RESUMO
Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.
Assuntos
Neoplasias Pancreáticas , Humanos , Feminino , Masculino , Brasil , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/diagnóstico , Estudos Retrospectivos , Pancreatectomia/métodos , Pâncreas/patologiaRESUMO
OBJECTIVE: To analyze abdominal drain on the first postoperative day and evaluate its predictive nature for the diagnosis of Pancreatic Fistula exclusion, seeking to establish a cutoff point from which lower values demonstrate safety in excluding the possibility of this complication. METHODS: From August 2017 to June 2020, data from 48 patients undergoing pancreatic resection were collected and analyzed from a prospective cohort. The patients were divided into two groups, one group consisting of patients who did not develop PF (Group A), and the other composed of patients who developed PF (Group B). The receiver operation characteristic curve was constructed, and cutoff points were evaluated by calculating sensitivity and specificity. RESULTS: Group A brought 30 patients together (62.5%) and Group B brought 18 patients together (37.5%). The 444 U/L value was the most satisfactory cutoff point for the receiver operation characteristic curve (CI 0.690-0.941), with a sensitivity of 94.4% and a specificity of 60%, thus being able to select 18 of 30 patients who did not succumb to PF. CONCLUSIONS: Abdominal drain on the first postoperative day can be used as a predictive factor in the diagnosis of PF exclusion (CI 0.690-0.941), with the value of 444 U/L being the best performance cutoff point.
Assuntos
Pancreatectomia , Fístula Pancreática , Amilases , Drenagem , Humanos , Pancreatectomia/efeitos adversos , Fístula Pancreática/diagnóstico , Fístula Pancreática/etiologia , Pancreaticoduodenectomia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Valor Preditivo dos Testes , Estudos ProspectivosRESUMO
SUMMARY OBJECTIVE: To analyze abdominal drain on the first postoperative day and evaluate its predictive nature for the diagnosis of Pancreatic Fistula exclusion, seeking to establish a cutoff point from which lower values demonstrate safety in excluding the possibility of this complication. METHODS: From August 2017 to June 2020, data from 48 patients undergoing pancreatic resection were collected and analyzed from a prospective cohort. The patients were divided into two groups, one group consisting of patients who did not develop PF (Group A), and the other composed of patients who developed PF (Group B). The receiver operation characteristic curve was constructed, and cutoff points were evaluated by calculating sensitivity and specificity. RESULTS: Group A brought 30 patients together (62.5%) and Group B brought 18 patients together (37.5%). The 444 U/L value was the most satisfactory cutoff point for the receiver operation characteristic curve (CI 0.690-0.941), with a sensitivity of 94.4% and a specificity of 60%, thus being able to select 18 of 30 patients who did not succumb to PF. CONCLUSIONS: Abdominal drain on the first postoperative day can be used as a predictive factor in the diagnosis of PF exclusion (CI 0.690-0.941), with the value of 444 U/L being the best performance cutoff point.
Assuntos
Pancreatectomia/efeitos adversos , Fístula Pancreática/diagnóstico , Fístula Pancreática/etiologia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Drenagem , Valor Preditivo dos Testes , Estudos Prospectivos , Pancreaticoduodenectomia , AmilasesRESUMO
OBJECTIVE: To create a structured computed tomography (CT) report for the systematic evaluation of pancreatic ductal adenocarcinoma (PDAC), based on the opinions of clinicians and surgeons. MATERIALS AND METHODS: This was a prospective study in which we applied a 21-item questionnaire to experts in pancreatic diseases in order to create a model of a structured abdominal CT report. The questionnaire addressed the location and size of PDACs, as well as their effects on adjacent structures and on the vasculature, together with metastases. We used a Likert scale to determine which of those parameters should be included in the model. RESULTS: A total of 18 experts (12 surgeons and 6 clinicians) from 9 institutions completed the questionnaire. All of the experts agreed that the following (if present) should be described in the CT report on a PDAC: the degree of enhancement; the diameter and location of the lesion; pancreatic duct obstruction; biliary dilatation; pancreatic atrophy; liver metastases; peritoneal nodules; ascites; lymph node enlargement; and invasion of adjacent structures. More than 80% of the experts agreed that the report should also describe the relationship between the PDAC and the surrounding vasculature. CONCLUSION: We have developed a template for a CT report on patients with PDAC, based on the opinions of experts involved in the treatment of such patients.
OBJETIVO: Criar um modelo de laudo estruturado de tomografia computadorizada (TC) para a avaliação sistemática do adenocarcinoma ductal de pâncreas (ADP) com base na opinião de clínicos e cirurgiões. MATERIAIS E MÉTODOS: Realizamos estudo prospectivo aplicando um questionário abrangendo 21 tópicos para um modelo de laudo estruturado de TC do abdome na avaliação do ADP, contemplando dados sobre localização e tamanho tumoral, efeito sobre estruturas adjacentes, comprometimento vascular e presença de metástases. Utilizamos a escala de Likert para definir a pertinência de se inserir uma determinada informação. RESULTADOS: Obtivemos respostas de 18 especialistas de 9 instituições (12 cirurgiões e 6 clínicos). Todos concordaram que deveriam ser descritos o grau de realce, o diâmetro da lesão, a localização e a presença ou a ausência de: obstrução do ducto pancreático, dilatação biliar, atrofia pancreática, metástases hepáticas, nódulos peritoniais, ascite, linfonodomegalias e invasão de estruturas adjacentes. Mais de 80% concordaram que deveria ser mencionada a relação do tumor com os vasos circunjacentes. CONCLUSÃO: Elaboramos um modelo de laudo estruturado de TC para a avaliação do ADP de pâncreas, atendendo às expectativas dos especialistas envolvidos no atendimento destes pacientes.
RESUMO
Abstract Objective: To create a structured computed tomography (CT) report for the systematic evaluation of pancreatic ductal adenocarcinoma (PDAC), based on the opinions of clinicians and surgeons. Materials and Methods: This was a prospective study in which we applied a 21-item questionnaire to experts in pancreatic diseases in order to create a model of a structured abdominal CT report. The questionnaire addressed the location and size of PDACs, as well as their effects on adjacent structures and on the vasculature, together with metastases. We used a Likert scale to determine which of those parameters should be included in the model. Results: A total of 18 experts (12 surgeons and 6 clinicians) from 9 institutions completed the questionnaire. All of the experts agreed that the following (if present) should be described in the CT report on a PDAC: the degree of enhancement; the diameter and location of the lesion; pancreatic duct obstruction; biliary dilatation; pancreatic atrophy; liver metastases; peritoneal nodules; ascites; lymph node enlargement; and invasion of adjacent structures. More than 80% of the experts agreed that the report should also describe the relationship between the PDAC and the surrounding vasculature. Conclusion: We have developed a template for a CT report on patients with PDAC, based on the opinions of experts involved in the treatment of such patients.
Resumo Objetivo: Criar um modelo de laudo estruturado de tomografia computadorizada (TC) para a avaliação sistemática do adenocarcinoma ductal de pâncreas (ADP) com base na opinião de clínicos e cirurgiões. Materiais e Métodos: Realizamos estudo prospectivo aplicando um questionário abrangendo 21 tópicos para um modelo de laudo estruturado de TC do abdome na avaliação do ADP, contemplando dados sobre localização e tamanho tumoral, efeito sobre estruturas adjacentes, comprometimento vascular e presença de metástases. Utilizamos a escala de Likert para definir a pertinência de se inserir uma determinada informação. Resultados: Obtivemos respostas de 18 especialistas de 9 instituições (12 cirurgiões e 6 clínicos). Todos concordaram que deveriam ser descritos o grau de realce, o diâmetro da lesão, a localização e a presença ou a ausência de: obstrução do ducto pancreático, dilatação biliar, atrofia pancreática, metástases hepáticas, nódulos peritoniais, ascite, linfonodomegalias e invasão de estruturas adjacentes. Mais de 80% concordaram que deveria ser mencionada a relação do tumor com os vasos circunjacentes. Conclusão: Elaboramos um modelo de laudo estruturado de TC para a avaliação do ADP de pâncreas, atendendo às expectativas dos especialistas envolvidos no atendimento destes pacientes.
RESUMO
BACKGROUND: The solid pseudopapillary neoplasm is a rare tumor of the pancreas. However, it´s etiology still maintain discussions. AIM: To analyze it´s clinical data, diagnosis and treatment. METHODS: A retrospective study of medical records of all patients treated from January 1997 until July 2015. RESULTS: Were identified 17 cases. Most patients were women (94.11%) and the average age was 32.88 years. The main complaint was abdominal mass (47.05%). The most frequent location was in the body/tail of the pancreas (72.22%) and the most frequently performed surgery was distal pancreatectomy with splenectomy (64.70%). No patient had metastases at diagnosis. Conservative surgery for pancreatic parenchyma was performed in only three cases. The rate of complications in the postoperative period was 35.29% and the main complication was pancreatic fistula (29.41%). No patient underwent adjuvant treatment. CONCLUSIONS: The treatment is surgical and the most common clinical presentation is abdominal mass. Distal pancreatectomy with splenectomy was the most frequently performed surgery for its treatment.
RACIONAL: A neoplasia sólida pseudopapilar é tumor raro de pâncreas de tratamento cirúrgico. No entanto, sua causa ainda gera discussões. OBJETIVO: Analisar os dados clínicos, do diagnóstico e do tratamento da dessa neoplasia. MÉTODOS: Estudo retrospectivo com dados médicos de pacientes tratados entre janeiro de 1997 a julho de 2015. RESULTADOS: Foram identificados 17 casos. A maioria era de mulheres (94,11%) e a média de idade foi de 32,88 anos. A principal queixa era massa abdominal (47,05%). A localização mais frequente era no corpo/cauda do pâncreas (72,22%) e a operação mais realizada foi a pancreatectomia corpocaudal com esplenectomia (64,70%). Nenhum caso apresentou metástase no momento do diagnóstico. Operação conservadora de parênquima pancreático foi realizada em apenas três casos. A taxa de complicações no pós-operatório foi de 35,29% e a principal complicação foi fístula pancreática (29,41%). Nenhum paciente realizou adjuvância no seguimento. CONCLUSÕES: A mais comum apresentação clínica da neoplasia sólida pseudopapilar é de massa abdominal. Ela é de tratamento cirúrgico e pancreatectomia corpocaudal com esplenectomia é o procedimento mais realizado para seu tratamento.
Assuntos
Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Adolescente , Adulto , Feminino , Humanos , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
ABSTRACT Background: The solid pseudopapillary neoplasm is a rare tumor of the pancreas. However, it´s etiology still maintain discussions. Aim: To analyze it´s clinical data, diagnosis and treatment. Methods: A retrospective study of medical records of all patients treated from January 1997 until July 2015. Results: Were identified 17 cases. Most patients were women (94.11%) and the average age was 32.88 years. The main complaint was abdominal mass (47.05%). The most frequent location was in the body/tail of the pancreas (72.22%) and the most frequently performed surgery was distal pancreatectomy with splenectomy (64.70%). No patient had metastases at diagnosis. Conservative surgery for pancreatic parenchyma was performed in only three cases. The rate of complications in the postoperative period was 35.29% and the main complication was pancreatic fistula (29.41%). No patient underwent adjuvant treatment. Conclusions: The treatment is surgical and the most common clinical presentation is abdominal mass. Distal pancreatectomy with splenectomy was the most frequently performed surgery for its treatment.
RESUMO Racional: A neoplasia sólida pseudopapilar é tumor raro de pâncreas de tratamento cirúrgico. No entanto, sua causa ainda gera discussões. Objetivo: Analisar os dados clínicos, do diagnóstico e do tratamento da dessa neoplasia. Métodos: Estudo retrospectivo com dados médicos de pacientes tratados entre janeiro de 1997 a julho de 2015. Resultados: Foram identificados 17 casos. A maioria era de mulheres (94,11%) e a média de idade foi de 32,88 anos. A principal queixa era massa abdominal (47,05%). A localização mais frequente era no corpo/cauda do pâncreas (72,22%) e a operação mais realizada foi a pancreatectomia corpocaudal com esplenectomia (64,70%). Nenhum caso apresentou metástase no momento do diagnóstico. Operação conservadora de parênquima pancreático foi realizada em apenas três casos. A taxa de complicações no pós-operatório foi de 35,29% e a principal complicação foi fístula pancreática (29,41%). Nenhum paciente realizou adjuvância no seguimento. Conclusões: A mais comum apresentação clínica da neoplasia sólida pseudopapilar é de massa abdominal. Ela é de tratamento cirúrgico e pancreatectomia corpocaudal com esplenectomia é o procedimento mais realizado para seu tratamento.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Adulto Jovem , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/diagnóstico , Estudos RetrospectivosRESUMO
PURPOSE: To test the use of diffusion-weighted magnetic resonance imaging (DW-MRI) to differentiate between different degrees of severity of acute pancreatitis (AP). METHOD: Thirty-six patients who underwent DW-MRI and magnetic resonance cholangiopancreatography were divided into patients with mild AP (mAP, n = 15), patients with necrotizing AP (nAP, n = 8), and patients with a normal pancreas (nP, n = 15; controls). The pancreas was divided into head, body, and tail, and each segment was classified according to image features: pattern 1, normal; pattern 2, mild inflammation; and pattern 3, necrosis. Apparent diffusion coefficients (ADCs) were measured in each segment and correlated with clinical diagnoses. RESULTS: A total of 108 segments was assessed (three segments per patient). Segments classified as pattern 1 in the nP and mAP groups showed similar ADC values (P = 0.29). ADC values calculated for the pancreatic segments grouped according to the different image patterns (1-3) were significantly different (P < 0.001). Comparisons revealed significant differences in signal intensity between all three patterns (P < 0.05). CONCLUSIONS: DW-MRI was a compatible and safe image option to differentiate tissue image patterns in patients with mAP, nAP, and nP, mainly in those with contraindications to contrast-enhanced MRI (which is classically required for determining the presence of necrosis) or computed tomography. ADC measures allowed precise differentiation between patterns 1, 2, and 3.