89.7%. The rate of compliance in the entire cohort was 63.4%. The rate of compliance of patients from June 2017 to May 2019 (68.4%) was higher than that of patients from May 2015 to May 2017(59.6%). The compliance rate of patients in different disease categories from high to low in order was inflammatory diseases of the pancreas(84.4%, 27/32), pancreatic carcinomas (67.9%, 178/262), pNEN(60.2%,53/88), other lesions of the pancreas (56.1%,23/41), and pancreatic cystic lesions(49.4%, 44/89). The compliance rate of patients with different MDT advice from high to low in order was best supportive care(78.6%,22/28), antitumor approaches beyond surgery(71.6%,159/222), further tests(62.6%, 77/123), surgery(53.7%, 65/121) and follow-up(49.2%, 31/63). In patients suggested for surgery, the compliance rate of patients with carcinomas(67.4%, 33/49) was higher than patients with other kinds of neoplasms. Conclusions: MDT clinic could facilitate the diagnosis of pancreatic diseases conveniently and inexpensively. The overall compliance rate of MDT clinic patients is rather low, and patients with carcinomas have a relative high rate of compliance with the suggestion of surgery.


Assuntos
Ambulatório Hospitalar , Pancreatopatias/diagnóstico , Pancreatopatias/terapia , Cooperação do Paciente , China , Estudos de Coortes , Humanos , Neoplasias Pancreáticas/diagnóstico , Equipe de Assistência ao Paciente , Estudos Retrospectivos
8.
Medicine (Baltimore) ; 98(51): e18487, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31861032

RESUMO

Pancreatic cancer is one of the most malignant tumors worldwide. DNA replication plays a critical role in the occurrence and development of pancreatic cancer. TYMS encodes thymidylate synthase, which is important for DNA synthesis. The TYMS gene has been assessed in some tumors. However, the specific role of TYMS in pancreatic cancer has not been identified. This study was designed to clarify the diagnostic and prognostic significance of TYMS in pancreatic cancer.The Cancer Genome Atlas (TCGA) database was used to compare TYMS expression in pancreatic cancer, and ROC curve analysis was used to investigate its diagnostic value. The correlation between clinical characteristics and TYMS expression was analyzed, and the prognostic value of TYMS expression in the patients with pancreatic cancer was assessed by Kaplan-Meier curves and Cox analysis.TYMS was upregulated in pancreatic cancer and associated with poor overall survival (OS) and recurrence-free survival (RFS). Univariate and multivariate survival analysis demonstrated that TYMS is an independent risk factor for OS and RFS in patients with pancreatic cancer.The upregulation of TYMS in pancreatic cancer leads to unfavorable OS and RFS in patients, and represents a diagnostic and prognostic biomarker for patients with pancreatic cancer.


Assuntos
Neoplasias Pancreáticas/metabolismo , Timidilato Sintase/metabolismo , Biomarcadores Tumorais/metabolismo , Estudos de Casos e Controles , China/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/mortalidade , Prognóstico , Regulação para Cima
9.
Medicine (Baltimore) ; 98(50): e18102, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31852070

RESUMO

RATIONALE: Intraductal papillary and mucinous neoplasms of the pancreas (IPMN) are preneoplastic lesions diagnosed with an increasing incidence. Recently, several groups have described, in up to 70% of IPMN, activating mutations of the G-protein alpha stimulatory sub-unit (Gsα subunit) gene (GNAS). GNAS-activating somatic, post-zygotic, mutations are also associated with McCune-Albright syndrome (MCAS) characterized by fibrous dysplasia, precocious puberty, and café-au-lait spots. PATIENT CONCERNS: We herein report a patient with McCune Albright Syndrome that presented with malignant IPMN and underwent pancreatic resection. DIAGNOSES AND INTERVENTIONS: Leucocyte and duodenum juice DNA analysis, endoscopically collected from secretin-stimulated pancreatic juice revealed the same (GNAS) activating mutation also found in the invasive pancreatic colloid adenocarcinoma arising from intestinal subtype IPMN. OUTCOMES: Thirty months after surgery, the patient was alive with recurrence (bone only metastasis). LESSONS: In this observation, we show that MCAS should be view as a new genetic predisposition to IPMN associated pancreatic cancer, and consequently a targeted screening in this high-risk population might be proposed.


Assuntos
Adenocarcinoma Mucinoso/genética , Carcinoma Ductal Pancreático/genética , Cromograninas/genética , Displasia Fibrosa Poliostótica/genética , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Predisposição Genética para Doença , Mutação , Neoplasias Pancreáticas/genética , Adenocarcinoma Mucinoso/diagnóstico , Adenocarcinoma Mucinoso/etiologia , Biópsia por Agulha , Carcinoma Ductal Pancreático/diagnóstico , Carcinoma Ductal Pancreático/etiologia , Cromograninas/metabolismo , Análise Mutacional de DNA , DNA de Neoplasias/genética , Endossonografia , Feminino , Displasia Fibrosa Poliostótica/complicações , Displasia Fibrosa Poliostótica/diagnóstico , Subunidades alfa Gs de Proteínas de Ligação ao GTP/metabolismo , Humanos , Pessoa de Meia-Idade , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/etiologia , Tomografia Computadorizada por Raios X
10.
Medicine (Baltimore) ; 98(50): e18275, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31852099

RESUMO

RATIONALE: Surgery for patients with multiple endocrine neoplasia type 1(MEN-1) related gastrinoma remains controversial and total pancreatectomy (TP) has rarely been performed. We reported a case of patient with MEN-1 related gastrinoma treated by TP. PATIENT CONCERNS: A 46-year-old female was admitted to our hospital due to abdominal distension and diarrhea for 2 years. The patient underwent pituitary tumor resection and kidney stone lithotripsy 10 years ago. DIAGNOSES: Abdominal computed tomography showed single lesion in the duodenum and multiple lesions throughout the pancreas. The patient's gastrin level was significantly increased (1080 pg/ml). These findings in combination with the pituitary tumor history suggested the presence of gastrinoma associated with MEN-1 syndrome. INTERVENTION: An exploratory laparotomy was performed. Intraoperative ultrasound confirmed the numerous tumors diffusely distributed throughout the pancreas and the patient eventually underwent TP. OUTCOMES: Twelve months later, the patient was hospitalized again for anastomotic fistula and underwent a partial gastrectomy, small bowel resection and drainage of the abscess. One month later, she received gastrostomy and jejunostomy due to digestive tract fistula, and died a month later (14 months after TP). LESSONS: There still might be the possibility of recurrence even after radical surgical resection of gastrinomas, and we suggest the need to measure the basal acid output and maintain regular anti-acid therapy in the long-term follow-up of patients with MEN-1 related gastrinoma.


Assuntos
Neoplasias Duodenais/etiologia , Gastrinoma/cirurgia , Neoplasia Endócrina Múltipla Tipo 1/etiologia , Pancreatectomia/efeitos adversos , Neoplasias Pancreáticas/cirurgia , Biópsia , Neoplasias Duodenais/diagnóstico , Evolução Fatal , Feminino , Gastrinoma/diagnóstico , Humanos , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Tomografia Computadorizada por Raios X
11.
Chirurgia (Bucur) ; 114(5): 550-563, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31670630

RESUMO

Digestive cancers diagnosed during pregnancy are rare and are sporadically reported. The shift of pregnancy towards the age interval of 30-39 years old (and even 40-49 years old) could explain the more and more frequent superposition of pregnancy and cancer. Pancreatic Neuroendocrine Tumours (PNET) originate in the insular endocrine tissue and are extremely rare; they have a slow, less aggressive neoplastic development, with the capacity of secreting and storing different peptides and neuroamines. From the large group of NET tumours, approximately 70% have a gastroenterohepatic (GET) localisation and represent less than 2% of the digestive tract tumours. PNET incidence is extremely reduced, of approximately 1/100.000. The association of pregnancy and PNET in literature is exceptional until now only 39 cases were reported. The most frequent PNET in pregnant patients is the insulinoma with the starting point in pancreatic B-cells, with 27 out of 39 PNET worldwide reported cases until 2012. Establishing a diagnosis in pregnant patients is difficult in the first period of pregnancy because physiologically, the glucose tends to be low. This may be a consequence of the increased secretion of insulin and sensibility to insulin, probably as an effect of increased oestrogen levels. Furthermore, there are other signs which could suggest the existence of a "problem", which are relatively common during the first trimester of pregnancy: unexplained fatigue, vomiting, hypotension, rare episodes of hypoglycaemia. Performing paraclinical tests on these patients is difficult; an essential indicator of the opportunity for the investigation is the pregnancy trimester. The precaution about a foetal injury when indicating an invasive exploration (imaging, endoscopy, etc.) delays the diagnosis. The insulinomas have a rather benign potential than an aggressive nature, and their prognosis is usually favourable, with therapeutic possibilities easy to administrate and control in pregnant patients. Medical treatment is used in patients when there is a positive diagnosis but the topography of the insulinoma was not identified, when the symptomatology is easy to control through conserving therapy until the foetus is mature enough or immediately after birth, in pregnant patients who refuse the surgical procedure, or in the case of metastasis of malignant insulinoma. Surgical treatment indeed remains the only therapeutic method, but most often it is scheduled usually after birth, or if the situation requires, as late as possible after the foetus has a convenient age (after 28 weeks).


Assuntos
Insulinoma/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Complicações Neoplásicas na Gravidez/diagnóstico , Adulto , Feminino , Humanos , Insulinoma/cirurgia , Pessoa de Meia-Idade , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Gravidez , Complicações Neoplásicas na Gravidez/cirurgia , Resultado do Tratamento
12.
Chirurgia (Bucur) ; 114(5): 639-649, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31670640

RESUMO

Pancreatic neuroendocrine tumors (PNETs) are rare and characterized by widely variable clinical presentation and often challenging surgical management. Methods: Retrospective study conducted during the last 15 years at the First Surgical Clinic of the Iasi St Spiridon University Hospital, and which included all the patients diagnosed with pancreatic endocrine tumors by immunohistochemistry. Results: There were 26 cases diagnosed with PNET. The male/female ratios was 7/19 and mean age 41.93 +- 2.48 years (range 20-79 years). Of the PNET cases 13 were insulinomas, 5 gastrinomas, 2 gastrinomas associated with other endocrine neoplasms (Wermer syndrome), 5 non-functional endocrine pancreatic tumors and 1 ACTHoma. Clinical manifestations depended on tumor type: hypoglycemia and Whipple triad for insulinoma, Zollinger Ellison syndrome and complicated peptic ulcer (hemorrhage, perforation) for gastrinoma, Cushing syndrome for ACTHoma. Biological diagnosis included biological markers (e.g. insulin, gastrin and cortisol). Tumor site and size at diagnosis were determined by ultrasound, CT-scan, angiography, PETscan, octreoscan and intraoperative ultrasound. Surgical procedures for PNET insulinomas were: tumor resection - 6 cases; left splenopancreatectomy - 3 cases; left spleen-preserving pancreatectomy - 2 cases; pancreaticoduodenectomy - 2 cases. We also present 4 cases of gastrinoma with multiple ulcers and multiple surgical interventions for hemorrhage and perforation with peritonitis. The two patients with Wermer syndrome also had ulcers complicated with hemorrhage and peritonitis and parathyroid adenoma. Nonfunctional pancreatic endocrine tumors were diagnosed in 5 women of which in 3 the tumors were located in the pancreatic tail (in which splenopancreatectomy and left pancreatectomy with spleen preservation were performed) and in 2 in the pancreatic head (in which pancreaticoduodenectomy and Beger type operation were performed). Conclusions: Knowledge of clinical signs of secreting tumors and exploring the patients are of crucial importance for management of PNETs. Immunohistochemistry is mandatory for confirming the diagnosis and assessing the proliferation and biological behavior of the tumor, thus facilitating the administration of specific therapy. Aggressive surgical treatment is indicated, even in advanced stages.


Assuntos
Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Pancreatectomia/métodos , Neoplasias Pancreáticas/diagnóstico , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
13.
Pathologe ; 40(Suppl 3): 311-315, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31705231

RESUMO

BACKGROUND: Cytology has a key role in the step-wise diagnostic approach to pancreatic mass lesions. Brush cytology and ultrasound-guided endoscopic fine-needle aspiration provide specimens for diagnosis prior to surgical or conservative therapy. The diagnostic system of the Papanicolaou Society of Cytopathology provides a conceptual framework for reporting these specimens. Cystic lesions represent a particular challenge in pancreatic cytology, as in many instances a purely morphological approach will not result in an adequate diagnostic interpretation. Noteworthy from a conceptual point of view is how the Papanicolaou Society System incorporates non-morphological methods: laboratory chemical (CEA >192 ng/ml) and molecular (KRAS and/or GNAS mutations) findings are part of the formal diagnostic criteria for neoplastic cysts. RESULTS: The Bern experience shows that such an integrated approach results in a significantly increased diagnostic yield. Among 83 samples analyzed, adequate DNA could be extracted in 79 samples (95%). Next generation sequencing identified pathogenic mutations in 46 cases (58%). Of these, in 35 (76%) a neoplastic cyst could not have been diagnosed by morphology alone. CONCLUSION: These findings illustrate a new perspective for diagnostic situations, where morphology alone does allow for a sufficient diagnostic work-up. Along this line of thinking, liquid biopsy should not be regarded as a replacement, but rather an extension of the cytology's diagnostic armamentarium, according to the principle of "doing more with less."


Assuntos
Pâncreas/citologia , Cisto Pancreático , Neoplasias Pancreáticas , Análise Mutacional de DNA , DNA de Neoplasias/genética , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Humanos , Mutação , Cisto Pancreático/diagnóstico , Cisto Pancreático/genética , Cisto Pancreático/patologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia
14.
Pan Afr Med J ; 33: 238, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692695

RESUMO

Multiple endocrine neoplasia type 1 (MEN1) is a rare disease, defined as a tumor developing in at least two endocrine glands including the anterior pituitary gland, the parathyroid glands and the duodenopancreatic endocrine tissue. This disorder, inherited in an autosomal dominant pattern, is caused by mutations in the MEN1 gene encoding the tumor suppressor menin and located on chromosome 11q13. However, sporadic cases account for 8-14%. The first endocrine lesion may be solitary in approximately 75% of cases. However, all major alterations can be inaugural. We here report a case of multiple endocrine neoplasia type 1 revealed by aggressive somatoprolactinic pituitary adenoma which didn't respond to conventional treatment. The detection of primary hyperparathyroidism as well as neuroendocrine tumor of the pancreas seven years later make this a very particular case. Therapeutic options are discussed within the multidisciplinary team specialized in endocrine diseases.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Adenoma/diagnóstico , Adenoma/patologia , Adulto , Humanos , Hiperparatireoidismo Primário/diagnóstico , Masculino , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Hipofisárias/patologia , Prolactina/metabolismo
15.
Khirurgiia (Mosk) ; (11): 81-87, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31714535

RESUMO

Intraductal papillary mucinous neoplasm (IPMN) is the most common cystic pancreatic tumor. There are 3 ductal types of this tumor depending on localization. Several histological subtypes determine clinical course and prognosis. The problems of diagnosis, surgical treatment and follow-up are reviewed in several guidelines. Literature review devoted to pancreatic IPMN is presented in the article, the latest guidelines are compared.


Assuntos
Neoplasias Intraductais Pancreáticas/diagnóstico , Neoplasias Intraductais Pancreáticas/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Humanos , Neoplasias Intraductais Pancreáticas/patologia , Neoplasias Pancreáticas/patologia , Guias de Prática Clínica como Assunto , Prognóstico
16.
Medicine (Baltimore) ; 98(46): e18001, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725669

RESUMO

RATIONALE: Pediatric pancreatic teratoma (PPT) is a rare tumor with unclear clinicopathologic features and treatment strategy. PATIENT CONCERNS: A 13-month-old boy was admitted to the hospital with a complaint of a palpable epigastric mass. DIAGNOSES: The lesion was diagnosed as benign mature cyst teratoma via postoperative pathological examination. INTERVENTIONS: Pylorus-preserving pancreaticoduodenectomy (PPPD) was performed on the patient. The entire mass was resected from the head of the pancreas and sent to the laboratory for frozen section evaluation. OUTCOMES: The patient was followed up for 15 months. He did not undergo recurrence or PPPD-related complications. LESSONS: The differential diagnosis of retroperitoneal occupying lesions among children must consider mature cystic teratomas. Compete surgical resection combined with subsequent postoperative outpatient follow-up remains the primary choice for the management of PPTs.PPTs are extremely rare tumors with unclear clinicopathologic features and treatment strategy. This study aims to explore the clinical characteristics of and treatment strategy for these tumors. We reported a 13-month-old patient with pancreatic teratoma who underwent pylorus-preserving PPPD. The operation lasted approximately 6 hours. The mass was completely removed, and the patient recovered uneventfully. Complete surgical resection combined with outpatient follow-up is the primary choice for the management of PPTs.


Assuntos
Neoplasias Pancreáticas/patologia , Teratoma/patologia , Humanos , Lactente , Masculino , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia/métodos , Teratoma/diagnóstico , Teratoma/cirurgia
17.
Pan Afr Med J ; 34: 32, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31762900

RESUMO

Pancreatic insulinoma is a rare, often benign, neuroendocrine tumor which may give rise to life-threatening consequences due to hypoglycemia-related accidents. Adrenal deficiency can also cause hypoglycemia. We report the case of a 68-year old patient hospitalized for recurrent hypoglycaemia. Tests were performed that showed endogenous hyperinsulinism, adrenocorticotropin deficiency and hypergonadotropic hypogonadism. The patient received hydrocortisone without improvement. Five years later topography showed insulinoma. This study highlights the clinical, biological, radiological and therapeutic features of insulinoma as well as laboratory test results and shows that insulinoma can cause adrenocorticotropic deficiency and peripheral hypogonadism.


Assuntos
Insuficiência Adrenal/etiologia , Hipogonadismo/etiologia , Insulinoma/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Idoso , Humanos , Hidrocortisona/administração & dosagem , Hiperinsulinismo/etiologia , Hipoglicemia/etiologia , Insulinoma/complicações , Masculino , Neoplasias Pancreáticas/complicações
19.
Arkh Patol ; 81(5): 11-21, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31626200

RESUMO

An important role in the differentiation of tissues in different organs is played by transforming factors (TFs); pancreatic and duodenal homebox 1 (PDX-1) is one of the earliest factors for pancreatic cells. Many malignant tumors, including neuroendocrine tumors (NETs), are similar in structure, and therefore the actual problem of oncomorphology is to search for narrow-specific markers and TFs. AIM: to comparatively analyze and assess the value of the expression of the TF PDX-1 in NETs and non-NETs of different localization and histogenetic origin. MATERIAL AND METHODS: Anti-PDX-1 antibodies were used to study 528 tumors divided into 3 groups: Group 1 included 394 NETs, among them there were those of the pancreas (n=173), stomach (n=46), bowel (n=65), lung (n=40), thymus (n=8), kidney (n=6), Merkel's cell carcinomas (n=14), NETs of the breast (n=3), larynx (n=2), trachea (n=2), bladder (n=1), and metastatic NETs (n=34) of unknown primary site; Group 2 consisted of 16 tumors, of them there were paragangliomas (n=6), medullary thyroid cancers (MTC) (n=6) and adrenal pheochromocytomas (APCC) (n=4); Group 3 comprised 118 non-NETs, among them there were tumors of the pancreas (n=54), stomach (n=26), bowel (n=17), lung (n=11), breast (n=3), kidney (n=4), adrenal glands (n=2), and bladder (n=1). RESULTS: PDX-1 was positive in 75.1% (130/173) of pancreatic NETs, all insulinomas (50/50), gastrinomas (11/11), somatostatinomas (3/3), ACTH-producing tumors (2/2); PDX-1 was positive in the non-functioning pancreatic NETs, all PPomas (19/19), 76.1% (35/46) of NETs without the hormone detected, 50% (2/4) of calcitoninomas, and 21.1% (8/38) of silent glucagonomas. PDX-1 was positive in 32.4% (11/34) of carcinoids and 50% (6/12) of neuroendocrine carcinomas, all duodenal NETs (18/18), 90% (9/10) of rectal carcinoids and 30.8% (4/13) colonic carcinoids, 37.5% (3/8) of thymic/mediastinal carcinoids, 66.7% (4/6) of kidney carcinoids, and 37.5% (9/24) of metastatic NETs of unknown primary site. PDX-1 was negative in all carcinoids of the colon and sigmoid (0/5), ileum and jejunum (0/24), lung (0/40), trachea (0/2), larynx (0/2), Merkel's cell carcinoma (0/14), breast (0/3), bladder (0/1), as well as MTC (0/6), APCC (0/4), and paragangliomas (0/6). PDX-1-positive non-NETs included 81.8% (18/22) of adenocarcinomas (AC) and all serous cystic, mucinous cystic, intraductal and acinar cell tumors of the pancreas (4/4, 3/3, 2/2, and 3/3), 57.1% of AC (8/14) and 83.3% of signet ring cell carcinomas of the stomach (10/12), 56.2% AC of the bowel (9/17), bladder cancer (1/1). PDX-1 was negative in all anaplastic cancers (0/2) and solid pseudopapillary tumors of the pancreas (0/20), cancers of the lung (0/11), kidney (0/4), breast (0/3), and adrenal glands (0/2). CONCLUSION: The expression of PDX-1 is very specific for most digestive tract NETs and non-NETs. Pancreatic ductal and acinar cell tumors and gastric signet ring cell carcinomas are most commonly PDX-1-positive. Most tumors that do not originate from the digestive tract have a PDX-1 negative immunophenotype.


Assuntos
Proteínas de Homeodomínio/metabolismo , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Transativadores/metabolismo , Biomarcadores Tumorais/metabolismo , Humanos
20.
J Craniofac Surg ; 30(7): e674-e677, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31574790

RESUMO

INTRODUCTION: Von Hippel-Lindau (VHL) syndrome is a group of rare autosomal dominant hereditary diseases that involve multiple organs. Due to its high recurrence rate and complex and diverse clinical manifestations, VHL is prone to being either misdiagnosed or missed entirely. Therefore, patients with VHL syndrome have a poor prognosis. CLINICAL FEATURES: This study reports the details of 2 patients, a 55-year-old male and a 37-year-old female, who were diagnosed as having VHL syndrome with a positive family history. The male patient presented with upper abdominal discomfort 2 years prior to the current study, and was diagnosed in another hospital as having a space-occupying lesion at the head of the pancreas. After undergoing hemangioblastoma resection of the right cerebellar hemisphere 1 month ago, he was admitted to the hospital for recent aggravation of upper abdominal discomfort to receive further diagnosis and treatment. The female patient previously underwent right ovarian cystectomy and T5-T6 intramedullary hemangioblastoma resection. She was diagnosed at another hospital as having pancreatic cancer, and was admitted to the hospital for recent aggravation of upper abdominal discomfort to receive further diagnosis and treatment. CONCLUSION: The diagnosis and treatment of VHL syndrome is currently relatively difficult. It poses a substantial threat to patients and their families. The early and timely diagnosis and treatment of VHL syndrome can improve patients' prognosis and rates of survival.


Assuntos
Erros de Diagnóstico , Doença de von Hippel-Lindau/diagnóstico por imagem , Adulto , Feminino , Hemangioblastoma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/diagnóstico , Prognóstico
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