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

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.

Compromiso cardiovascular secundario a tumor carcinoide metastásico / Cardiovascular compromise secondary to metastatic carcinoid tumor

Resumen Los tumores carcinoides son neoplasias pertenecientes a la familia de tumores neuroendocrinos y presentan baja incidencia en la población en general; sin embargo, hasta el 50 % pueden debutar con compromiso cardiovascular. Se presenta el caso de un paciente, sin antecedentes cardiovasculares quien presentó signos y síntomas sugestivos de falla cardiaca derecha. Se le realizó ecocardiografía transtorácica que evidenció compromiso de ambas válvulas cardíacas derechas por tumor neuroendocrino metastásico, llevando al diagnóstico de sindrome carcinoide. Con el fin de tener presente dicha entidad en la práctica diaria de la cardiología clínica se revisan datos claves de abordaje diagnóstico y terapéutico.

Abstract Carcinoid tumors are neoplasms belonging to the neuroendocrine tumor family and have a low incidence in the general population; however, up to 50% may debut with cardiovascular involvement. We present the case of a patient, without a cardiovascular history, who presented signs and symptoms suggestive of right-sided heart failure. A transthoracic echocardiography was performed, which evidenced the involvement of both right heart valves by a metastatic neuroendocrine tumor, leading to the diagnosis of carcinoid syndrome. In order to take this entity into account in the daily practice of clinical cardiology, key data of diagnostic and therapeutic approach are reviewed.

Quadruple valve replacement for carcinoid heart disease

Abstract Introduction: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. There are only four previous isolated case reports in the literature. Methods: All three patients underwent quadruple valve replacement during a single operation. Right ventricular outflow tract reconstruction with a pericardial patch was performed in all patients. For 24 hours prior to surgery, all patients received intravenous octreotide, which continued in intensive care for at least 24 hours. Results: Mean cross-clamp and bypass times were 175 (range 164-197 minutes) and 210 (range 195-229 minutes) minutes, respectively. Mean intensive treatment unit (ITU) and inpatient stays were 2.3 (range 2-3 days) and 12 (range 9-16 days) days, respectively. One patient was reopened for bleeding 4 hours postoperatively from a ventricular pacing wire site. None required a permanent pacemaker postoperatively. There were no other complications in any patient. The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1. Postoperative echocardiography showed no paravalvular leaks and well-functioning prostheses in all cases. Conclusion: Surgery to replace all four valves is feasible with excellent medium-term survival and a very low rate of complications. Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement.

Clinical study of umbilical compress using anti-cancer Xiaogu formula in the management of malignant ascites / 国际中医中药杂志

Objective To observe the clinical efficacy of umbilical compress with anti-cancer Xiaogu formula in managing malignant ascites. Methods A total of 56 patients with malignant ascites who met the inclusion criteria were randomized into 28 patients in the treatment group and 28 patients in the control group. The control group was treated with one type of diuretic, and the treatment group was given umbilical compress using Anti-Cancer Xiaogu formula and diuretics. Both groups were treated for 14 days. The changes of abdominal girth, 24 h urine volume, ascites efficacy, TCM syndrome scores, quality of life and adverse reactions were observed. Results The decrease in the maximum depth difference of the ascites in the treatment group was significantly greater than that of the control group (1.16 ± 1.29 vs. 0.00 ± 1.34, Z=-2.553). The difference was statistically significant (P<0.05). The decrease in abdominal girth in the treatment group was significantly larger than that in the control group (0.57 ± 0.55 vs. 2.61 ± 0.28, Z=-2.264). The difference was statistically significant (P<0.05). The in 24-hour urine volume in the treatment group after intervention (-800.18 ± 64.12 vs.-683.57 ± 55.38, Z=-1.770) was no statistically significant (P>0.05). The response rate in the treatment group was 92.9％ (26/28), while that of the control group was 89.3％ (25/28).treatment group was 71.4％, while that of the control group was 35.7％. The difference was statistically significant (P<0.05). The increase in KPS in the treatment group was significantly higher than that of the control group. The difference was statistically significant (P<0.05). Conclusions Anti-cancer Xiaogu umbilical cord combined with diuretic can reduce the degree of malignant ascites, alleviate clinical symptoms, improve quality of life and decrease the occurrence of adverse reactions when used concomitantly with diuretics in the management of malignant ascites.

Tratamiento quirúrgico de la enfermedad metastasica del síndrome carcinoide: hepatectomía parcial usando un bypass veno-venoso y posterior cambio valvular tricúspide. Reporte de caso / Surgical treatment of carcinoid syndrome metastatic disease: Partial hepatectomy with veno-venous bypass and subsequent tricuspid valve replacement. Case report

Abstract Introduction: Carcinoid syndrome is a relatively rare condition that may affect the liver and the right heart. Some of these cases may require surgical treatment with a multidisci-plinary approach. Case report: We report the case of a patient with progressive dyspnoea, arterial hypotension and facial flushing, diagnosed with liver and cardiac involvement from carcinoid syndrome. The patient was taken to surgery in two different occasions, first for metastatic liver resection, and then to subsequent cardiac surgery for tricuspid valve replacement. Conclusions: We report here a case review, including the clinical course, the intra-operative management, and the information available in the literature regarding which procedure to perform initially in these types of cases which are not very common in clinical practice.

Resumen Introducción: El síndrome carcinoide es una condición relativamente rara que puede afectar el hígado y el corazón derecho. En algunos pacientes se requiere tratamiento quirúrgico y un manejo multidisciplinario. Presentación del caso: Presentamos una paciente con diagnóstico de síndrome carcinoide con compromiso hepático y cardíaco, quien inició con disnea progresiva hasta el reposo, hipotensión arterial y rubor facial. La paciente fue llevada a cirugía en dos tiempos: la resección de las metástasis hepáticas inicialmente y, en un segundo tiempo, cirugía cardíaca para el cambio de la válvula tricúspide. Ambas intervenciones se realizaron con éxito y sin complicaciones. Conclusión: Presentamos aquí una revisión del caso, la evolución y el manejo intraoperatorio y la información disponible en la literatura para decidir qué procedimiento se debe realizar en primera instancia, ya que estos casos no son frecuentes en la práctica clínica.

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.

Síndrome carcinoide y compromiso valvular cardiaco, a propósito de 2 casos: Report of two cases / Tricuspid and pulmonary valve involvement in carcinoid syndrome

Carcinoid is a rare neuroendocrine tumor typically arising in the gastrointestinal tract that can cause heart valve involvement. We report two patients with carcinoid syndrome and tricuspid/pulmonary valve lesions. A 33-year-old male presenting with fatigue and weight loss: A tumor in the tail of the pancreas was found on an abdominal CAT scan. The percutaneous biopsy was informed as a carcinoid tumor. A trans-esophageal echocardiogram showed a tricuspid and pulmonary valve involvement, which was replaced surgically. The biopsy informed an extensive fibrous and myxoid degeneration of the valves. A 35-year-old male with a carcinoid syndrome and cardiac failure: An echocardiogram showed a severe tricuspid stenosis and severe pulmonary regurgitation. The patient was subjected to a double surgical valve replacement. The pathology report of the excised valve showed a deforming fibrous and myxoid valvulopathy.

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...

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 carcinóide: relato de caso / Carcinoid syndrome: case report

Os tumores carcinóides são raros, com incidência aproximada de um a dois casos por 100.000 habitantes. Estima-se que a síndrome carcinóide - que se caracteriza por flushing, diarréia, dor abdominal e, em menor freqüência, pelagra, broncoespasmo e doença valvar cardíaca - ocorra em menos de 10% dos doentes. Entretanto, nos casos avançados a incidência varia de 40 a 50%. Estudos retrospectivos e de série de casos mostram que as manifestações cutâneas são freqüentes nessa entidade, embora sejam raramente relatadas. Apresenta-se um caso de síndrome carcinóide diagnosticadoa partir das manifestações dermatológicas.

Carcinoid tumors are rare neoplasms with an estimated incidence of 1 to 2 cases per 100,000 inhabitants. The malignant carcinoid syndrome, characterized by flushing, diarrhea, abdominal pain and, less frequently, pellagra, bronchospasm and valvular disease, occurs in less than 10% of patients. Nevertheless, in advanced stages of the disease, the incidence is about 40 to 50%. Retrospective studies and descriptive case series have shown that cutaneous involvement is relatively common but has rarely been reported. We present a case of carcinoid syndrome diagnosed with basis on skin findings on clinical examination.