RESUMO
Chronic secretory diarrhea is a frequent entity. Causes are multiple, that is why reaching final diagnosis can result in delay with complications that this causes in the general condition of the patient. We present the case of one older adult with chronic diarrhea, mild hypokalemia and metabolic hyperchloremic acidosis secondary to a VIPoma in retroperitoneum which is an unusual location of this type of tumor that was diagnosed by biopsy guided by tomography.
Assuntos
Diarreia/etiologia , Neoplasias Retroperitoneais/diagnóstico , Vipoma/diagnóstico , Idoso , Biópsia , Doença Crônica , Humanos , Masculino , Radiografia Intervencionista , Neoplasias Retroperitoneais/complicações , Neoplasias Retroperitoneais/patologia , Tomografia Computadorizada por Raios X , Vipoma/complicações , Vipoma/patologiaRESUMO
Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.
Assuntos
Fístula Arteriovenosa/cirurgia , Hepatectomia/métodos , Artéria Hepática/anormalidades , Artéria Hepática/cirurgia , Veia Porta/anormalidades , Veia Porta/cirurgia , Fístula Arteriovenosa/diagnóstico , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/cirurgia , Criança , Transtornos da Nutrição Infantil/diagnóstico , Transtornos da Nutrição Infantil/terapia , Embolização Terapêutica/métodos , Varizes Esofágicas e Gástricas/cirurgia , Feminino , Hemorragia Gastrointestinal/cirurgia , Humanos , Hipertensão Portal/cirurgia , Ligadura/métodos , Resultado do Tratamento , Vipoma/diagnóstico , Vipoma/terapiaRESUMO
All pancreatic neuroendocrine tumors derive from Langerhans islet cells and have a low incidence. Half of them are functioning tumors that produce diverse hormones and occasionally cause serious clinical endocrine syndromes. They may be malignant, but they have a better survival, if compared to pancreatic ductal adenocarcinoma. Insulinoma, gastrinoma, glucagonoma, VIPoma (VIP=vasoactive intestinal peptide), somatostatinoma and ACTHoma are functioning tumors and they may also be part of Multiple Endocrine Neoplasia type I (MEN 1) syndrome and of von Hippel-Lindau disease. Diagnosis of non-functioning tumors is usually late, when they reach a big size and have even developed nodal and hepatic metastases. Nowadays, there are effective medical treatments for the medical problems secondary to excessive hormone production. For example, the hypergastrinemia typical of the Zollinger-Ellison syndrome in gastrinoma, can be adequately managed. Surgical resection is the most advisable therapy for pancreatic endocrine tumors, especially when they are small, when long time survival is better. Pre and intra operative imagenology is a great aid to locate these tumors. There are several surgical alternatives, according to the tumor size and location within the pancreas. Furthermore, palliative therapy can be used in disseminated disease. Treatment success is the result of a multidisciplinary medical team work of endocrinologists, surgeons, gastroenterologists, pathologists and geneticists.
Assuntos
Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Gastrinoma/diagnóstico , Gastrinoma/terapia , Humanos , Insulinoma/diagnóstico , Insulinoma/terapia , Masculino , Somatostatinoma/diagnóstico , Somatostatinoma/terapia , Vipoma/diagnóstico , Vipoma/terapiaRESUMO
Antecedentes. Los tumores endocrinos del páncreas son neoplasias derivadas de sus células neuroendocrinas o las situadas en la pared del duodeno. Objetivo. Describir las características clínicas, métodos diagnósticos y de tratamiento de los tumores endocrinos del páncreas, así como analizar la experiencia con estos tumores en el Instituto Nacional de la Nutrición ®Salvador Zubirán¼. Resultados. los tumores endocrinos del páncreas son neoplasias de crecimiento lento. Los más comunes son el insulinoma, el gastrinoma y los tumores no funcionales. El cuadro clínico de los tumores funcionales está ocasionado por el efecto de los péptidos secretados y el diagnóstico se establece al demostrar elevación de la hormona correspondiente. Para evaluar la localización de los tumores e investigar la presencia de metástasis, se han empleado diversos estudios de imagen con resultados poco satisfactorios en cuanto a localización. Recientemente se han empleado estudios dinámicos que permiten regionalizar la elevación hormonal y tanto el ultrasonido transendoscópico como transoperatorio parecen ser estudios promisorios para la localización del tumor. El tratamiento curativo consiste en la resección quirúrgica, e incluso la resección de metástasis hepáticas ha demostrado mejoría en la sobrevida. En el INNSZ se han administrado a 38 pacientes con tumores endocrinos del páncreas en un periodo de 32 años. El tumor más frecuente fue el insulinoma, seguido por los tumores no funcionales y el gastrinoma. La gran mayoría de los insulinomas fueron benignos, no así el resto de los tumores. Conclusiones. Los tumores endocrinos del páncreas son lesiones poco frecuentes, con cuadros clínicos muy variados en relación a la hormona que producen, son difíciles de localizar antes de la cirugía y se caracterizan por cursar con una evolución muy favorable a juzgar por la alta frecuencia de curación de las lesiones benignas y la supervivencia prolongada de los pacientes con tumores malignos
Assuntos
Humanos , Intervalo Livre de Doença , Gastrinoma/diagnóstico , Glucagonoma/diagnóstico , Insulinoma/diagnóstico , Insulinoma/fisiopatologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/fisiopatologia , Vipoma/diagnósticoRESUMO
BACKGROUND: Endocrine tumors of the pancreas arise from pancreatic and duodenal neuroendocrine cells. OBJECTIVE: To review clinical features, diagnosis and treatment of endocrine tumors of pancreas, as well as our experience at the Instituto Nacional de la Nutrición "Salvador Zubirán" (INNSZ). RESULTS: Endocrine tumors of pancreas are slow growing tumors. The most frequent tumors are insulinoma, gastrinoma and non-functioning tumors. Clinical picture of functioning tumors is determined by the action of the secreting hormone, and the diagnosis is established once high levels of the tumor secretin peptide are demonstrated. In order to localize the tumor and to search for metastasis, several imaging studies have been used. In general, image studies have demonstrated poor sensitivity in finding the primary lesion. Recently, dynamic studies have shown their ability to regionalize the peptide increase. Endoscopic and intraoperative ultrasound have shown promising results. Surgical resection is the only curative treatment, resection of hepatic metastasis can even improve survival. In the INNSZ, 38 patients with endocrine tumors of the pancreas have received initial treatment in a 32-year period. The most common neoplasia is the insulinoma, followed by non-functioning tumors and gastrinoma. Most insulinomas were benign, where as the malignancy rate of non-functioning. CONCLUSIONS: Endocrine tumors of the pancreas are rare lesions, with a variety of clinical pictures related to the hormone produced. Localizing studies are generally poor. Favorable outcome meaning cure of benign lesions or long survival despite the presence of malignancy occur in most patients.