Divertículo de Meckel con tumor neuroendocrino: presentación inusual / Meckel's diverticulum with neuroendocrine tumor: unusual presentation

El divertículo de Meckel es una malformación congénita que suele presentarse como un hallazgo incidental asintomático. Puede complicarse por procesos inflamatorios o tumores, cursando con sintomatología abdominal sumamente inespecífica, lo que complica su diagnóstico oportuno. Aunque la incidencia de neoplasias malignas en estos divertículos es baja, los tumores neuroendocrinos son los más representativos. Presentamos el caso de una paciente de 72 años que consultó por dolor abdominal y deposiciones melénicas, con múltiples nódulos intrahepáticos sugestivos de tumores neuroendocrinos y hallazgo intraoperatorio incidental de diverticulitis aguda de Meckel con metástasis peridiverticular de un tumor neuroendocrino. (AU)

Meckel's diverticulum is a congenital malformation that usually presents as an incidental finding. It can be complicated by inflammatory processes or tumors, with non-specific abdominal symptoms which delay its timely diagnosis. Although the incidence of malignant neoplasms in these diver-ticula is low, neuroendocrine tumors are the most representative. We present the case of a 72-year-old female patient who consulted for abdominal pain and melenic bowel movements, with multiple intrahepatic nodules suggestive of neuroendocrine tumors and an incidental intraoperative finding of acute Meckel's diverticulitis with peridiverticular metastasis of a neuroendocrine tumor. (AU)

Tumores Neuroendocrinos: un desafío transversal / Update on neuroendocrine tumors

Neuroendocrine Tumors (NETs) encompass a wide variety of tumors arising from neuroendocrine cells, which produce bioactive substances. The incidence of NETs increased significantly lately, becoming one of the most common tumors of the digestive tract. Their clinical presentation is as diverse as their capacity for hormone production. Carcinoid syndrome is the most common hormonal syndrome produced by NETs and is characterized by diarrhea, flushing and cardiac valvular lesions. New research brought multiple changes in the classification of these neoplasms and a new understanding about their diagnosis and treatment, promoting a multidisciplinary approach. Somatostatin analogues, radiation, biological, and cytotoxic drugs have improved the prognosis of these patients, which entails a great challenge for healthcare providers.

Tumor neuroendocrino Tipo 1: reporte de un caso y revisión de la literatura / Localized neuroendocrine tumor of the stomach: a case report and literature review

Resumen Introducción: Los tumores neuroendocrinos (TNE), son tumores compuestos por células productoras de péptidos y aminas. Los TNE gástricos, representan el 1% de todas las neoplasias, sin embargo su incidencia ha ido en aumento. Son generalmente asintomáticos y no funcionantes. El tratamiento es generalmente la resección local. Caso Clínico: paciente de 48 años con sospecha de cáncer gástrico; su estudio demuestra un TNE gástrico bien diferenciado tipo 1. Se realiza etapificación y se define en comité oncológico la vigilancia endoscópica. El hallazgo de un TNE, en el estudio de cáncer gástrico, es un hallazgo poco frecuente. Debido al aumento progresivo en la realización de endoscopías digestivas altas, secundario a la alta prevalencia de cáncer gástrico en nuestro país, se espera que aumenten hallazgos como un TNE. Es por esto que realizamos una revisión de la literatura y planteamos algunas conclusiones al respecto.

Introduction: Neuroendocrine tumors (NETs) are composed of cells that produce peptides and amines. Gastric NETs represent 1% of all neoplasms; however their incidence has been increasing. They are usually asymptomatic and non-functioning. The treatment is usually local resection. Case Report: We present the case of a 48-year-old patient who was suspected of gastric cancer; her study shows a well-differentiated type 1 gastric NET. Staging is performed and endoscopic surveillance is defined in the oncology board. The finding of a NET, in the study of gastric cancer, is a rare finding. Due to the progressive increase in the performance of upper gastrointestinal endoscopies, secondary to the high prevalence of gastric cancer in our country, it is expected to increase findings as a NET. That is why we conducted a review of the literature and made some conclusions about it.

Neuroendocrine tumor in a child with common variable immunodeficiency / Tumor neuroendócrino em criança com imunodeficiência comum variável

ABSTRACT Objective: To report a case of a child with primary immunodeficiency who at eight years developed digestive symptoms, culminating with the diagnosis of a neuroendocrine tumor at ten years of age. Case description: One-year-old boy began to present recurrent pneumonias in different pulmonary lobes. At four years of age, an immunological investigation showed a decrease in IgG and IgA serum levels. After the exclusion of other causes of hypogammaglobinemia, he was diagnosed with a Common Variable Immunodeficiency and started to receive monthly replacement of human immunoglobulin. The patient evolved well, but at 8 years of age began with epigastrium pain and, at 10 years, chronic persistent diarrhea and weight loss. After investigation, a neuroendocrine tumor was diagnosed, which had a rapid progressive evolution to death. Comments: Medical literature has highlighted the presence of gastric tumors in adults with Common Variable Immunodeficiency, emphasizing the importance of early diagnosis and the investigation of digestive neoplasms. Up to now there is no description of neuroendocrine tumor in pediatric patients with Common Variable Immunodeficiency. We believe that the hypothesis of digestive neoplasm is important in children with Common Variable Immunodeficiency and with clinical manifestations similar to the case described here in the attempt to improve the prognosis for pediatric patients.

RESUMO Objetivo: Relatar um caso de criança portadora de imunodeficiência primária que, aos oito anos, desenvolveu sintomas digestivos, culminando com o diagnóstico de tumor neuroendócrino aos dez anos de idade. Descrição do caso: Menino, com um ano de idade, começou a apresentar pneumonias de repetição em diferentes lobos pulmonares. Aos quatro anos, a investigação imunológica mostrou diminuição dos níveis séricos de IgG e IgA. Após exclusão de outras causas de hipogamaglobulinemia, teve diagnóstico de imunodeficiência comum variável, passando a receber reposição mensal de imunoglobulina humana. Evoluiu bem, porém, aos oito anos, começou com epigastralgia e, aos dez anos, diarreia crônica persistente e perda de peso. O quadro culminou com o diagnóstico de tumor neuroendócrino intestinal, de rápida progressão, com óbito do paciente. Comentários: A literatura tem chamado a atenção para tumores gástricos em adultos com imunodeficiência comum variável, alertando para a importância do diagnóstico precoce e da pesquisa de neoplasias digestivas. Até o momento, não há descrição de tumor neuroendócrino em pacientes pediátricos portadores de imunodeficiência comum variável. Acredita-se ser importante a hipótese de neoplasia digestiva diante de crianças com imunodeficiência comum variável e com manifestações clínicas semelhantes ao caso descrito, na tentativa de melhorar o prognóstico para pacientes pediátricos.

The rare case of a cystic pancreatic neuroendocrine tumor

The pancreatic neuroendocrine tumors (PanNETs) most commonly present as solid neoplasms; however, very rarely, they may present primarily as cystic neoplasms. Most of the cystic PanNETs are non-secreting tumors, and the radiological features are not well defined. Hence pre-operative diagnosis is usually challenging and the tumors are misdiagnosed as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, serous cystic neoplasms, solid pseudopapillary neoplasms, and non-neoplastic cysts. However, the management depends on the accurate diagnosis of these cystic lesions, which poses a dilemma. Herein, we report the case of a cystic PanNET in the tail of the pancreas, which was clinically and radiologically misdiagnosed as a mucinous cystic neoplasm. This case is reported to highlight this issue to the medical community regarding the diagnostic difficulty in such rare non-functioning pancreatic neuroendocrine tumors.

Rectal carcinoid tumor: diagnosis and management / Tumor carcinoide de reto: diagnóstico e orientação

ABSTRACT The authors present a case of rectal carcinoid tumor in an asymptomatic patient who sought a coloproctology service with the purpose of colorectal cancer prevention. During colonoscopy, a polyp lesion was found in the rectum, and it was resected with a polypectomy loop. Anatomopathological examination revealed a rectal carcinoid tumor with compromised margins. The lesion site was resected again and pathological exam no longer showed neoplasia. A systematic review of the issue was performed, discussing diagnosis aspects, difficulties in the choice of therapeutic approaches, and prognosis. The conclusion is that the rarity of the disease brings difficulties in the choice of treatment; although it has a good prognosis in most cases, its malignant potential cannot be underestimated.

RESUMO Apresentamos um caso clínico de um tumor carcinóide de reto em um paciente assintomático que procurou um serviço de colo-proctologia com o objetivo de fazer prevenção ao câncer colorretal. Durante a colonoscopia observou-se uma lesão polipóide no reto, a qual foi ressecada com alça de polipectomia. O exame anatomopatológico evidenciou tumor carcinóide de reto com comprometimento de margem. O local foi novamente ressecado e o resultado patológico não mais evidenciou neoplasia. Neste artigo é feita uma revisão bibliográfica do assunto abordando aspectos diagnósticos, dificuldade na escolha da conduta terapêutica e prognóstica. O trabalho conclui que a raridade do caso implica em dificuldades na escolha da conduta e que apesar de ter bom prognóstico, em uma parcela dos casos o seu potencial maligno não deve ser desprezado.

Tumores neuroendócrinos quísticos no funcionantes de páncreas. a propósito de un caso / Non-fuctional cystic neuroendocrine tumors of pancreas. a case report

Constituyen un grupo heterogéneo de tumores que pueden originarse virtualmente en todos los órganos; su etiopatogenia, diagnóstico y tratamiento aún generan discusiones, aunque se sabe que todos tienen algo en común: células neuroendócrinas como origen de los tumores, con similitud bioquímica e histológica con las células de la cresta neural. Se presenta el caso de una paciente, que fue operada en nuestro servicio y cuyo diagnóstico final fue tumor neuroendocrino quístico no funcionante de agresividad intermedia.

They constitute a heterogeneous group of tumors that can originate in virtually all organs; Its etiopathogenesis, diagnosis and treatment still generate arguments, although it is known that they all have something in common: neuroendocrine cells as the origin of tumors, with biochemical and histological similarity with neural crest cells. We present the case of a patient, who was operated on in our service and whose final diagnosis was non-functioning cystic neuroendocrine tumor of intermediate aggressiveness.

Gastric neuroendocrine tumor: review and update / Tumor neuroendócrino gástrico: revisão e atualização

ABSTRACT Introduction: The frequency of gastric neuroendocrine tumors is increasing. Reasons are the popularization of endoscopy and its technical refinements. Despite this, they are still poorly understood and have complex management. Aim: Update the knowledge on gastric neuroendocrine tumor and expose the future perspectives on the diagnosis and treatment of this disease. Method: Literature review using the following databases: Medline/PubMed, Cochrane Library and SciELO. Search terms were: gastric carcinoid, gastric neuroendocrine tumor, treatment. From the selected articles, 38 were included in this review. Results: Gastric neuroendocrine tumors are classified in four clinical types. Correct identification of the clinical type and histological grade is fundamental, since treatment varies accordingly and defines survival. Conclusion: Gastric neuroendocrine tumors comprise different subtypes with distinct management and prognosis. Correct identification allows for a tailored therapy. Further studies will clarify the diseases biology and improve its treatment.

RESUMO Introdução: A frequência de tumores neuroendócrinos gástricos está aumentando. As razões são a popularização da endoscopia e seus refinamentos técnicos. Apesar disso, os gástricos ainda são pouco compreendidos e têm manejo complexo. Objetivo: Atualizar os conhecimentos nos tumores neuroendócrinos gástricos e expor as perspectivas futuras no diagnóstico e tratamento. Método: Revisão da literatura utilizando as seguintes bases de dados: Medline/PubMed, Cochrane Library e SciELO. Os descritores da busca foram: carcinóide gástrico, tumor neuroendócrino gástrico, tratamento. Dos artigos selecionados, 38 foram incluídos nesta revisão. Resultados: Tumores neuroendócrinos gástricos são classificados em quatro tipos clínicos. A identificação correta do tipo clínico e grau histológico é fundamental, pois a conduta é variável e define a sobrevida. Conclusão: Tumor neuroendócrino gástrico possui diferentes subtipos com tratamento e prognóstico distintos. A identificação correta destes e seu entendimento permite o tratamento individualizado. Estudos futuros ajudarão a esclarecer a biologia desta doença e melhorar o tratamento.

Somatostatinoma duodenal / Duodenal somastotinoma

Alrededor del 70%de los tumores endocrinos bien diferenciados asientan en el tracto gastrointestinal. Los tumores duodenales bien diferenciados constituyen solo el 2,6 % de todos los tumores neuroendocrinos (NET).El somatostatinoma es un tumor raro que se localiza en páncreas o duodeno con una incidencia de 1:40 millones.La neurofibromatosis tipo I es una enfermedad autosómica dominante, la mutación en el gen supresor de tumores NF1 favorece la aparición de neoplasias en estos pacientes.

About 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated tumors account for only 2.6% of all neuroendocrine tumors. Somatostatinomas are rare neuroendocrine tumors (NETs) with an incidence of 1 in 40 million. These unusual tumors arise predominantly in the pancreas and peripancreatic duodenum. Neurofibromatosis type I is an autosomal dominant disease, the mutation in the tumor suppressor gene NF1 favors the appearance of neoplasms in these patients.

Tumor e síndrome carcinoide. Relato de caso / Carcinoid tumor and syndrome. Case report

As neoplasias neuroendócrinas são tumores raros, cuja prevalência varia de 0,7 a 4,48 casos em 100 mil habitantes. Menos de um quinto dos pacientes tem a síndrome carcinoide, que pode ser marcada por flushing, diarreia, dor abdominal, alterações cardíacas, pulmonares e pelagra. A dosagem do ácido 5-hidroxi-indolacético urinário e da cromogranina A sérica, exames de imagem e o estudo anatomopatológico da lesão auxiliam no diagnóstico. Neste estudo, relata-se o caso de paciente do sexo masculino, 47 anos, que apresentava diarreia intermitente com evolução de 5 anos e, 2 anos após, dor abdominal e empachamento, bem como percepção de flushing em face, tronco e partes proximais de membros superiores, inicialmente episódico e que, posteriormente, tornou-se fixo, com momentos de exacerbação. Marcadores ácido 5-hidroxi-indolacético urinário e cromogranina A foram positivos. Exame de imagem e estudo anatomopatológico/imuno-histoquímica de lesões focais hepáticas demonstraram tratar-se de tumor neuroendócrino. A cintilografia com octreotide marcado demonstrou lesões hepáticas já conhecidas. Trata-se, portanto, de um tumor neuroendócrino associado à síndrome carcinoide. Foi proposto tratamento com análogo de somatostatina. A síndrome carcinoide é uma manifestação rara dos tumores neuroendócrinos, mas sua identificação precoce é de suma importância para que possa ser oferecido tratamento com intuito curativo e melhor qualidade de vida.

Neuroendocrine tumors are rare. Their prevalence ranges from 0.7 to 4.48 cases per 100,000 inhabitants. Less than 1/5 of the patients have carcinoid syndrome, which can be marked by flushing, diarrhea, abdominal pain, cardiac and pulmonary disorders, pellagra. The measurement of urinary 5-hydroxyindoleacetic acid, the serum chromogranin A, imaging studies, and pathological study of the lesion support the diagnosis. In this study we report the case of a 47-year-old male patient, with five years of intermittent diarrhea and three years of abdominal pain and bloating, as well as perception of flushing in the face, trunk and proximal portions of the upper limbs, initially episodic but that became fixed with moments of exacerbation. The 5-hydroxy-indoleacetic acid and chromogranin A markers were positive. Imaging studies and the histopathological study/immunohistochemistry of the focal hepatic lesions demonstrated that these lesions were neuroendocrine tumors. The marked octreotide scintigraphy showed known liver lesions. It is, therefore, a neuroendocrine tumor associated with carcinoid syndrome. Treatment with a somatostatin analog was proposed. Carcinoid syndrome is a rare manifestation of neuroendocrine tumors, but its early detection is of paramount importance, so that clinicians can offer treatment with curative intent and better quality of life.

Tumor neuroendócrino de reto: relato de caso / Rectal neuroendocrine tumor: case report

Introdução e objetivo: o tumor neuroendócrino de reto é uma patologia rara que apresenta incidência de 0,86 por 100.000 habitantes. Cerca de 50% dos pacientes não apresenta sintomas específicos e sua lesão é semelhante a pólipos intestinais, sendo, a única opção de cura, a completa ressecção da lesão ainda localizada. O presente relato tem por objetivo apresentar uma neoplasia rara e ressaltar a importância de seu diagnóstico e ressecção precoces, sendo por muitas vezes diagnosticado apenas em colonoscopias de rotina ao acaso. Relato de caso: paciente de 40 anos, feminina, apresentando há três meses quadro de azia acompanhada de epigastralgia e dor abdominal difusa de fraca intensidade, sem outras comorbidades. Realizada endoscopia com hérnia hiatal e esofagite erosiva leve. A colonoscopia evidenciou lesão submucosa de 1 a 1,5 centímetros. Como tratamento foi realizado a mucosectomia e o laudo anatomopatológico de tumor neuroendócrino de reto grau 1. Paciente realizou exames laboratoriais e tomografia computadorizada de abdome, sendo normais, e colonoscopia de controle após três meses com completa cicatrização do local e sem novas lesões. Conclusão: quanto mais precoce é o diagnóstico, melhor é o estadiamento da lesão, podendo ser feito o tratamento apenas com ressecção endoscópica, apresentando ótimo prognóstico. Deve-se conhecer as características e classificações deste tipo de tumor que prevalece no trato gastrointestinal para melhor abordagem diagnóstica e terapêutica do paciente.

Introduction and Aim: The rectal neuroendocrine tumor is a rare patology present in scale of 0,86 per 100.000 in habitants. About 50% of the cases do not present specific symptoms. The lesion looks like an intestinal polips. The only option of the guaranteed cure is the complete resection of the localized lesion. The present report has an objective to present one rare neoplasm and to highlight the importance of the diagnosis and the procosis resection. The tumor is diagnosis most of the times, only in a routine colonoscopy. Case report: 40-years-old female patient, presenting by 3 months heartburn, with epigastralgia and low intensity of diffuse abdominal pain, without others comorbidity. It was realized one endoscopy showing one hernia hiatal and low erosive esophagitis. The colonoscopy showed submucosal lesion with 1 to 1,5 centimeters. For the treatment it was accomplished a mucosectomy and a pathology report of a rectal neuroendocrine tumor with degree one. Patient realized laboratory exams and abdominal computerized tomography. The result was normal and control colonoscopy after 3 months with completed healing of the local and no new lesions. Conclusion: the more precosis the diagnosis better the staging of the lesion, it may be realized the treatment only with the endoscopy resection and presents good prognosis. Must know the characteristics and classifications about this type of tumor that prevails at the intestinal tract for the better approach diagnosis and therapeutic.

Síndrome de zolinger - Elison / Syndrome of Zolinger - Elison

Resumen:El síndrome de Zollinger - Ellison es una endocrinopatía que fue descrita en 1955 por los doctores Robert Zollinger y Edwin Ellison, quienes propusieron la triada diagnóstica que incluye hipersecreción gástrica ácida, úlcera péptica y gastrinoma. Esta enfermedad predomina en mujeres entre los 50 y 60 años de edad. Según su etiología, este síndrome se clasifica en una forma esporádica o asociada a neoplasia endocrina múltiple tipo 1 (NEM - 1).Más de la mitad de los gastrinomas se localizan en la pared duodenal, el páncreas es la segunda ubicación en frecuencia. Existen localizaciones ectópicas en ovario, mesenterio, hígado y ducto biliar. A nivel histopatológico se encuentran células tumorales redondas, con núcleos pequeños y nucléolos prominentes. La hipersecreción ácida gástrica está asociada a un defecto en la inhibición del retrocontrol negativo de la somatostatina sobre las células G antrales productoras de gastrina. Clínicamente, los pacientes manifiestan dolor abdominal, diarrea, pirosis, náuseas y vómitos; relacionados principalmente a la formación de úlceras pépticas. El diagnóstico debe incluir una medición en los niveles séricos de gastrina y valores de pH gástrico. El tratamiento de primera línea es la terapia antisecretora, principalmente con inhibidores de la bomba de protones. Los estudios de imágenes son deutilidad para detectar metástasis y evaluar la enfermedad quirúrgicamente resecable. Se debe hacer diagnóstico diferencial con otros tumores neuroendocrinos y causas de hipergastrinemia.

Abstract:Zollinger - Ellison syndrome is an endocrinopathy that was first described in 1955 by doctors Robert Zollinger and Edwin Ellison, who proposed the diagnostic triad that includes gastric acid hypersecretion, peptic ulcer and gastrinoma. This disease predominates in women between 50 and 60 years old. Based on the etiology, the syndrome is classified in sporadic or associated with multiple endocrine neoplasia type 1 (NEM - 1). Over half of gastrinomas are located in the duodenal wall, the pancreas is the second frequency location. There are ectopic locations, such as ovary, mesentery, liver and bile duct. Round cells, small nuclei and prominent nucleoli, are the main hispathologycal characteristics. Gastric acid hypersecretion is associated with a defect in the negative feedback inhibition of somatostatin on G antral gastrin-producing cells. Clinically, patients present abdominal pain, diarrhea, heartburn, nausea and vomiting; primarily related to the development of peptic ulcers. Diagnosis includes a measurement in serum gastrin levels and gastric pH values. The first line treatment is the antisecretory therapy, primarily proton-pump inhibitor. Imaging studies are useful to detect metastases and evaluate the surgically resectable disease. Neuroendocrine tumors and hypergastrinemia causes are the main differential diagnoses, the clinician should consider.

Secreción ectópica de ACTH en un paciente con antecedentes de enfermedad de Cushing / ACTH's ectopic secretion in a patient with precedents of Cushing's disease

Hombre de 54 años con antecedentes de enfermedad de Cushing 32 años antes de la consulta. Ingresó por edemas asociados a astenia y adinamia. En el laboratorio se constató hipopotasemia y alcalosis metabólica. Se realizó diagnóstico humoral de síndrome de Cushing secundario a secreción ectópica de hormona adrenocorticotropa (ACTH). En la tomografía de tórax se halló un tumor de 3 × 3 cm en el mediastino anterior. La anatomía patológica de la pieza quirúrgica fue compatible con un carcinoide tímico. Este paciente sufrió en dos oportunidades un síndrome de Cushing, la primera por enfermedad (adenoma hipofisiario) y la segunda vez por secreción ectópica de ACTH (SEA) una asociación no descripta, en nuestro conocimiento, en la literatura médica.

A 54-year-old man, with a history of Cushing’s disease diagnosed 32 years earlier, presented with edema, asthenia and general malaise. Abnormal laboratory studies depicted hypokalemia and metabolic alkalosis. A CT scan of the chest revealed a 3 × 3 cm tumor in the anterior mediastinum. The pathology was consistent with a thymic carcinoid. These findings led to a diagnosis of biochemical Cushing’s syndrome secondary to ectopic secretion of ACTH. Thus, this patient suffered twice of Cushing’s syndrome. The first instance was the consequence of an ACTH - secreting pituitary adenoma and the second of an ectopic secretion of ACTH. To the best of our knowledge this is the first such case reported in the medical literature.

Tumor neuroendocrino ileal con síndrome carcinoide, patología de difícil diagnóstico y pronóstico variable / Ileal neuroendocrine tumor with carcinoid syndrome, neoplasia of difficult diagnosis and variable prognosis

Los tumores neuroendocrinos (TNE) presentan dificultad en el diagnóstico por sus síntomas inespecíficos y el manejo es un reto. Se presenta el caso de un varón de 78 años de edad, con dolor crónico en hemiabdomen superior. Ingresó a emergencia con obstrucción intestinal y síntomas de síndrome carcinoide (SC). En cirugía se resecó tumor ileal con resultado anatomopatológico de tumor neuroendocrino, cromogranina positivo, índice proliferativo ki 67 de 2 a 3 por ciento. El paciente continuó con síntomas de SC, altos niveles de 5-HIA, metástasis hepáticas en RMN. Se inició octreotide con adecuada respuesta clínica. La experiencia con el presente caso indica estar alerta con los síntomas de TNE funcionantes, debido al aumento en su incidencia. Es de utilidad solicitar marcadores inmunohistoquimicos. La identificación de un TNE con índice de mitosis y proliferación bajo indica buen pronóstico...

The neuroendocrine tumor (NET) is of difficult diagnosis due to nonspecific symptoms; management is also a challenge. We present the case of a 78 year-old male who suffered of chronic upper abdominal pain and was admitted to emergency with intestinal obstruction and symptoms of carcinoid syndrome (CS). At surgery an ileal tumor was resected with pathology report of NET positive for chromogranin A and ki 67 proliferative index of 2-3 per cent. The patient continued with CS symptoms and presented high levels of 5-HIAA and liver metastases by MRI. Octreotide was started with good outcome. Experience with this case indicates to be alert at symptoms of functioning NET, due to an increase in its incidence. It is useful to request immunohistochemical markers. Identification of NET with low grade mitosis and proliferation signal good prognosis...

Appendiceal Neuroendocrine Tumor with Lymph Node Metastasis in a Teenager / 대한소화기학회지

Neuroendocrine tumor (NET) is a cancer-like tumor that occurs mostly in the gastrointestinal system. Within the gastrointestinal tract, NET most commonly occurs in the rectum whereas appendix is very rarely involved. In most cases of appendiceal NET, it is found at a relatively early stage compared to other NETs because appendiceal NET frequently presents with acute appendicitis because appendiceal NET frequently presents with acute appendicitis even when the size is smaller than 1 cm. Therefore, it is very rare for lymph node metastasis to occur in a young adult. Herein, we report a rare case of grade 1 appendiceal NET with lymph node metastasis which developed in a teenage male.

Síndrome de Cushing ACTH Dependiente, secreción ectópica: desarrollo de un caso / ACTH Dependent Cushing Syndrome due to ectopic secretion: report of a case

Se presenta el caso de un paciente varón de 32 años, derivado a nuestro servicio para evaluación de cuadro de 8 meses de evolución caracterizado por edema y rubicundez facial, obesidad central, edema en miembros inferiores e hipertensión arterial, compatible con síndrome de Cushing clínico. Aportaba estudios previos con cortisol séríco 29,8 y 33 µg/dl (determinaciones realizadas con un mes de diferencia), ACTH 72,8 pg/ml, cortisol salival 2,1 µg/dl, cortisol libre urinario (CLU) 993,4 µg/24 hs. Los estudios imagenológicos (ecografía doppler renal, TAC de abdomen y pelvis c/contraste oral y e.v., TAC de cráneo s/contraste y RMI de cráneo c/contraste e.v.) no aportaron datos relevantes. Se confirmó bioquímicamente el síndrome de Cushing dependiente de ACTH: cortisol sérico (8 hs) 34,8 µg/dl, ACTH (8 hs) 72 pg/ml, cortisol libre urinario 828 µg/24 hs. El test de Nugent no mostró freno. El test de Liddle (8 mg oral dexametasona 23 hs) produjo un descenso del cortisol plasmático de solo 21%. La función tiroidea, las gonadotrofinas y la prolactina séricas eran normales. La radiografía de tórax mostró mediastino ensanchado e imagen nodular parahiliar basal derecha; esto se confirmó por TAC. La formación nodular medía 20 x 13mm, era de bordes lisos y aspecto inespecífico. Se exploró quirúrgicamente esta lesión, con diagnóstico intraoperatorio de población linfoide de pequeño tamaño. Se realizó nodulectomía por toracotomía con la sospecha de lesión linfoproliferativa. El diagnóstico anatomopatológico definitivo: tumor neuroendócrino bien diferenciado ...

The case of a male patient aged 32, referred to our service for evaluation of 8-month history of facial redness and edema,central obesity, lower limb edema and arterial hypertension consistent with clinical Cushing syndrome is presented. He hadprevious studies showing serum cortisol 29.8 and 33 mg/dl (determinations performed one month apart), ACTH 72.8 pg/ml, salivary cortisol 2.1 µg/dl, urinary free cortisol (UFC) 993.4 µg/24 h. Imaging studies (renal ultrasound doppler, CTof the abdomen and pelvis with oral and iv contrast, skull CT without contrast and skull RMI with iv contrast) did notprovide relevant data. Serum cortisol (8 hours) 34.8 µg/dl, ACTH (8 h) 72 pg/ml, urinary free cortisol 828 µg/24 h: anACTH-dependent Cushing’s syndrome was biochemically confirmed. Nugent’s test was negative. Overnight Liddle’s test (8mg oral dexamethasone 23 h) resulted in a modest decrease (21%) in plasma cortisol. Thyroid function, serum gonadotropinsand prolactin were normal. The chest radiograph showed widened mediastinum and a right basal parahilar nodularimage; this was confirmed by CT. The nodule measured 20 x 13 mm, it had smooth edges and nonspecific appearance. Thislesion was explored surgically, with intraoperative diagnosis of small lymphoid population. Lumpectomy was performed bythoracotomy The final pathological diagnosis was well-differentiated neuroendocrine tumo...

Hipoglicemia hiperinsulínica: a propósito de un caso / Hyperinsulinemic hypoglycemia: report of a case

El diagnóstico de hipoglicemia se confirma con la triada de Whipple: síntomas de hipoglicemia, concentración plasmática baja de glucosa, y desaparición de los síntomas con la elevación de los niveles de glucosa. Se presenta el caso de una paciente de 26 años de edad, que consultó a nuestro servicio en julio de 2013 por episodios intermitentes que se iniciaron en octubre del año precedente, consistentes en visión borrosa, cambios conductuales y discurso incoherente, sin relación con la ingesta o el ayuno, que aumentaron en frecuencia los meses previos a la consulta y llegaron a asociarse a pérdida de conocimiento y relajación de esfínteres, requiriendo asistencia médica prehospitalaria. Ocasionalmente hubo registros bajos de glicemia capilar, y hubo mejoría sintomatológica con la administración de glucosa e.v. Durante una prueba de ayuno presentó una hipoglicemia sintomática con glicemia 48 mg/dl, insulinemia 21,1 µU/ml, péptido C 2.257 ng/ml. La ecografía de abdomen identificó una imagen sólida en la unión de cuerpo y cola del páncreas. La lesión fue enucleada quirúrgicamente, y la anatomía patológica confirmó su origen neuroendócrino .

The diagnosis of hypoglycemia is confirmed by Whipple’s triad: symptoms of hypoglycemia, low plasma glucose concentrations, and disappearance of symptoms after glucose administration. The case of a 26-year-old woman is presented. She consulted our Service in July 2013 due to intermittent episodes that had begun in October of the preceding year, characterized by blurred vision, slurred speech, and behavioral changes, unrelated to food ingestion. They increased in frequency, and were associated eventually with loss of consciousness and loss of sphincters control, requiring medical care. Occasionally, low capillary glucose levels were recorded, and there was improvement in symptoms with i.v. glucose administration ev.During a fasting test she had symptomatic hypoglycemia with serum glucose of 48 mg/dl, serum insulin 21.1 µU/ml, and serum C-peptide 2.257 ng/ml. Abdominal ultrasound identified a rounded image in the union of the body and tail of the páncreas; this was confirmed by MRI. The lesion was surgically enucleated, and the pathology report confirmed its neuroendocrine origin .