Crisis carcinoide como una causa poco frecuente de choque distributivo / Carcinoid crisis as a rare cause of distributive shock

Los tumores neuroendocrinos son neoplasias que suelen tener un comportamiento clínico maligno, son provenientes de células entero cromafines y/o células productoras de gastrina. Según su origen anatómico, se clasifican en tumores del intestino anterior (comprometen estómago, duodeno, pulmones y páncreas), intestino medio (parte distal del duodeno) e intestino posterior (colon transverso hasta el recto). El síndrome carcinoide, presente en la mitad de los casos al momento del diagnóstico, se caracteriza por desencadenar episodios de diarrea, taquicardia, hipotensión, rubor (por el desarrollo de telangiectasias), y según la gravedad, valvulopatías cardiacas. Por otro lado, la crisis carcinoide, una complicación infrecuente, está relacionada con episodios de choque, que cuando ocurren son consecuencia de la liberación en la circulación sistémica de aminas vasoactivas, posterior a un evento desencadenante. A continuación, se presenta el caso de un paciente masculino con antecedente de un tumor neuroendocrino de intestino delgado, quien luego de ser llevado a embolización de metástasis hepáticas, presentó una crisis carcinoide, y finalmente un choque distributivo refractario a tratamientos convencionales, incluyendo octreotide y vasopresores, que culminó con la muerte del paciente. Se realiza la discusión del caso clínico y la presentación de la literatura disponible, donde se describe la epidemiología, patogénesis, diagnóstico, clínica y tratamiento de esta entidad

Neuroendocrine tumors are neoplasms that usually have a malignant clinical behavior. They arise from enterochromaffin-like and/or gastrin-producing cells. According to their anatomical location they can be classified as foregut tumors (af- fecting stomach, duodenum, lungs and pancreas), midgut tumors (affecting distal portion of the duodenum) and hindgut tumors (affecting transverse colon to rectum). Carcinoid syndrome, occurring in half of the cases at the time of diagnosis, is characterized by episodes of diarrhea, tachycardia, hypotension, flushing (due to telangiectasia), and heart valve disease depending on their severity. On the other hand, the carcinoid crisis, a rare complication that is related to episodes of shock, occur as a consequence of the release of vasoactive amines into the systemic circulation after a triggering event. Here we describe a case of a male patient with a history of neuroendocrine neoplasm, who after embolization of hepatic metastatic lesions presented a carcinoid crisis, ending with a vasodilatory shock, refractory to conventional treatment including octreotide and vasopressors, which resulted in the death of the patient. A discussion of the clinical report and a review of the available literature are presented, including the epidemiology, pathogenesis, diagnosis, clinical manifestations and treatment of this entity

Diabetes Insipidus Induced by Combination of Short-acting Octreotide and Lanreotide for Recurrent Carcinoid Crisis of Neuroendocrine Tumour: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Somatostatin analogue is useful in carcinoid crisis for symptom control. Optimal dosing of somatostatin analogues for carcinoid symptoms is not known. This case highlighted management issues using combination short-acting octreotide infusion with long-acting lanreotide during carcinoid crisis. The patient had left lung neuroendocrine tumour that metastasized to his liver and bone, post left lobectomy. Due to extensive metastasis to the liver causing recurrent carcinoid crisis, he required shorter interval long-acting lanreotide with continuous infusion of short-acting octreotide, which led to transient diabetes insipidus. Symptoms resolved with discontinuation of treatment. Somatostatin analogues, especially in combination, may inhibit the posterior pituitary resulting in diabetes insipidus. Prompt withdrawal of short-acting somatostatin analogue and initiation of desmopressin can reverse the complication. It is important to recognize this complication with combination of octreotide and lanreotide injections to avoid serious complications.

Carcinoid syndrome: update on the pathophysiology and treatment

Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.

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 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...

Síndrome Carcinoide: Presentación de caso y revisión de la literatura / Carcinoid syndrome: case report and literature review

Se presenta el caso de una mujer de 63 años de edad, con cuadro clínico crónico de un año de evolución caracterizado por diarrea esteatorreica, asociado a episodios de dolor abdominal difuso, tipo cólico, “sensación de bochornos” y enrojecimiento en cara y tronco superior. El abordaje diagnóstico de la diarrea crónica es un reto para los médicos generales y especialistas, más aún, cuando se acompaña de manifestaciones inespecíficas como dolor abdominal y la presencia de “bochornos”. La coexistencia de varios de los anteriores síntomas, obliga a descartar diversas patologías que representan alta morbimortalidad para el paciente. El síndrome de intestino irritable, el feocromocitoma, el hipertiroidismo, el síndrome carcinoide, entre otras, son patologías a excluir en todo caso. El presente artículo pretende brindar el diagnóstico diferencial de las patologías que presentan dichos síntomas, buscando conducir al lector hasta el diagnóstico definitivo de la paciente.

A 63-year-old woman reported a chronic clinical evolution of one year characterized by steatorrhea, associated with episodes of diffuse abdominal pain, cramping and “hot flashes” also redness on the face and upper trunk . The diagnostic approach of chronic diarrhea is a challenge for physicians and specialists, especially, when accompanied by nonspecific manifestations such as abdominal pain and the presence of “hot flashes”. The coexistence of several of these symptoms must be ruled various pathologies that represent high morbidity and mortality for the patient. Irritable bowel syndrome, pheochromocytoma, hyperthyroidism, carcinoid syndrome, among others, are conditions to exclude in any case. This article aims to provide the differential diagnosis of the diseases that have these symptoms, seeking to lead the reader to the definitive diagnosis of the patient.

Síndrome carcinoide cardiaco / The carcinoid heart disease

El síndrome carcinoide cardiaco es una patología muy infrecuente que ocurre en el 50 por ciento de los pacientes con síndrome carcinoide maligno, y es caracterizado por la fibrosis de las válvulas tricúspide y pulmonar, así como del endocardio ventricular derecho, lo cual puede provocar una insuficiencia cardiaca derecha, que repercute directamente en el pronóstico de estos pacientes. Se reporta el caso de un masculino de 29 años, portador de síndrome carcinoide con metástasis hepáticas, referido para valoración cardiológica por presentar disnea de pequeños esfuerzos, con posterior diagnóstico de síndrome carcinoide cardiaco. Se discuten las características clínicas, diagnóstico y tratamiento de esta patología tan infrecuente...

Tumor carcinoide pulmonar típico / Typical lung carcinoid tumor

Mujer de 42 años de edad, con tres años de enfermedad caracterizada por episodios de tos productiva, fiebre y disnea, tratada con antibióticos por infecciones bronquiales recurrentes que coincidían con episodios de rubor facial, diarreas acuosas, de una a dos veces por mes, de carácter autolimitado. Presentó episodios de hemoptisis leve. Pese a tener frotis negativo para M. tuberculosis, recibió tratamiento por supuesta tuberculosis pleural, en un centro de salud, sin mejoría clínica, por lo que fue hospitalizada. La radiografía de tórax mostró atelectasia del lóbulo medio e inferior derecho. La tomografía evidenció neoproliferación hilio-basal derecha con marcada captación de medio de contraste, linfonodos metastásicos en hilio y mediastino derecho, incluidos los del grupo subcarinal, con atelectasia crónica en lóbulo medio con bronquiectasias cilíndricas. En la primera broncoscopias, en el bronquio intermediario derecho, se observó unalesión polipoide avascularizada, redondeada, cubierta por membrana blanquecina que ocluía el 100% de la luz, muy friable, que sangraba al roce del cepillo. El Papanicolaou del aspirado bronquial reveló citología sugestiva de neoplasia maligna. En la segunda broncoscopia con biopsia, se demostró tumor carcinoide típico, con inmunohistoquímica positiva para panqueratina y cromogranina.

A 42 year-old female with 3-year disease characterized by episodes of productive cough, fever and dyspnea. She was treated with antibiotics for recurrent respiratory infections, coincident with episodes of facial flushing and watery and self-limited diarrhea which occurred 1-2 times monthly. She had mild bouts of hemoptysis. Despite negative smears for M. tuberculosis, she received 4-drug treatment for pleural tuberculosis, in a primary health center, without clinical improvement, so she was hospitalized. X-ray chest film showed atelectasis of right middle and lower lobe. Computed tomography showed right hilum neoproliferation with marked uptake of contrast, hilar and mediastinal lymph node metastasis including subcarinal group, with middle lobe atelectasis and chronic cylindrical bronchiectasis. A first bronchoscopy revealed, in the right intermediate bronchus, an avascularizaded, polypoid lesion, rounded, covered with a white membrane occluding 100% of bronchus lumen, very friable and bleeding to the brush touch, whose Papanicolaou smear revealed a cytology suggestive of malignancy. The second bronchoscopy and biopsy showed a typical carcinoid tumor with positive immunohistochemistry for panqueratine and chromogranin.

Tumores neuroendocrinos gastrointestinales / Gastrointestinal neuroendocrine tumors

Los tumores neuroendocrinos gastrointestinales (TNEG), también llamados tumores carcinoides gastrointestinales, son neoplasias derivadas de células neuroendocrinas. Pueden formarse en todo el sistema gastrointestinal, siendo el íleon el lugar de origen más frecuente. Su forma de aparición es esporádica o asociada a síndromes familiares como von Hippel Lindau y neurofibromatosis. Los TNEG se diagnostican en su mayoría ya cuando la neoplasia ha invadido ganglios linfáticos u otros órganos distantes, ejemplo de esto es el síndrome carcinoide que se desarrolla por invasión hepática tumoral. La cirugía es el único tratamiento curativo...

Gastrointestinal neuroendocrine tumors (TNEG), also called gastrointestinal carcinoid tumors, are neoplasms arising from neuroendocrine cells. They may form all around the gastrointestinal system, being the ileum the most common place of origin. Its form of onset is sporadic or associated with familial syndromes such as von Hippel Lindau and neurofibromatosis. TNEG are mostly diagnosed when the tumor has already invaded lymph nodes or distant organs, being an example of this the carcinoid syndrome; which develops as a manifestation of hepatic invasion. Surgery is the only curative treatment...

Unusual Histological Findings of the Appendix Following Appendectomy; a Ten Year Histopathologic Study

Lesions of the appendix are common. The appendix is however exposed to other uncommon but deleterious lesions which if not detected early and prompt attention given immediately; may result in morbidity and mortality.Materials and methods This was a retrospective study which involved 1171 respondents aged between 2 and 80 years. Demographic data were obtained from the archives of the histopathology department of University of Benin Teaching Hospital. Results were analysed with chi square test and results presented in frequencies tables.Results A total of 1171 samples were analysed out of which 17 were miscellaneous findings. This included 7 diagnosed for chronic granulomatous. Parasitic infestations were observed in only two female and 1 male case respectively and their ages ranged between 21 and 50 years. One case each was seen in Carcinoid; intestinal necrosis; intestinal infarction; chronic HPV; haemorrhagic appendix (which was found in a patient between 70 and 80 year age range).ConclusionsThis study has further emphasised the need for routine histopathologic analysis of all appendices following appendectomies regardless of the macroscopic appearance

Collision adenoma-carcinoid tumour of the colon complicated by carcinoid syndrome

Tumours consisting of a glandular component, either an adenoma or adenocarcinoma, and a carcinoid component are uncommon. These tumours can be differentiated into collision, composite or amphicrine tumours. Most cases reported in the literature were mixed adenocarcinoma-carcinoid tumours. To date, only four cases of mixed adenoma carcinoid tumours have been reported in the literature. This case report describes a unique case of collision adenoma-carcinoid tumour in the colon complicated by carcinoid syndrome in a 45-year-old woman who presented with a one-month history of diarrhoea and weight loss. She developed recurrence of the carcinoid component of the tumour four months after endoscopic resection. We conclude that carcinoid syndrome can occur in an adenoma-carcinoid tumour; however, the prognosis of this condition is uncertain.

Síndrome carcinoide: relato de caso / Carcinoid syndrome: case report

JUSTIFICATIVA E OBJETIVOS: O tumor carcinoide é uma neoplasia rara, com prevalência de apenas 2,47 a 4,48 casos para cada 100.000 habitantes/ano, sendo a síndrome carcinoide ainda mais rara, estando presente em apenas 5% a 7% dessas neoplasias.Na imensa maioria das vezes, só se detecta o tumor quandoo paciente já apresenta sintomas da síndrome, tais como: flushing, diarreia, dor abdominal, telangiectasia, alterações cardíacas, broncoespasmo e pelagra. O objetivo deste estudo foi o de alertar para se incluir a suspeita de síndrome carcinoide como diagnóstico diferencial com outras condições que podem se apresentar com sintomas similares, como climatério, feocromocitoma, anafilaxia, uso de certos medicamentos, ingestão alcoólica e até mesmo a febre. RELATO DO CASO: Paciente do sexo feminino, 42 anos, apresenta há seis anos quadro de flushing na face, pescoço, tronco e membros superiores, evoluindo com eritema telangectásico e há um ano diarreia. Suspeitou-se de síndrome carcinoide, que se confirmou por meio da dosagem do ácido 5-hidroxindolacético na urina de 24h. A investigação prosseguiu com colonoscopia em que se encontrou lesão tumoral em íleo terminal, cuja histopatologia confirmou tumor carcinoide. Foi realizado tratamento com doses mensais de análogos da somatostatina de ação prolongada e programada cirurgia de ressecção tumoral. CONCLUSÃO: A síndrome carcinoide é uma manifestação rara e tardia do tumor, mas é fundamental que o clínico saiba identificá-la, pois apesar do prognóstico já ser desfavorável nessa fase, é possível ainda proporcionar melhor qualidade de vida ao paciente, com bom controle dos sintomas.

BACKGROUND AND OBJECTIVES: Carcinoid tumors are rare with a prevalence of only 2.47 to 4.48 cases per 100.000 inhabitants/year and the carcinoid syndrome is even rarer, present in 5% to 7% of the cases. In most patients, the neoplasm is detectedonly when the syndrome symptoms turn evident - flushing, diarrhea, abdominal pain, telangiectasia, cardiac manifestations, bronchospasm and pellagra. This case report objective was to alert for the importance of including carcinoid syndrome as a differential diagnosis of other conditions that could present similar symptoms like climacterium, pheochromocytoma, anaphylaxis, use of some medications, alcohol ingestion and also fever. CASE REPORT: Female patient, 42 years, has been presentingfor six years flushing episodes on face, neck, trunk and arms.Lately, could be observed telangiectasia and diarrhea. Carcinoid syndrome was suspected and the measurement of 24 hour urine5-hydroxyindoleacetic acid confirmed the diagnosis. The investigation proceeded with a colonoscopy which evidenced a tumoral lesion on the terminal ileum and the histopathology confirmed carcinoid tumor. Treatment was based on mensal doses of longacting somatostatin analogue for posterior tumor resection. CONCLUSION: Carcinoid syndrome is a rare and late tumor manifestation but it is fundamental for the clinician to knowhow to identify the symptoms because although the prognosisis already disfavorable at this stage, it is still possible to providequality of life and good symptoms control.

Síndrome de carcinoide con manifestación inicial a nivel valvular cardíaco: a raíz de un caso clínico / Carcinoid syndrome presenting as valvular diseas: report of one case

Carcinoid syndrome is observed in one third of carcinoid tumors and usually appears when there are liver metastases. One of the main complications of this syndrome is the appearance of tricuspid or pulmonary valvular disease. We report a 56 years old male presenting with malaise and a weight loss of 10 kg. On physical examination, a heart murmur suspicious of a double tricuspid lesion was found. The echocardiogram was suggestive of a carcinoid valvular disease. The abdominal CAT scan showed a small bowel tumor. Urinary 5-hydroxy-indol- acetic acid values were highly elevated. The patient was subjected to excision of the distal ileum, liver metastasectomy and hemicolectomy. The pathological study of the surgical piece confirmed the diagnosis of carcinoid tumor. Two years after surgery, the patient is in stable conditions.

Carcinoide gastrointestinal: experiencia de siete años en el Instituto Nacional del Cáncer (2000-2006) / Carcinoid tumors: retrospective review of 25 patients

Background: The incidence of carcinoid tumors (CT) has increased in the last five decades. These supposedly benign tumors may not always behave as such. The largest series of CT show that the most common primary tumor site (appendiceal) changed over the years. Aim: To evaluate the management of gastrointestinal CT at the National Cancer Institute from 2000 to 2006, describe their anatomic location and estimate disease specific survival. Material and Methods: Review of the database of the Cancer Committee, during a period of 7 years, looking for patients with a pathological diagnosis of CT, whose clinical records were analyzed. Results: The records of 25 patients with gastrointestinal CT, aged 18 to 79 years (19 females) were analyzed. Five years disease specific survival was 91.1 percent. The most common location was appendiceal in 56 percent of cases, with a 5 years disease specific survival of 100 percent. Right hemicolectomy was performed in seven patients with appendiceal carcinoid. Other seven patients only underwent appendectomy. Five years disease specific survival was 100 percent in both groups. Conclusions: The appendix is still the most common location of CT in this series and long term survival is acceptable.

Los tumores carcinoides (TC) gastrointestinales han aumentando su incidencia en las últimas 5 décadas. Inicialmente estos tumores eran considerados de comportamiento benigno, sin embargo, con el tiempo han mostrado ser capaces de diseminarse y tener una evolución más agresiva. En general se considera al apéndice cecal como la ubicación más frecuente de TC pero esto ha cambiado en el último tiempo según publicaciones internacionales. El objetivo de este trabajo es presentar nuestra experiencia en el manejo de los TC gastrointestinales en el período 2000-2006 en el Instituto Nacional del Cáncer (INC) reportando una serie de casos, describir su ubicación anatómica y estimar la sobrevida específica de enfermedad. Método: Estudio descriptivo. Se revisó la base de datos del Comité Oncológico del INC, durante un período de 7 años, incluyendo pacientes con diagnóstico anatomopatológico de TC. Se revisaron las fichas clínicas y se recolectaron registros de defunción. Resultados: 25 pacientes con diagnóstico de TC confirmado por patología, presentando una sobrevida específica de enfermedad a 5 años de 91,1 por ciento. La ubicación más frecuente de TC fue apéndice cecal (56 por ciento) y este grupo de pacientes presenta sobrevida específica de enfermedad de 100 por ciento a 5 años. De los TC apendiculares, en 7 pacientes se realizó una hemicolectomía derecha en un segundo tiempo, y en 7 pacientes sólo se realizó apendicectomía, con sobrevida específica de enfermedad de 100 por ciento en ambos grupos a 5 años (p = 0,475). Conclusiones: El apéndice cecal continúa siendo el lugar más frecuente de presentación del TC y la sobrevida alejada es adecuada en esta serie de pacientes.

Tumores neuroendocrinos gastroenteropancreáticos / Gastroenteropancreatic neuroendocrine tumors (GEP-NETS)

Los tumores neuroendocrinos gastroenteropancreáticos son neoplasias raras distribuidas a lo largo del tubo digestivo y poseen características peculiares, como la captación de sales de plata, la expresión de marcadores de célula neuroendocrina y los gránulos secretorios de contenido hormonal. Según su tamaño, localización anatómica y la presencia de metástasis, estos tumores debutan con distintas características clínicas y pronóstico. El diagnóstico temprano, que requiere de un alto grado de sospecha y una confirmación con estudios especializados, resulta invaluable para tratar estas lesiones a tiempo y aumentar la sobrevida de los pacientes. El tratamiento quirúrgico es la herramienta de primera mano, y otras terapias médicas ayudan a mejorar los síntomas y la calidad de vida de aquellos pacientes con lesiones irresecables. En esta revisión, se tratan los aspectos más relevantes en cuanto a la clasificación, morfología, localización, diagnóstico y tratamiento de estas neoplasias gastrointestinales, y al final, se expone la única experiencia colombiana sobre la epidemiología y el manejo de los tumores neuroendocrinos.

Gastroenteropancreatic neuroendocrine tumors (GEP-NETS) are rare neoplasms which can occur anywhere in the gastrointestinal tract. Their particular characteristics include uptake of silver salts, neuroendocrine cell marker expression and hormonal secretory granules. Depending on their size, anatomical location and upon whether or not metastasis has occurred, these tumors can show different clinical patterns and have different prognoses. Early diagnosis is essential for treating these lesions and improving the patients’ prognoses, but it requires a high degree of suspicion and confirmation by special testing. Surgical treatment is the first choice, but other medical therapy can be helpful for patients who have unresectable disease. This review presents the most relevant aspects of classification, morphology, methods of locating tumors, diagnosis and treatment of GEP-NETS. It presents only the Colombian experience in the epidemiology and management of these tumors.

Echocardiographic evaluation of intracardiac masses

Echocardiography plays a fundamental role in the evaluation of patients with an intracardiac mass. The ability to distinguish tissue characteristics, location, attachment, shape, size, and mobility non-invasively, quickly, and without the use of ionizing radiation makes echocardiography the ideal diagnostic modality. With careful attention to mass location and morphology, and appropriate application of clinical information, echocardiography can usually distinguish between the three principal intracardiac masses: tumor, thrombus, and vegetation. Equivocal transthoracic findings typically indicate the need for a transesophageal evaluation, during which the atria and great vessels might be better imaged. Surgical intervention is often indicated based on possible echocardiographic findings, without the need for additional time-consuming procedures. This review will focus on cardiac tumors

Síndrome carcinoides / Carcinoid syndrome

A raíz del estudio de un caso clínico, se describen las diarreas neuroendocrinas, señalando su escasa frecuencia; analizando su fisiopatología y detallando sus manifestaciones clínicas. Entre éstas destaca la diarrea que se caracteriza por ser hipersecretora, alta (entérica), liquida, voluminosa (mas de 500 ml) y explosiva. En todos estos cuadros se secretan sustancias hormonales que estimulan la motilidad intestinal y la secreción de agua y de electrolitis por las células de las criptas de Lieberkhun. De todas las diarreas neuro-endocrinas, las mas conocidas son el carcinoides y el vipoma o síndrome Werner-Morrison (pseudocólera pancreático).