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.

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

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 (-)).

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 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.

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.

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.

Caracterización de pacientes con tumores neuroendocrinos en un hospital de referencia de alta complejidad / Characterization of patients with neuroendocrine tumors in a referral hospital

Introducción. Los tumores neuroendocrinos comprenden una amplia familia tumoral, siendo de mayor frecuencia aquellos localizados en el tracto gastrointestinal, los pulmones, el timo y el páncreas. Ocurren con poca frecuencia y cursan con un comportamiento biológico variable. El objetivo del presente estudio fue describir las características demográficas y clínicas de los pacientes con esta entidad en un hospital de alta complejidad. Métodos. Estudio descriptivo con revisión de historias clínicas correspondientes a pacientes con tumor neuroendocrino, cuya muestra histológica fue analizada en el Departamento de Patología del Hospital Pablo Tobón Uribe, Medellín, Colombia, entre 2010 y 2015. Se describen frecuencias de localización, grado de diferenciación y perfil de inmunohistoquímica. Resultados. Se revisaron 111 historias, de las que el 51,5 % correspondió a pacientes masculinos, con una mediana de edad al momento del diagnóstico de 48 años. Las comorbilidades más frecuentes fueron: hipertensión arterial (38,6 %), hipotiroidismo (15,8 %) y diabetes mellitus (11,9 %). El antecedente de neoplasia estuvo presente en el 13,9 % de los casos. Un 59,4 % de las muestras correspondieron a tumores primarios, el 34,8 % estaban localizados en el páncreas. El hígado fue el órgano más comprometido por metástasis (73,3 %). De los tumores neuroendocrinos, el 27 % correspondió a tumores grado 1 del tracto gastrointestinal o del páncreas, el 36 % a grado 2 y el 27,9 % a grado 3. El 3,6 % correspondió a tumores pulmonares de grado bajo o intermedio y el 5,4 % a tumores pulmonares de alto grado. Los marcadores tumorales con mayor positividad fueron sinaptofisina (97,2 %), citoqueratinas AE1/AE3 (95 %), CD56 (91,3 %) y cromogranina (87,8 %). Discusión. Los tumores neuroendocrinos tienen características clínicas e histopatológicas diversas. Conocer las características de los pacientes afectados permite una aproximación de la epidemiología local aplicable a futuras investigaciones

Introduction. Neuroendocrine tumors comprise a broad family of tumors, the most common being those located in the gastrointestinal tract, lungs, thymus, and pancreas. They occur infrequently and have a variable biological behavior. The objective of this study was to describe the demographic and clinical characteristics of patients with this entity in a highly complex hospital.Methods. Descriptive study with review of medical records corresponding to patients with neuroendocrine tu-mors, whose histological sample was analyzed in the Pathology Department of the Pablo Tobón Uribe Hospital in Medellín between 2010 and 2015. Frequencies of location, degree of differentiation and immune-histochemistry profile are described.Results. One hundred eleven histories were reviewed, of which 51.5% corresponded to male patients, with a median age at diagnosis of 48 years. The most frequent comorbidities were arterial hypertension (38.6%), hypothyroidism (15.8%) and diabetes mellitus (11.9%). Medical history of neoplasia was present in 13.9% of the cases. 59.4% of the samples corresponded to primary tumors, 34.8% were located in the pancreas. The liver was the organ most compromised by metastasis (73.3%). Of the neuroendocrine tumors, 27% corresponded to grade 1 tumors of the gastrointestinal tract or pancreas, 36% to grade 2 and 27.9% to grade 3; 3.6% corresponded to low or intermediate grade lung tumors and 5.4% to high-grade lung tumors. The tumor markers with the highest positivity were synaptophysin (97.2%), cytokeratins AE1/AE3 (95%), CD56 (91.3%) and chromogranin (87.8%).Discusion. Neuroendocrine tumors have diverse clinical and histopathologic features. Knowing the characteristics of affected patients allows an approximation of local epidemiology applicable to future research

Distribución Topográfica y Manejo de Tumores Neuroendocrinos del Tubo Digestivo / Topographic Distribution and Management of Neuroendocrine Tumors of the Digestive Tube

Introducción: Los tumores neuroendocrinos (TNE) son un grupo de neoplasias que se originan a partir de células enterocromafínicas, especialmente ubicadas en el tubo digestivo. El objetivo del presente estudio es escribir la distribución topográfica, el manejo multidisciplinario y diagnóstico patológico según la OMS de los tumores neuroendocrinos del tubo digestivo. Métodos: El presente es un estudio descriptivo, retrospectivo de los casos con diagnóstico anatomopatológico confirmado de tumor neuroendocrino localizados en el tubo digestivo entre enero del 2011 a diciembre del 2018 en el Instituto Oncológico Nacional del Ecuador- SOLCA de Guayaquil. Se describe topografía y tipo de tratamiento establecido con frecuencias y porcentajes. Resultados: Ingresaron al estudio 21 casos. La localización más frecuente fue el apéndice cecal n = 8 (38 %), en estómago n=4 (19 %), intestino delgado n=3 (14 %). El diagnóstico patológico en estadio G1 (65 %); G2 (24%) y G3 (12%). La primera línea de tratamiento fue la cirugía con intención curativa n=19 (90.5 %) y tratamiento endoscópico n=2 (9.5 %). Los pacientes sometidos a cirugía, n=16/19; (84 %) obtuvieron niveles de resección 0 (R0) y permanecieron en observación clínica, los demás sujetos en el estudio recibieron tratamiento adyuvante con somatostatina sola n=1 (4.8 %) o combinación de somatostatina y radioterapia n=2 (9.5 %). Conclusión: El diagnóstico de tumor neuroendocrino de tubo digestivo es una etiología oncológica poco frecuente. El tratamiento quirúrgico en esta serie de casos está enfocada en una acción curativa de tipo quirúrgico.

Introduction: Neuroendocrine tumors (NETs) are a group of neoplasms that originate from enterochromaffin cells, especially located in the digestive tract. The objective of the present study is to write the topographic distribution, the multidisciplinary management and pathological diagnosis according to the WHO of the neuroendocrine tumors of the digestive tract. Methods: This is a descriptive, retrospective study of the cases with a confirmed anatomopathological diagnosis of neuroendocrine tumor located in the digestive tract between January 2011 to December 2018 at the National Oncology Institute of Ecuador- SOLCA of Guayaquil. The topography and type of treatment established with frequencies and percentages are described. Results: 21 cases were entered into the study. The most frequent location was the cecal appendix n = 8 (38%), stomach n = 4 (19%), small intestine n = 3 (14%). The pathological diagnosis in stage G1 (65%); G2 (24%) and G3 (12%). The first line of treatment was surgery with curative intent n = 19 (90.5%) and endoscopic treatment n = 2 (9.5%). Patients undergoing surgery, n = 16/19; (84%) obtained resection levels 0 (R0) and remained under clinical observation, the other subjects in the study received adjuvant treatment with somatostatin alone n = 1 (4.8%) or combination of somatostatin and radiotherapy n = 2 (9.5%). Conclusion: The diagnosis of neuroendocrine tumor of the digestive tract is a rare oncological etiology. The surgical treatment in this series of cases is focused on a surgical action of a surgical type.

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

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

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

Bronchial carcinoid tumors: second primary neoplasms and outcomes of surgical treatment / Tumores carcinoides brônquicos: segunda neoplasia primária e desfechos do tratamento cirúrgico

ABSTRACT Objective: To analyze determinants of prognosis in patients with bronchial carcinoid tumors treated surgically and the potential concomitance of such tumors with second primary neoplasms. Methods: This was a retrospective analysis of 51 bronchial carcinoid tumors treated surgically between 2007 and 2016. Disease-free survival (DFS) was calculated by the Kaplan-Meier method, and determinants of prognosis were evaluated. Primary neoplasms that were concomitant with the bronchial carcinoid tumors were identified by reviewing patient charts. Results: The median age was 51.2 years, 58.8% of the patients were female, and 52.9% were asymptomatic. The most common histology was typical carcinoid (in 80.4%). Five-year DFS was 89.8%. Ki-67 expression was determined in 27 patients, and five-year DFS was better among the patients in whom Ki-67 expression was ≤ 5% than among those in whom it was > 5% (100% vs. 47.6%; p = 0.01). Concomitant primary neoplasms were observed in 14 (27.4%) of the 51 cases. Among the concomitant primary neoplasms that were malignant, the most common was lung adenocarcinoma, which was observed in 3 cases. Concomitant primary neoplasms were more common in patients who were asymptomatic and in those with small tumors. Conclusions: Surgical resection is the mainstay treatment of bronchopulmonary carcinoid tumors and confers a good prognosis. Bronchial carcinoid tumors are likely to be accompanied by second primary neoplasms.

RESUMO Objetivo: Analisar os determinantes do prognóstico em pacientes com tumores carcinoides brônquicos tratados cirurgicamente e possível segunda neoplasia primária concomitante. Métodos: Trata-se de uma análise retrospectiva de 51 tumores carcinoides brônquicos tratados cirurgicamente entre 2007 e 2016. A sobrevida livre de doença (SLD) foi calculada pelo método de Kaplan-Meier, e os determinantes do prognóstico foram avaliados. As neoplasias primárias concomitantes aos tumores carcinoides brônquicos foram identificadas por meio da análise dos prontuários dos pacientes. Resultados: A mediana de idade foi de 51,2 anos, 58,8% dos pacientes eram do sexo feminino e 52,9% eram assintomáticos. A classificação histológica mais comum foi carcinoide típico (em 80,4%). A SLD em cinco anos foi de 89,8%. A expressão de Ki-67 foi determinada em 27 pacientes, e a SLD em cinco anos foi melhor nos pacientes nos quais a expressão de Ki-67 foi ≤ 5% do que naqueles nos quais a expressão de Ki-67 foi > 5% (100% vs. 47,6%; p = 0,01). Neoplasias primárias concomitantes foram observadas em 14 (27,4%) dos 51 casos. Entre as neoplasias primárias malignas concomitantes, a mais comum foi o adenocarcinoma pulmonar, observado em 3 casos. Neoplasias primárias concomitantes foram mais comuns em pacientes assintomáticos e naqueles com tumores pequenos. Conclusões: A resseção cirúrgica é o principal tratamento de tumores carcinoides broncopulmonares e propicia um bom prognóstico. É provável que tumores carcinoides brônquicos se relacionem com segunda neoplasia primária.

Hallazgos por imagen de tumor carcinoide bronquial en hospital de la habana / Imaging findings of bronchial carcinoid tumor in haban-hospital

Se presenta un caso de adolescente sin comorbilidades, quien cursó durante tres meses con infecciones respiratorias a repetición sin mejoría a pesar de manejo médico apropiado. En imagen de control posterior a la hospitalización, se documenta en estudios de imagen lesión del lóbulo inferior derecho con crecimiento endoluminal y mediante broncoscopía se confirma la presencia de una masa tumoral en la luz del bronquio para dicho lóbulo. Se realiza biopsia y el estudio anatomopatológico reporta tumor carcinoide variedad típica. A pesar que es una entidad poco frecuente, el tumor carcinoide es una neoplasia neuroendócrina que se puede presentar en el sistema respiratorio en este grupo poblacional y se debe considerar en el contexto de infecciones respiratorias a repetición. Los estudios de imagen deberían ser parte de la evaluación de todo paciente que cursa con complicaciones de infecciones respiratorias y en el diagnóstico diferencial de otras entidades respiratorias.

It is present a case of teenager without comorbidities, who had recurrent respiratory infections during three months without improving besides proper medical treatment. Imaging post-hospitalization control documented in imaging studies a right lower lobe lesion with endobronchial grow and bronchoscopy confirmed a growing into the lumen tumoral mass of such lobular bronchi. A biopsy was performed and an pathological study reported carcinoid tumor typical type. Even though it is a rare entity, carcinoids tumors are neuroendocrine neoplasms that can be found in the respiratory system among this population group and it should be considered in a repetitive respiratory infections scene. Imaging studies should be part of every patient evaluation that course with complications as well as a tool for other entities differential diagnosis.

Tumor carcinoide del apéndice cecal en mujer adolescente: a propósito de un caso / Carcinoid Tumor of the Caecal Appendix in a Adolescent Woman: in Regards to a Case

Los carcinoides son tumores neuroendocrinos de baja incidencia en la población general. Los tumores carcinoides apendiculares pueden presentarse de manera asintomática, los cuales se descubren en las biopsias de las piezas operatorias de apendicectomías. En otros casos la manifestación habitual de estos tumores es el proceso inflamatorio agudo del apéndice cecal, razón por la cual el diagnóstico definitivo se conoce solo con el informe histopatológico. Se presenta el caso de tumor carcinoide del cuerpo apendicular diagnosticado en una adolescente de 15 años intervenida quirúrgicamente por sospecha de apendicitis aguda. El interés por presentar el caso radica en su aparición poco frecuente en la adolescencia así como su localización en cuerpo o tercio medio del órgano.

Carcinoids are neuro-endocrine tumors of low incidence in the general population. Appendix carcinoid tumors may present asymptomatically, which are discovered in biopsies of the surgical pieces of appendectomies. In other cases the usual manifestation of these tumors is the acute inflammatory process of the caecal appendix, so the concluding diagnosis is only known with the histo-pathological report. A 15 year old case of carcinoid tumor of the appendix body diagnosed in this adolescent who was performed a surgery due to suspicion of acute appendicitis. The interest of this case presentation is related to its infrequent presentation in adolescence so as its location in body or middle third medium of the organ

Manejo endoscópico de los tumores neuroendocrinos rectales: estudio multicéntrico en Perú / Endoscopic treatment of rectal neuroendocrine tumors: multicentre study in Peru

Introducción: La detección incidental de tumores neuroendocrinos (TNE) rectales se ha incrementado, pero todavía no hay consenso apropiado para su tratamiento. Objetivo: Evaluar la eficacia y seguridad del tratamiento endoscópico de los TNE rectales menores de 20 mm, con énfasis en el "tratamiento estandarizado". Material y métodos: De enero del 2012 a abril del 2016, se incluyeron pacientes con TNE rectales menores de 20 mm que se resecaron mediante mucosectomía convencional (RME), mucosectomía asistida con endoligadura (RME-L) y disección submucosa endoscópica (DSE). Se consideró como "tratamiento estandarizado" aquel con indicación precisa y rigurosidad técnica (RME-L para lesiones ≤ 10 mm y DSE para lesiones entre 11 y 20 mm). Se evaluaron las tasas de resección completa, así como la incidencia de complicaciones. Resultados: 23 pacientes con 23 TNE rectales fueron incluidos. La edad promedio fue 52,96 ± 12,44 años. El tamaño promedio fue de 8,8 ± 3,4mm. Las tasas de resección completa con RME, RME-L y DSE fueron 50 (3/6), 92,8 (13/14) y 100% (3/3), respectivamente; mientras que con el tratamiento estandarizado y convencional fueron 100 (14/14) y 55,5% (5/9), respectivamente. Las complicaciones ocurrieron en 4 casos (17,4%), sangrado en 2 y perforación en 2, todos resueltos por endoscopía. Conclusiones: El tratamiento endoscópico mediante RME-L y DSE es eficaz y seguro para la resección de TNE rectales menores de 20 mm. El tratamiento estandarizado potencializa la eficacia de la terapia endoscópica.

Introduction: The incidental detection of rectal neuroendocrine tumors (NET) has increased but there is no proper consensus about treatment. Objective: Evaluate the efficacy and safety of endoscopic treatment for rectal NET less than 20 mm, with emphasis in "standardized treatment". Material and methods: From January 2012 to April 2016, we included patients with rectal NET less than 20 mm resected by conventional EMR, EMR-B and ESD. We considered as "standardized treatment" the one that has precise indication and technical rigor (EMR-B for lesions ≤ 10 mm and ESD for lesions between 11 and 20 mm). We evaluate complete resection rates and incidence of complications. Results: 23 patients with 23 rectal NET were included. The mean age was 52.96 ± 12.44 years. The mean tumor diameter was 8.8 ± 3.4 mm. The complete resection rates by conventional EMR, EMR-Band ESD were 50 (3/6), 92.8 (13/14) and 100% (3/3), respectively; while by standardized and conventional treatment were 100 (14/14) and 55.5% (5/9), respectively. Complications occurred in 4 cases (17.4%), bleeding in 2 and perforation in 2, all of them solved by endoscopy. Conclusions: Endoscopic treatment by EMR-B and ESD is efficacious and safe for rectal NET ≤ 20 mm. Standardized treatment improve the efficacy of endoscopic treatment.