Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort.

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.

Ethyl-pyruvate reduces lung injury matrix metalloproteinases and cytokines and improves survival in experimental model of severe acute pancreatitis

PURPOSE: To investigate if the ethyl-pyruvate solution could reduce mortality in AP and/or diminish the acute lung injury. METHODS: Forty male rats, weighing between 270 to 330 grams were operated. An experimental model of severe AP by injection of 0.1ml/100g of 2.5% sodium taurocholate into the bilio-pancreatic duct was utilized. The rats were divided into two groups of ten animals each: CT - control (treatment with 50ml/kg of Ringer's solution, intraperitoneal) and EP (treatment with 50ml/kg of Ringer ethyl- pyruvate solution, intra-peritoneal), three hours following AP induction. After six hours, a new infusion of the treatment solution was performed in each group. Two hours later, the animals were killed and the pulmonary parenchyma was resected for biomolecular analysis, consisting of: interleukin, myeloperoxidase, MDA, nitric oxide, metalloproteinases and heat shock protein. In the second part of the experiment, another, 20 rats were randomly divided into EP and CT groups, in order to evaluate a survival comparison between the two groups. RESULTS: There were no significant differences in IL-1B,IL-10, MMP-9, HSP70, nitric oxide, MPO, MDA (lipidic peroxidation) concerning both groups. The levels of IL-6 were significantly diminished in the EP group. Furthermore, the MMP-2 levels were also reduced in the EP group (p<0.05). The animals from the EP treatment groups had improved survival, when compared to control group (p<0.05). CONCLUSION: The ethyl-pyruvate diminishes acute lung injury inflammatory response in acute pancreatitis and ameliorates survival when compared to control group, in the experimental model of necrotizing acute pancreatitis.

Resultados do tratamento da pancreatite aguda grave / Severe acute pancreatitis: results of treatment

OBJETIVO: Avaliar os resultados do Protocolo de Atendimento de pacientes com diagnóstico de pancreatite aguda grave. MÉTODOS: Foram analisados, consecutivamente, a partir de janeiro de 2002, idade, sexo, etiologia, tempo de internação, tipo de tratamento e mortalidade de 37 pacientes portadores de pancreatite aguda grave. RESULTADOS: A idade dos pacientes variou de 20 a 88 anos (média de 50 anos); 27% foram do sexo feminino e 73% do masculino. O tempo médio global de internação foi 47 dias. Treze pacientes foram tratados cirurgicamente; a média de operações realizadas foi duas por paciente. Ocorreram seis óbitos dentre os pacientes submetidos ao tratamento cirúrgico (46%) e dois óbitos no grupo submetido somente ao tratamento clínico (8,3%). A mortalidade global foi 21% CONCLUSÃO: Após a modificação na forma de abordagem dos pacientes com pancreatite aguda grave, houve diminuição da mortalidade e uma tendência para a conduta expectante.

OBJECTIVE: To evaluate the results of the Protocol for treatment of patients with severe acute pancreatitis. METHODS: We consecutively analyzed age, gender, etiology, length of hospital stay, type of treatment and mortality of 37 patients with severe acute pancreatitis from January 2002. RESULTS: The patients' ages ranged from 20 to 88 years (average 50 years), 27% were female and 73% male. Mean overall hospital stay was 47 days. Thirteen patients were treated surgically, the average operations per patient was two. There were six deaths among patients undergoing surgical treatment (46%) and two deaths in the group submitted to medical treatment alone (8.3%). The overall mortality was 21%. CONCLUSION: After modification in the form of management of patients with severe acute pancreatitis, there was a decrease in mortality and a trend for conservative management.

Castleman's disease mimetizing pancreatic tumor.

CONTEXT: Angiofollicular lymph node hyperplasia or Castleman's disease is a rare clinical condition. Knowledge about etiology and physiopathology; and treatment management as well are yet to be defined. Unicentric presentation of this disease affecting single lymph nodes in the mediastinum seems to be the most common presentation. Castleman's disease localized in the pancreas topographic area that mimics a pancreatic neoplasm is an even more uncommon event, with available published data of less than 15 cases until now. CASE REPORT: We present a 64-year-old male patient with a six-month past history of asthenia, adynamia, and lack of general clinical conditions. Imaging studies showed a nodular hypoechoic mass in the pancreatic head. Enucleation of the lesion was performed. Histopathological study revealed unicentric form of Castleman's Disease. CONCLUSIONS: Castleman's disease mimetizing pancreatic tumor is uncommon and it also curses with a difficult preoperative diagnosis. Surgery seems to be the best therapeutic alternative for this disease.

Expressão das proteínas p53, p16 e ki67 no adenocarcinoma da cabeça do pâncreas e sua relação com a sobrevida e diferenciação celular / Expression of p53, p16 and Ki67 proteins in ductal adenocarcinoma of the pancreatic head and their relation with survival and cell differentiation

Objective: To determine the expression of p53, p16 and Ki-67 and its relevance in survival and cell differentiation. Methods: Fifteen duodenopancreatectomized patients were included. Immunohistochemical expression of p53, p16 and Ki-67 was determined in paraffin embedded tumor blocks. The relation of these expressions with different variables was studied. Results: Ninetythree per cent of tumors showed expression of p53 and p16. Ki- 67 was expressed in 86.66% of tumors (labeling index plus or minus LI 11.91 ± 9.47). The presence of combined alterations was not related to significant differences in tumor type, stage or survival; similar results were obtained analyzing isolated expressions. When groups of p16 and Ki-67 expressions where created, the median survival was not significant. However, there was a slightly better survival in patientswith focal expression of p16 (median survival 20.75 versus 14.34), when compared to patients with diffuse expression. Conclusion: The overexpression of p53, p16 and Ki-67 was not related to survival or tumor grade, when comparing isolated or combined expressions.

Objetivo: Determinar a expressão de p53, p16 e Ki-67 e sua relevância na sobrevida e diferenciação celular. Métodos: Foram incluídos 15 pacientes submetidos a duodenopancreatectomia. A expressão imunohistoquímica de p53, p16 e Ki-67 foi determinada em blocos tumorais embebidos em parafina. Foi estudada a relação dessas expressões com as variáveis. Resultados: Noventa e três por cento dos tumores apresentaram expressão de p53 e p16. Ki-67 estava expresso em 86,66% dos tumores (índice proliferativo mais ou menos IP 11,91 ± 9,47). A presença de alterações combinadas não estava relacionada a diferenças significativas no tipo tumoral, no estágio ou na sobrevida; resultados semelhantes foram obtidos com a análise de expressões isoladas. Quando foram criados os grupos de expressões de p16 e Ki-67, a sobrevida mediana não era significativa. Entretanto, havia uma sobrevida discretamente melhor nos pacientes com expressão focal do p16 (sobrevida mediana 20,75 versus 14,34) em comparação com pacientes com expressão difusa. Conclusão: A superexpressão das proteínas p53, p16 e Ki-67 não estava relacionada à sobrevida ou ao grau tumoral quando se compararam as expressões isoladas ou combinadas.

Expression of p53, p16 and Ki67 proteins in ductal adenocarcinoma of the pancreatic head and their relation with survival and cell differentiation.

OBJECTIVE: To determine the expression of p53, p16 and Ki-67 and its relevance in survival and cell differentiation. METHODS: Fifteen duodenopancreatectomized patients were included. Immunohistochemical expression of p53, p16 and Ki-67 was determined in paraffin embedded tumor blocks. The relation of these expressions with different variables was studied. RESULTS: Ninety-three per cent of tumors showed expression of p53 and p16. Ki-67 was expressed in 86.66% of tumors (labeling index - LI 11.91 ± 9.47). The presence of combined alterations was not related to significant differences in tumor type, stage or survival; similar results were obtained analyzing isolated expressions. When groups of p16 and Ki-67 expressions where created, the median survival was not significant. However, there was a slightly better survival in patients with focal expression of p16 (median survival 20.75 versus 14.34), when compared to patients with diffuse expression. CONCLUSION: The overexpression of p53, p16 and Ki-67 was not related to survival or tumor grade, when comparing isolated or combined expressions.

Acidose metabólica por hiperglicemia transitória pós enucleação de insulinoma pancreático / Metabolic acidosis secondary to transient hyperglycemia after pancreatic insulinoma enucleation

INTRODUÇÃO: Insulinoma é a neoplasia endócrina mais frequente dos tumores funcionantes do pâncreas. Origina-se a partir das células beta das ilhotas de Langerhans e caracteriza-se pela produção excessiva de insulina, com consequente hipoglicemia. O tratamento de escolha é a remoção cirúrgica da neoplasia. O presente relato tem como objetivo apresentar uma complicação metabólica pouco observada.RELATO DO CASO: Homem de 41 anos de idade há dois anos com tonturas, visão turva e convulsões. Os sintomas estavam bem relacionados com períodos prolongados de jejum e melhoravam com as refeições, e durante um dos episódios foi constatada a presença de hipoglicemia, melhorando os sintomas imediatamente após administração de glicose endovenosa. A pesquisa glicêmica revelou intensa hipoglicemia. Ultrassonografia, tomografia computadorizada e ressonância magnética de abdome não revelaram nenhum tipo de alterações no pâncreas. Com a hipótese diagnóstica de hiperglicemia orgânica por provável insulinoma, o paciente foi submetido à enucleação da lesão. No 5º dia do pós-operatório surgiu fístula pancreática e acidose metabólica com resolução satisfatória. O laudo histopatológico mostrou tumor endócrino de pâncreas de 1,5 cm.CONCLUSÃO: Todo paciente submetido à ressecção de insulinoma pancreático deve realizar o pós-operatório imediato em unidades de terapia intensiva, monitorando de forma rigorosa os níveis de glicemia como prevenção de acidose metabólica.

INTRODUCTION: The insulinoma is the most frequent endocrine neoplasm among the functional pancreatic tumors. It originates in the beta cells of the islets of Langerhans and is characterized by the oversecretion of insulin, leading to hypoglycemia. The treatment of choice is the surgical excision of the tumor. The aim of the present report is to describe a rarely observed metabolic complication.CASE REPORT: The case is presented of a 41-year-old man with a 2-year history of dizziness, blurred vision and seizures. The symptoms were closely related to prolonged fasting and improved with eating; hypoglycemia was found during one of the episodes. Symptoms were relieved immediately after intravenous administration of glucose. Blood glucose workup showed severe hypoglycemia. Abdominal ultrasonography, computed tomography and magnetic resonance imaging did not show any alteration in the pancreas. With the diagnostic hypothesis of organic hypoglycemia from a likely insulinoma, the patient underwent the enucleation of the lesion. On the 5th postoperative day, a pancreatic fistula appeared, as well as metabolic acidosis which resolved satisfactorily. The histopathological report showed a 1.5-cm endocrine pancreatic tumor.CONCLUSION: Every patient submitted to pancreatic insulinoma resection should stay in an intensive care unit during the immediate postoperative period and their glycemic levels must be monitored closely to prevent metabolic acidosis.

Insulinomas do pâncreas: diagnóstico e tratamento / Pancreatic insulinomas: diagnosis and treatment

RACIONAL: O insulinoma é uma neoplasia que pertence ao grupo de tumores neuroendócrinos do pâncreas; embora raro, é o mais freqüente entre eles. OBJETIVO: Avaliar os resultados clínico e imunohistoquímico de doentes com insulinoma de pâncreas submetidos a tratamento cirúrgico. PACIENTES E MÉTODOS: Foram detalhados o quadro clínico, exames laboratoriais e de imagem, dando ênfase aos aspectos cirúrgicos, complicações e acompanhamento, a médio e longo prazos, de 12 doentes portadores de insulinoma. A cirurgia foi indicada para todos, com base nas evidências clínicas e laboratoriais de hipoglicemia e hiperinsulinemia. RESULTADOS: Em sete doentes conseguiu-se a localizaçäo prévia da lesäo; em cinco, sua localizaçäo foi identificada durante a cirurgia mediante palpaçäo e através do ultra-som intra-operatório. O procedimento mais freqüentemente utilizado foi a ressecçäo pancreática em oito doentes. A complicaçäo cirúrgica mais freqüente foi a fístula pancreática, observada em sete doentes. Dois apresentaram tardiamente diabetes mellitus. Näo houve mortes na presente casuística. CONCLUSÄO: Na totalidade dos doentes obteve-se a reversäo clínica dos sintomas, caracterizada pelo desaparecimento da tríade de Whipple, além de normalizaçäo ou aumento dos níveis de glicemia, quando comparados aos valores do pré-operatório

[Pancreatic insulinomas: diagnosis and treatment]. / Insulinomas do pâncreas: diagnóstico e tratamento.

BACKGROUND: Despite its rarity, the insulinoma is the most common pancreatic neuroendocrine tumor. OBJECTIVE: Analyze clinical and immunohistochemical data from surgical resection of the pancreas insulinoma. METHOD: Twelve cases are described, concerning surgical aspects, complications and medium-long term outcome of patients. They underwent surgical treatment due to clinical suspicion and biochemical diagnosis of hypoglycemia and hyperinsulinism. RESULTS: The insulinoma was identified preoperatively in seven patients, while intraoperative ultrasonography and palpation were necessary for diagnosis in the other cases. Eight patients underwent pancreatic resection and pancreatic leak was observed in seven cases. Two patients developed diabetes mellitus and no mortality occurred in the current series. CONCLUSION: All patients presented satisfactory outcome and remained asymptomatic with normal glicemia levels.