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.

Tumor carcinoide de células caliciformes apendicular con metástasis endometrial

El tumor carcinoide de células caliciformes es una neoplasia mixta casi exclusiva del apéndice, con diferenciación neuroendocrina y mucinosa. La afección metastásica endometrial por carcinomas extragenitales, especialmente el tipo de células de anillo de sello, es rara. Se presenta un caso de tumor carcinoide de células caliciformes apendicular con metástasis endometrial. Se trató de paciente femenina de 70 años quien presentó sangrado genital. El examen ginecológico mostró sangrado genital en moderada cantidad de color rojo-marrón y cuello uterino endurecido. En la biopsia endometrial se halló nidos de células de anillo de sello. La impresión diagnóstica provisional fue carcinoma mal diferenciado de probable origen intestinal. Durante la intervención, el útero tenía consistencia pétrea, el apéndice cecal era fibrótico y engrosado y el epiplón engrosado con nódulos tumorales. Las secciones anatómicas del cuello y cuerpo uterino mostraron focos tumorales. En el apéndice cecal se encontró acúmulos de pequeño tamaño compuestos por células caliciformes. La inmunotinción fue positiva a sinaptofisina, CDX-2, EMA, CK20, CD56 focal. Estos hallazgos confirmaron el diagnóstico de tumor carcinoide de células caliciformes, un tumor caracterizado por infiltración de la pared apendicular por pequeños nidos o cordones de células caliciformes con mucina intracitoplasmática y expresión focal de marcadores neuroendocrinos. Estas neoplasias tienen un comportamiento más agresivo que los tumores neuroendocrinos. La metástasis endometrial es rara y puede ser confundida con un carcinoma primario de células de anillo de sello. Se debe considerar como diagnóstico diferencial después de excluir otros tumores primarios.

Goblet cell carcinoid tumor is an almost exclusive mixed neoplasm of the appendix with neuroendocrine and mucinous differentiation. Endometrial metastatic involvement by extragenital carcinomas, especially the signet ring cell type, is rare. A case of appendiceal goblet cell carcinoid tumor with endometrial metastasis is presented. This was a 70-year-old female patient who presented with genital bleeding. The gynecological examination showed moderate red-brown genital bleeding and hardened cervix. Endometrial biopsy reported nests of signet ring cells. The provisional diagnostic impression was poorly differentiated carcinoma of probable intestinal origin. During surgery, the uterus had a stony consistency, the cecal appendix was fibrotic and thickened, and the omentum was thickened with tumor nodules. Anatomical sections of the cervix and uterine body showed tumor foci. In the cecal appendix, small clusters composed of goblet cells were found. Immunostaining was positive for synaptophysin, CDX-2, EMA, CK20, focal CD56. These findings confirmed the diagnosis of goblet cell carcinoid tumor, a tumor characterized by infiltration of the appendiceal wall by small nests or cords of goblet cells with intracytoplasmic mucin and focal expression of neuroendocrine markers. These neoplasms have a more aggressive behavior than neuroendocrine tumors. Endometrial metastasis is rare and can be mistaken for a primary signet ring cell carcinoma. It should be considered as a differential diagnosis after other primary tumors have been excluded.

Síndrome de Hedinger / Hedinger syndrome

Resumen La afectación cardíaca por tumor carcinoide (síndrome de Hedinger) sigue siendo una causa importante de morbilidad y mortalidad entre los pacientes con síndrome carcinoide y tumores neuroendocrinos metastásicos. El estudio de imagen inicial ante la sospecha clínica es el ecocardiograma transtorácico, el cual es importante para la detección temprana de la afectación cardíaca. La cirugía cardíaca, en casos apropiados, es la única terapia definitiva para el tumor carcinoide con afectación cardíaca y mejora los síntomas del paciente y la supervivencia. El tratamiento es complejo y multidisciplinario; el estado cardíaco, el síndrome hormonal y la carga tumoral son fundamentales para guiar el momento óptimo de la cirugía. Se presenta el caso de una paciente joven, con síndrome de Hedinger con afectación valvular tricúspide y pulmonar, quien recibió tratamiento quirúrgico y tuvo evolución exitosa.

Abstract Cardiac carcinoid tumor involvement (Hedinger's syndrome) remains a major cause of morbidity and mortality among patients with carcinoid syndrome and metastatic neuroendocrine tumors. The initial imaging study in case of clinical suspicion is the transthoracic echocardiogram, which is important for the early detection of cardiac involvement. Cardiac surgery, in appropriate cases, is the only definitive therapy for carcinoid tumor with cardiac involvement and improves patient symptoms and survival. Treatment is complex and multidisciplinary; cardiac status, hormonal syndrome, and tumor burden are essential to guide the optimal timing of surgery. The case of a young female patient with Hedinger's syndrome with tricuspid and pulmonary valve involvement, who received surgical treatment and had a successful evolution.

Middle ear adenomatous neuroendocrine tumors: suggestion for surgical strategy / Tumores neuroendócrinos adenomatosos da orelha média: sugestão de estratégia cirúrgica

Abstract Introduction Middle ear adenomatous neuroendocrine tumors are extremely rare neoplasms with epithelial and neuroendocrine differentiation, accounting for fewer than 2% of all middle and inner ear tumors. Universal standard surgical procedures for different stages of these tumors remain elusive due to the limitation of the small number of case reports or investigations. Objective(s) This study intends to investigate proper surgical strategies for patients with middle ear adenomatous neuroendocrine tumors. Methods Six patients with middle ear adenomatous neuroendocrine tumors who were treated at the Second Affiliated Hospital of Nanchang University (Nanchang, China) and the Eye, Ear, Nose, and Throat Hospital of Fudan University (Shanghai, China) respectively. Clinical characteristics and management strategies of patients were reviewed. The mean follow-up time was 63.7 months (range, 13-153 months). All the information was collected from medical records and prognosis postoperatively. Results Three patients underwent canal wall-up tympanomastoidectomy, including one patient with recurrence who underwent a previous tympanotomy; the other three patients underwent lateral temporal bone resection All of these patients were followed up with no evidence of recurrence or metastasis. Patients underwent canal wall-up surgery treatment accompanied with hearing function preservation measurements during follow-up periods. Conclusions Complete surgical resection provided good results for patients with middle ear adenomatous neuroendocrine tumors. The ossicular chain should be removed. Because of the propensity for local recurrence and invasiveness, as well as regional or distant metastasis of these tumors, it is necessary to schedule long-term follow-up and an observation plan postoperatively.

Resumo Introdução Os tumores neuroendócrinos adenomatosos da orelha média são neoplasias extremamente raras, com diferenciação epitelial e neuroendócrina, responsáveis por menos de 2% de todos os tumores de orelha média e interna. Os procedimentos cirúrgicos padrão universais para diferentes estágios desses tumores permanecem indefinidos, devido à limitação do pequeno número de relatos de casos ou investigações. Objetivo Este estudo foi feito com o objetivo de investigar estratégias cirúrgicas adequadas para pacientes com tumores neuroendócrinos adenomatosos da orelha média. Método Seis pacientes com tumores neuroendócrinos adenomatosos da orelha média foram tratados no Second Affiliated Hospital da Nanchang University (Nanchang, China) e no Eye, Ear, Nose, and Throat Hospital da Fudan University (Xanghai, China), respectivamente. As características clínicas e estratégias de tratamento dos pacientes foram revisadas. O tempo médio de seguimento foi de 63,7 meses (variação de 13 a 153 meses). Todas as informações foram coletadas dos prontuários e prognóstico no pós-operatório. Resultados Três pacientes foram submetidos à timpanomastoidectomia do tipo canal wall-up, inclusive um paciente com recorrência submetido a uma timpanotomia anterior; os outros três pacientes foram submetidos à ressecção lateral do osso temporal. Todos os pacientes foram acompanhados, sem evidência de recorrência ou metástase. Os pacientes foram submetidos ao tratamento cirúrgico com a técnica de canal wall-up acompanhado de medidas de preservação da função auditiva durante os períodos de seguimento. Conclusões A ressecção cirúrgica completa proporcionou bons resultados para pacientes com tumores neuroendócrinos adenomatosos da orelha média. A cadeia ossicular deve ser removida. Devido a propensão à recorrência e invasão local, bem como metástases regionais ou distantes desses tumores, é necessário um seguimento de longo prazo e um plano de seguimento no pós-operatório.

An unusual finding of tumorlet carcinoid and endobronchial hamartoma in the lobar bronchus / Hallazgo inusual de tumorlet carcinoide y hamartoma endobronquial en el bronquio lobar

SUMMARY: Neuroendocrine lung tumours are a group of different tumours that have similar morphological, immunohistochemical and molecular characteristics, and represents 1-2 % of all malignant lung tumours. Tumorlet carcinoids represent the nodular proliferation of hyperplastic neuroendocrine cells that is smaller than 5 mm in size. In this study, we reported the unusual finding of tumour carcinoid and endobronchial hamartoma in the same bronchus. A 49-year-old male patient with symptoms of prolonged severe cough and fever, and was treated for pneumonia. Since he did not adequately respond to antibiotic therapy lung CT scan was performed which showed middle lobe bronchus obstruction. Bronchoscopy revealed a lobulated whitish tumour which was biopsied and histopathological diagnosis was hamartoma. Tumour could not be completely removed during bronchoscopy, it was decided to surgically remove it. On serial section, during gross examination in the same bronchus, an oval yellowish area with a diameter of 3 mm was found along the bronchial wall. According to gross and histomorphological characteristics and immunophenotype of tumour cells, the diagnosis of tumour carcinoid was set. Diagnosis of carcinoids of the tumorlet type is usually an accidental finding and it can be seen on CT in the form of subcentimeter, single or multiple, nodular changes. Considering that the clinical picture is nonspecific, they should always be kept in mind as a possible differential diagnosis.

RESUMEN: Los tumores neuroendocrinos de pulmón son un grupo de tumores de diferentes características morfológicas, inmunohistoquímicas y moleculares similares, y representan el 1- 2 % de todos los tumores malignos de pulmón. Los carcinoides tumorales representan la proliferación nodular de células neuroendocrinas hiperplásicas de tamaño inferior a 5 mm. En este estudio reportamos el hallazgo inusual de tumor carcinoide y hamartoma endobronquial en el mismo bronquio. Un paciente varón de 49 años con síntomas de tos severa prolongada y fiebre fue tratado por neumonía. Al no responder adecuadamente a la terapia con antibióticos, se realizó una tomografía computarizada de pulmón que mostró obstrucción del bronquio del lóbulo medio. La broncoscopia reveló una tumoración blanquecina lobulada de la cual se tomó biopsia y el diagnóstico histopatológico fue hamartoma. No fue posible extirpar el tumor por completo durante la broncoscopia y se decidió extirparlo quirúrgicamente. En la sección seriada, durante el examen macroscópico en el mismo bronquio, se encontró un área amarillenta ovalada de 3 mm de diámetro a lo largo de la pared bronquial. De acuerdo a las características macroscópicas e histomorfológicas y de inmunofenotipo de las células tumorales, se estableció el diagnóstico de tumor carcinoide. El diagnóstico de carcinoides de tipo tumorlet suele ser un hallazgo accidental y se observan en la TC en forma de cambios nodulares subcentimétricos, únicos o múltiples. En consideración de que el cuadro clínico es inespecífico, siempre debe tenerse en cuenta como posible diagnóstico diferencial.

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

Compromiso tumoral de vía aérea en pediatría / Pediatric airway compromise by tumors

Los tumores traqueo bronquiales son extremadamente infrecuentes en la edad pediátrica e incluyen lesiones benignas y malignas. Por la baja frecuencia en niños y sintomatología respiratoria inespecífica, la sospecha diagnostica es habitualmente tardía. El tratamiento de elección en la mayoría de ellos es la resección quirúrgica abierta, sin embargo, la remoción endoscopia podría estar indicada es casos muy seleccionados con histología benigna y de localización accesible.

Tracheobronchial tumors are extremely rare in children and include benign and malignant lesions. Due to the low frequency in children and nonspecific respiratory symptoms, diagnostic suspicion is usually late. The treatment of choice in most of them is open surgical resection, however, endoscopy removal could be indicated in highly selected cases with benign histology and accessible location.

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.

Carcinoide atípico de timo en varón de 39 años: Reporte de caso / Atypical carcinoid of thymus in a 39-year-old-man: A case report

RESUMEN El carcinoide atípico (CA) de timo es la neoplasia más agresiva y rara que surge en el mediastino anterior y que pertenece a los tumores primarios neuroendocrinos de timo. La mayoría de los pacientes son asintomáticos y según la extensión de la enfermedad pueden presentar desde tos, disnea, dolor torácico hasta síndrome de vena cava superior. Esta presentación clínica inespecífica disminuye la probabilidad del diagnóstico temprano que, sumado con el estadio avanzado al debut y la imposibilidad de resección quirúrgica reduce la tasa de supervivencia. El objetivo es dar a conocer la presentación clínica, imagenológica y patológica en un varón de 39 años con CA de timo cuyo diagnóstico definitivo se basó en el estudio histopatológico (morfología carcinoide, mitosis 0-1/2 mm2, necrosis, Ki 67 = 12%) y marcadores inmunofenotípicos del tumor (CD 56 (+), Panqueratina (+), Sinaptofisina (+), TTF -1 (-)).

ABSTRACT The atypical carcinoid (AC) of thymus is the most aggressive and uncommon neoplasm in the anterior mediastinum, that belongs to the neuroendocrine primary tumors of thymus. Most patients are asymptomatic and according to the disease extension they may present with cough, dyspnea, chest pain, and superior cava vein syndrome. This non-specific clinical presentation reduces the likelihood for making an early diagnosis; and this, together with disease stage and lack of surgical resection reduces the survival rate. The aim of this paper is to describe the clinical presentation in a 39-year old male with thymus carcinoid whose diagnosis was based on histopathological studies (carcinoid morphology, 0-1/2 mitoses/mm2), necrosis, Ki67, 12%), and immunophenotypic tumor markers (CD56(+), Pankeratin (+), Synaptophysin (+), and TTF-1 (-)).

Insuficiencia cardíaca por un síndrome carcinoide: un reto diagnóstico. Reporte de caso / Carcinoid heart disease: a diagnostic challenge. Case Report

Resumen Introducción. La insuficiencia cardíaca es una patología frecuente que no solo es causada por isquemia miocárdica, hipertensión o valvulopatías, sino también por un gran número de enfermedades; sin embargo, en la práctica, muy pocas veces se aborda su etiología, y, por lo tanto, su manejo usualmente es sintomático, situación que puede repercutir negativamente en la evolución natural de la enfermedad, ya que es sabido que un diagnóstico etiológico oportuno puede mejorar el pronóstico de cualquier enfermedad. Los principales signos clínicos del tumor carcinoide típico son diarrea, flushing facial y broncoespamo, sin embargo el compromiso cardíaco también ha sido descrito en pacientes con esta condición (menos del 20%). Presentación del caso. Paciente masculino de 77 años que acudió al servicio de urgencias con un cuadro clínico de insuficiencia cardíaca descompensada. Teniendo en cuenta la pobre respuesta del paciente ante el manejo propuesto y la evidencia de diarrea crónica, se realizaron varios estudios diagnósticos, lo que permitió diagnosticarlo con un tumor neuroendocrino. Conclusiones. La insuficiencia cardíaca es una patología frecuente en la población general, sin embargo la identificación temprana de causas poco comunes, tales como el síndrome carcinoide, permitirá implementar un manejo oportuno e identificar complicaciones asociadas como la cardiopatía carcinoide, lo que tendrá un impacto positivo en la calidad de vida y pronóstico del paciente.

Abstract Introduction: Heart failure is a frequent disease that is not only caused by myocardial ischemia, hypertension or valve disease, but also by many other diseases. However, its etiology is rarely addressed, and therefore its management is usually symptomatic, which may affect the course of the disease, taking into account that an early etiological diagnosis could lead to better outcomes. The main clinical signs of the typical carcinoid tumor are diarrhea, facial flushing and bronchospasm, although heart failure symptoms have also been described in patients with this condition (less than 20%). Case presentation: A 77-year-old man visited the emergency department due to decompensated heart failure. Considering his poor response to the proposed treatment and symptoms such as chronic diarrhea, several diagnostic studies were performed, which allowed diagnosing him with a neuroendocrine tumor. Conclusions: Heart failure is a frequent pathology in the general population. However, the early detection of rare causes, such as carcinoid syndrome, will allow implementing adequate studies, staging and treatment in order to prevent complications such as carcinoid heart disease, which will have a positive impact on the patient's quality of life and prognosis.

Tumor carcinoide de duodeno: un raro e interesante hallazgo incidental / Carcinoid tumor of the duodenum: a rare and interesting incidental finding

RESUMEN Fundamento: el tumor carcinoide de localización duodenal es poco frecuente, representa 2 % de los tumores neuroendocrinos gastrointestinales. Por lo cual se presenta un caso de este, recientemente diagnosticado en el Hospital Clínico Quirúrgico Joaquín Albarrán de La Habana. Objetivo: realizar la comunicación de un caso de tumor carcinoide del duodeno y revisar la literatura con énfasis en el diagnóstico histopatológico. Presentación del caso: paciente masculino de 86 años de edad que fallece al quinto día de su ingreso. En la autopsia, durante el examen macroscópico de los intestinos, se revela a nivel de la primera porción del duodeno, una lesión que por su histopatología se correspondió con tumor carcinoide de duodeno. Conclusiones: el carcinoide duodenal es un tumor raro cuyo diagnóstico endoscópico o histológico se realiza en la gran mayoría de los casos de forma incidental; se asocia por lo general con una progresión benigna, aunque se recomienda la extracción endoscópica de tumores menores de 1 cm sin localización periampular o evidencia de invasión de la capa de propia muscular, evaluada mediante histología o ecografía endoscópica.

ABSTRACT Background: duodenal carcinoid tumor is rare; it represents 2 % of gastrointestinal neuroendocrine tumors. Therefore, a case of this is presented, recently diagnosed at the Joaquín Albarrán Clinical Surgical Hospital in Havana. Objective: to report a case of carcinoid tumor of the duodenum and review the literature with emphasis on the histopathological diagnosis. Clinical case: 86-year-old male patient who died on the fifth day after admission. At autopsy, during the macroscopic examination of the intestines, a lesion was revealed at the level of the first portion of the duodenum which, due to its histopathology, corresponded to a carcinoid tumor of the duodenum. Conclusions: duodenal carcinoid is a rare tumor whose endoscopic or histological diagnosis is made incidentally in the vast majority of cases; it is generally associated with a benign progression, although endoscopic removal of tumors smaller than 1 cm without periampullary location or evidence of invasion of the muscular layer is recommended, evaluated by histology or endoscopic ultrasound.

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.

Tumor carcinoide atípico solitario de timo: una entidad inusual que no debemos olvidar / Solitary atipic carcinoid tumor of the thymus: an unusual entity that we should not forget

Resumen: Los tumores neuroendocrinos primarios (NET) del mediastino son muy raros. Presentamos el caso de un tumor carcinoide atípico primario de timo. Un hombre de 52 años que fue a un examen médico porque se quejaba de tos no productiva sin hemoptisis. Se solicitó una radiografía de tórax donde se evidenció un mediastino notablemente ensanchado, con una tomografía de tórax que mostro una masa tumoral de 90 x 50 mm en el mediastino anterosuperior que comprime el arco aórtico y la arteria pulmonar sin un plano de clivaje adecuado. Debido a que el tumor era infiltrativo, se proporcionó escisión quirúrgica completa, quimioterapia y radioterapia al mediastino. El objetivo principal de nuestro estudio fue evaluar los hallazgos de CT y MRI con relación a la literatura mundial.

Abstract: Primary neuroendocrine tumors (NET) of the mediastinum are very rare. We present the case of a primary atypical carcinoid tumor of the thymus. A 52-year-old man who went to a medical examination because he complained of a non-productive cough without hemoptysis. A chest radiograph was requested where a markedly widened mediastinum was evidenced, with a thoracic tomography showing a tumor mass of 90 x 50 mm in the anterosuperior mediastinum that compresses the aortic arch and pulmonary artery without an adequate cleavage plane. Because the tumor was infiltrative, complete surgical excision, chemotherapy and radiotherapy were provided to the mediastinum. The main objective of our study was to evaluate the findings of CT and MRI in relation to universal literature.

Tumor carcinoide primario de ovario. Reporte de caso / Primary ovarian carcinoid tumor. Case report / Primary ovarian carcinoid tumor. Case report

RESUMEN Los tumores neuroendocrinos primarios del ovario son raros y consisten en un grupo de neoplasias heterogéneas que expresan marcadores inmunohistoquímicos similares. Los tumores carcinoides son las neoplasias neuroendocrinas más comunes, la mayoría surgen en el tracto gastrointestinal y broncopulmonar. Los tumores carcinoides primarios del ovario son entidades raras que representan aproximadamente 0,3% de todos los tumores carcinoides y menos del 0,1% de todas las neoplasias ováricas, con buen pronóstico y generalmente limitados al parénquima ovárico. Estos tumores surgen del sistema de células neuroendocrinas del estroma, epitelio superficial y teratomas ováricos. Las manifestaciones clínicas, en la mayoría de los casos, están asociadas con liberación de sustancias vasoactivas que causan síntomas, como enrojecimiento cutáneo, diarrea y broncoespasmo. Para realizar el diagnóstico es necesario el uso de imágenes radiológicas multimodales y análisis bioquímicos de marcadores tumorales neuroendocrinos. El tratamiento de primera línea es la resección del tumor siempre que sea posible. El pronóstico es generalmente favorable, excepto en algunos casos con metástasis. Se presenta un caso de tumor carcinoide primario de ovario.

ABSTRACT Primary neuroendocrine tumors are rare. They belong to a group of heterogeneous neoplasms that express similar immunohistochemical markers. Carcinoid tumors are the most common neuroendocrine neoplasms. Most of them arise in the gastrointestinal and bronchopulmonary tract. Primary carcinoid tumors of the ovary are rare entities that represent approximately 0.3% of all carcinoid tumors and less than 0.1% of all ovarian neoplasms, with good prognosis and generally limited to the ovarian parenchyma. These tumors arise from the ovarian stromal neuroendocrine cell system, superficial epithelium, and teratomas. In most cases, clinical manifestations are associated with the release of vasoactive substances that cause symptoms such as skin redness, diarrhea, and bronchospasm. For diagnosis it is necessary to use multimodal radiological images and biochemical analysis of neuroendocrine tumor markers. First-line treatment is tumor resection whenever possible. Prognosis is generally favorable, except in some cases with metastases. A case of primary ovarian carcinoid tumor is presented.

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.

The role of conventional echoendoscopy (eus) in therapeutic decisions in patients with neuroendocrine gastrointestinal tumors / O papel da ecoendoscopia convencional nas decisões terapêuticas em pacientes com tumores neuroendócrinos gastrointestinais

ABSTRACT Background: Gastrointestinal neuroendocrine tumors are rare, usually presented as subepithelial or polypoid tumors. Accurate diagnosis and indication of the type of resection are still challenging. Aim: To determine the effectiveness of echoendoscopy in determining the depth of the lesions (T) identified by endoscopy in order to evaluate surgical and/or endoscopic indication, and to evaluate the results of endoscopic removal in the medium term. Methods: Twenty-seven patients were included, all of whom underwent echoendoscopy for TN tumor staging and the evaluation of possible endoscopic resection. The parameters were: lesion size, origin layer, depth of involvement and identified perilesional adenopathies. The inclusion criteria for endoscopic resection were: 1) high surgical risk; 2) those with NET <2 cm; 3) absence of impairment of the muscle itself; and 4) absence of perilesional adenopathies in echoendoscopy and in others without distant metastases. Exclusion criteria were TNE> 2 cm; those with infiltration of the muscle itself; with perilesional adenopathies and distant metastases. The techniques used were: resection with polypectomy loop; mucosectomy with saline injection; and mucosectomy after ligation with an elastic band. The anatomopathological study of the specimens included evaluation of the margins and immunohistochemistry (chromogranin, synaptophysin and Ki 67) to characterize the tumor. Follow-up was done at 1, 6 and 12 months. Results: Resections with polypectomy loop were performed in 15 patients; mucosectomy in five; mucosectomy and ligation with elastic band in three and the remaining four were referred for surgery. The anatomopathological specimens and immunohistochemical analyzes showed positive chromogranin and synaptophysin, while Ki 67 was less than 5% among all cases. The medium-term follow-up revealed three recurrences. The average size of tumors in the stomach was 7.6 mm and in the duodenum 7.2 mm. Well-demarcated, hypoechoic, homogeneous lesions occurred in 75%; mucous layer in 80%; and the deep and submucosal mucosa in 70%. Conclusions: Echoendoscopy proved to be a good method for the study of subepithelial lesions, being able to identify the layer affected by the neoplasm, degree of invasion, echogenicity, heterogeneity, size of the lesion and perilesional lymph node involvement and better indicate the treatment option.

RESUMO Racional: Tumores neuroendócrinos gastrointestinais são raros geralmente apresentados como tumores subepiteliais ou polipoides. O diagnóstico preciso e a indicação do tipo de ressecção ainda são desafiadores. Objetivo: Determinar a eficácia da ecoendoscopia em determinar a profundidade das lesões (T) identificadas pela endoscopia com objetivo de avaliar indicação cirúrgica e/ou endoscópica, e avaliar os resultados da remoção endoscópica em seguimento em médio prazo. Métodos: Foram incluídos 27 pacientes todos submetidos à ecoendoscopia para estadiamento tumoral TN e à avaliação de possível ressecção endoscópica. Os parâmetros estudados foram: tamanho da lesão, camada de origem, profundidade do acometimento e adenopatias perilesionais identificadas. Os critérios de inclusão para ressecção endoscópica foram: 1) risco cirúrgico elevado; 2) aqueles com TNE <2 cm; 3) ausência de comprometimento da muscular própria; e 4) ausência de adenopatias perilesionais na ecoendoscopia e em outros sem metástases à distância. Os critérios de exclusão foram TNE >2 cm; os com infiltração da muscular própria; com adenopatias perilesionais e metástases à distância. As técnicas utilizadas foram: ressecção com alça de polipectomia; mucosectomia com injeção de solução salina; e mucosectomia após a ligadura com banda elástica. O estudo anatomopatológico dos espécimes incluiu avaliação das margens e imunoistoquímica (cromogranina, sinaptofisina e Ki 67) para caracterizar o tumor. O seguimento foi feito com 1, 6 e 12 meses. Resultados: Ressecções com alça de polipectomia foram realizadas em 15 pacientes; mucosectomia em cinco; mucosectomia e ligadura com banda elástica em três e os quatro restantes foram encaminhados para cirurgia. O anatomopatológico dos espécimes e as análises imunoistoquímicas mostraram cromogranina e sinaptofisina positivas, enquanto que o Ki 67 foi menor que 5% dentre todos os casos. O seguimento em médio prazo revelou três recidivas. A média de tamanho dos tumores no estômago foi de 7,6 mm e no duodeno 7,2 mm. As lesões bem demarcadas, hipoecóicas, homogêneas ocorreram em 75%; da camada mucosa em 80%; e da mucosa profunda e submucosa em 70%. Conclusões: A ecoendoscopia mostrou ser bom método para o estudo de lesões subepiteliais podendo identificar a camada acometida pela neoplasia, grau de invasão, ecogeneicidade, heterogeneidade, tamanho da lesão e acometimento linfonodal perilesional e melhor indicar a opção de tratamento.