Síndrome de Hedinger secundario a Tumor neuroendocrino bronquial. Presentación de un caso

El síndrome carcinoide es un síndrome paraneoplásico que se presenta en tumores neuroendocrinos. Aunque es una entidad infrecuente suele ser la primera manifestación de la enfermedad. La baja incidencia junto a la presentación inespecífica genera retrasos diagnósticos importantes. Se presenta el caso de una paciente con síntomas digestivos y tuforadas que posteriormente agrega insuficiencia cardíaca, logrando mediante un ecocardiograma típico y marcadores analíticos el diagnóstico de síndrome carcinoide. Posteriormente se evidencia que su origen en un tumor neuroendocrino bronquial. Conocer las características de este síndrome es fundamental para mantener una alta sospecha clínica en pacientes con síntomas sugestivos logrando un diagnóstico precoz y adecuado.

Carcinoid syndrome is a paraneoplastic syndrome that occurs in neuroendocrine tumors. Although It is an uncommon entity, it is usually the first manifestation of the disease. The low incidence besides the non-specific presentation generates important diagnostic delays. We present the case of a patient presenting digestive symptoms and flushing that subsequently adds heart failure, achieving though a typical echocardiogram and analytical markers the diagnosis of carcinoid syndrome. Later it is discovered its origin in a bronchial neuroendocrine tumor. Knowing the characteristics of this syndrome is essential to maintain a high clinical suspicion in patients with suggestive symptoms, in order to achieve an early and adequate diagnosis.

El síndrome carcinoide é um síndrome paraneoplásico que ocorre em tumores neuroendócrinos. Embora seja uma entidade rara, geralmente é a primeira manifestação da doença. A baixa incidência, juntamente com a apresentação inespecífica, resulta em atrasos importantes no diagnóstico. Apresentamos o caso de uma paciente com sintomas digestivos e ruborização cutânea, que posteriormente desenvolve insuficiência cardíaca. O diagnóstico de síndrome carcinoide foi estabelecido por meio de um ecocardiograma característico e marcadores analíticos. Posteriormente, foi evidenciada a origem em um tumor neuroendócrino brônquico. Conhecer as características deste síndrome é fundamental para manter uma alta suspeita clínica em pacientes com sintomas sugestivos, permitindo um diagnóstico precoce e adequado.

Refractory Diarrhea Associated with Carcinoid Syndrome Improved after Opioid Switching from Fentanyl to Morphine

Background: In the case of refractory diarrhea that cannot be treated with loperamide only, drugs such as octreotide and serotonin receptor antagonists are generally recommended. We have reported a case of refractory diarrhea associated with carcinoid syndrome in which symptoms improved only with opioid switching, without octreotide. Case: We experienced a case of a 28-year-old female with cervical cancer. She was diagnosed with recurrence after cervical cancer surgery and presented with pain and diarrhea. Her diarrhea did not improve sufficiently after taking loperamide. She was admitted to the palliative care hospital for symptom control due to persistent diarrhea and right lower extremity pain associated with bone metastasis. We diagnosed the cause of her diarrhea as carcinoid syndrome by some laboratory examination. For pain management, we switched opioids from transdermal fentanyl to continuous subcutaneous infusion of morphine. It resulted in pain relief and improvement in the frequency of diarrhea, and she was able to be discharged home. Conclusion: In cases of refractory diarrhea and in patients who need opioids, there is one option to use morphine. If it is effective, it may simply resolve both pain and diarrhea and reduce the use of multiple medications.

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

Neoplasias neuroendocrinas del intestino: serie de casos / Intestinal neuroendocrine tumors: case series

RESUMEN: Los tumores neuroendocrinos (TNE) intestinales representan el mayor porcentaje de este tipo de lesiones a nivel del aparato digestivo. El tratamiento de elección es la extirpación de la lesión primaria y sus linfonodos regionales. El objetivo de este estudio es reportar el resultado de pacientes portadores de TNE intestinales, tratados quirúrgicamente, en términos de morbilidad postoperatoria (MPO) y mortalidad. Serie de casos de pacientes con TNE intestinales intervenidos de forma consecutiva en Clínica RedSalud Mayor Temuco, entre 2006 y 2020. Las variables resultado fueron MPO y mortalidad. Otras variables de interés fueron localización y diámetro del tumor, tipo de cirugía y estancia hospitalaria. Se utilizó estadística descriptiva. Se trató a 11 pacientes (54,5 % mujeres), con una mediana de edad de 56 años. El 54,5 % de los tumores se localizó en yeyuno-íleon. La mediana del diámetro tumoral, tiempo quirúrgico y estancia hospitalaria fueron 2 cm, 75 min y 4 días, respectivamente. El tipo de resección más frecuente fue hemicolectomía derecha (63,6 %). La MPO fue 9,1 % (seroma en un paciente). No hubo reintervenciones ni mortalidad operatoria. Con una mediana de seguimiento de 18 meses, no se verificaron recurrencias. Los resultados reportados en relación a MPO y mortalidad, son adecuados en relación con la evidencia publicada.

SUMMARY: Intestinal neuroendocrine tumors (INETs) represent the highest percentage of this type of lesion in the digestive system. The treatment of choice is removal of the primary lesion and its regional lymph nodes. The aim of this study is to report the results of patients with INETs treated surgically, in terms of postoperative morbidity (POM) and mortality. Series of cases of patients with intestinal INETs operated consecutively at Clínica RedSalud Mayor Temuco, between 2006 and 2020. Result variables were POM and mortality. Other variables of interest were location and diameter of the tumor, type of surgery, and hospital stay. Descriptive statistics were used. Eleven patients (54.5 %) were treated, with a median age of 56 years. 54.5 % of the tumors were located in the jejunum-ileum. The median tumor diameter, surgical time, and hospital stay were 2 cm, 75 min, and 4 days, respectively. The most frequent type of resection was right hemicolectomy (63.6 %). The MPO was 9.1 % (seroma in one patient). There were no reoperations or operative mortality. With a median follow-up of 18 months, there were no recurrences. Reported results in relation to POM and mortality are adequate in relation to the published evidence.

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.

Tumor carcinoide primario de ovario que se presenta como enfermedad cardiaca carcinoide / Primary carcinoid tumor of the ovary presenting with carcinoid heart disease / Primary carcinoid tumor of the ovary presenting with carcinoid heart disease

RESUMEN El tumor carcinoide primario de ovario es una entidad poco frecuente que se caracteriza por la producción de sustancias vasoactivas que pasan directamente al torrente circulatorio y producen el llamado síndrome carcinoide. Estas sustancias también son las causantes de la formación de placas de fibrosis en las válvulas cardiacas, principalmente las derechas. Presentamos un caso de tumor carcinoide primario de ovario con afectación de la válvula tricúspide, que fue diagnosticado a partir de la lesión cardiaca. En la literatura están descritos casos clínicos aislados y revisiones de casos que nos pueden ayudar en el diagnóstico y en la toma de decisiones terapéuticas. Debemos conocer la existencia de estos tumores; la clínica debe alertarnos sobre su presencia. Es primordial un manejo multidisciplinar junto con endocrinólogos, cardiólogos, oncólogos, anatomo-patólogos, anestesistas, ginecólogos y cirujanos cardiacos, para ofrecer un tratamiento completo.

ABSTRACT Primary ovarian carcinoid tumor is a rare neoplasm characterized by the production of vasoactive substances that enter the bloodstream and give rise to the so-called carcinoid syndrome. These substances may also cause fibrotic plaques in the heart valves, mainly those in the right cardiac chambers. We describe the case of a primary ovarian carcinoid tumor with tricuspid valve involvement in which the diagnosis was prompted by identification of cardiac involvement. Knowledge of the clinical manifestations of neuroendocrine tumors is important in diagnosis and patient management. Recognition of typical clinical features may help suggest the correct diagnosis. Multidisciplinary discussion allows the formulation of a comprehensive management strategy involving endocrinologists, cardiologists, oncologists, pathologists, anaesthesiologists, gynaecologists and heart surgeons.

Anesthetic management of carcinoid syndrome: is octreotide enough? A case report / Manejo anestésico del síndrome carcinoide: es el octreótido suficiente? Presentación de un caso

Abstract: Carcinoid tumors are rare slow-growing neuroendocrine tissue neoplasms. Their ability to secrete bioactive substances to the systemic circulation is accountable for a clinical presentation known as carcinoid syndrome. Main symptoms include bronchoconstriction, flushing, diarrhea and hemodynamic instability. Octreotide, a somatostatin analogue, is the current mainstay for carcinoid syndrome treatment and perioperative management. However, no regimen has proven to be completely effective in preventing systemic manifestations and recent literature suggests that it might be an insufficient measure. We report a case of a 51-year-old male with a functioning small bowel neuroendocrine tumor and carcinoid syndrome presenting for a primary tumor resection, discussing possible pitfalls and key points in the care of these patients.

Resumen: Los tumores carcinoides son neoplasias de tejido neuroendocrino poco comunes y de crecimiento lento. Su capacidad para secretar sustancias bioactivas a la circulación sistémica es responsable por una presentación clínica conocida como síndrome carcinoide. Los principales síntomas incluyen broncoconstricción, enrojecimiento, diarrea e inestabilidad hemodinámica. Octreótido, un análogo de la somatostatina, es el pilar actual para el tratamiento del síndrome carcinoide y su manejo perioperatorio. Sin embargo, ningún tratamiento ha demostrado ser completamente eficaz para prevenir las manifestaciones sistémicas y estudios recientes indican que puede ser una medida insuficiente. Presentamos un caso de un varón de 51 años con un tumor neuroendocrino funcionante en el intestino delgado y un síndrome carcinoide, sometido a una resección del tumor primario, discutiendo posibles dificultades y puntos clave en la atención de estos pacientes.

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.

Pharmacological and Clinical Evaluation of Telotristat Ethyl: A Peripheral Tryptophan Hydroxylase Inhibitor for the Treatment of Carcinoid Syndrome / 中国药师

Telotristat ethyl is a peripheral tryptophan hydroxylase (TPH) inhibitor for the treatment of carcinoid syndrome. Telotristat ethyl inhibits TPH,thereby reducing the production of serotoin(5-HT) and the daily bowel movement. Pharmacology, pharmacokinetics,clinical studies and safety of telotristat ethyl were reviewed in the paper. Telotristat ethyl has become a novel treatment option for carcinoid syndrome patients. Telotristat ethyl is well tolerated with low incidence of side effects.

Mature cystic teratoma complicated with primary ovarian carcinoid:A report of 3 cases and literature review / 吉林大学学报(医学版)

Objective:To discuss the clinical characteristics,diagnosis,treatment experience and prognosis of mature cystic teratoma complicated with primary ovarian carcinoid,and to summarize its general charateriseics and to enhance the knowledge of clinician.Methods:Three female patients presented unilateral ovarian tumor in physical examination and were admitted to hospital.The tumor markers of three patients were normal. The preoperative diagnosis was ovarian tumor.Unilateral salpingo-oophorectomy was conducted in two patients.The resection of ovarian tumor was conducted in one patient.Results:The three patients underwent operation successfully.The postoperative pathological diagnosis was mature cystic teratoma complicated with primary ovarian carcinoid.The patients recovered well,who accepted 15-month follow-up with normal life and stable condition,and had no recurrence and metastasis.Conclusion:Mature cystic teratoma complicated with primary ovarian carcinoid is easy to be misdiagnosed before operation.Salpingo-oophorectomy is commonly used as the treatment method of mature cystic teratoma complicated with primary ovarian carcinoid and it can result in good effectiveness and better prognosis.

Mature cystic teratoma complicated with primary ovarian carcinoid: A report of 3 cases and literature review / 吉林大学学报(医学版)

Objective: To discuss the clinical characteristics, diagnosis, treatment experience and prognosis of mature cystic teratoma complicated with primary ovarian carcinoid, and to summarize its general charateriseics and to enhance the knowledge of clinician. Methods: Three female patients presented unilateral ovarian tumor in physical examination and were admitted to hospital. The tumor markers of three patients were normal. The preoperative diagnosis was ovarian tumor. Unilateral salpingo-oophorectomy was conducted in two patients. The resection of ovarian tumor was conducted in one patient. Results: The three patients underwent operation successfully. The postoperative pathological diagnosis was mature cystic teratoma complicated with primary ovarian carcinoid. The patients recovered well, who accepted 15-month follow-up with normal life and stable condition, and had no recurrence and metastasis. Conclusion: Mature cystic teratoma complicated with primary ovarian carcinoid is easy to be misdiagnosed before operation. Salpingo-oophorectomy is commonly used as the treatment method of mature cystic teratoma complicated with primary ovarian carcinoid and it can result in good effectiveness and better prognosis.

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.

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.

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.

Carcinoid Heart Disease: A Review.

Carcinoid tumors are rare malignancies able to spread and produce bioactive humoral products, mainly serotonin, which is responsible for the Carcinoid Syndrome (CS); its features are: flushing, diarrhea, bronchospasm and valvular heart disease. The Carcinoid Heart Disease (CHD) importantly worsens prognosis and it is found in up to 50% of patients with CS. After being produced by liver implants, serotonin finds its way straight into the right heart cavities, leading to valve tissue aggression and ventricular dysfunction. Early CHD diagnosis is still a challenge due to the asymptomatic initial stage, until right heart failure develops along with ascites, swelling and hepatomegaly. Echocardiography is still the main tool for diagnosis, especially due to its ability to appropriately evaluate ventricular and valve function, cardiac morphology and hemodynamics. Tricuspid regurgitation, pulmonic stenosis and dilated cardiomyopathy are the main impairments found in CHD. Magnetic resonance imaging has also developed well in this field, and it is now believed to be essential, due to accurate right cavities evaluation and fibrosis detection. For better prognosis, early diagnosis must be pursued, which has impact on clinical management and valve repair surgical decision.

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.