Divertículo de Meckel con tumor neuroendocrino: presentación inusual / Meckel's diverticulum with neuroendocrine tumor: unusual presentation

El divertículo de Meckel es una malformación congénita que suele presentarse como un hallazgo incidental asintomático. Puede complicarse por procesos inflamatorios o tumores, cursando con sintomatología abdominal sumamente inespecífica, lo que complica su diagnóstico oportuno. Aunque la incidencia de neoplasias malignas en estos divertículos es baja, los tumores neuroendocrinos son los más representativos. Presentamos el caso de una paciente de 72 años que consultó por dolor abdominal y deposiciones melénicas, con múltiples nódulos intrahepáticos sugestivos de tumores neuroendocrinos y hallazgo intraoperatorio incidental de diverticulitis aguda de Meckel con metástasis peridiverticular de un tumor neuroendocrino. (AU)

Meckel's diverticulum is a congenital malformation that usually presents as an incidental finding. It can be complicated by inflammatory processes or tumors, with non-specific abdominal symptoms which delay its timely diagnosis. Although the incidence of malignant neoplasms in these diver-ticula is low, neuroendocrine tumors are the most representative. We present the case of a 72-year-old female patient who consulted for abdominal pain and melenic bowel movements, with multiple intrahepatic nodules suggestive of neuroendocrine tumors and an incidental intraoperative finding of acute Meckel's diverticulitis with peridiverticular metastasis of a neuroendocrine tumor. (AU)

Liver transplant for metastatic neuroendocrine tumors: a single-center report / Transplante de fígado para tumores neuroendócrinos metastáticos: série de casos

ABSTRACT BACKGROUND: Neuroendocrine tumors are rare neoplasms of uncertain biological behavior. The liver is one of the most common sites of metastases, occurring in 50% of patients with metastatic disease. AIMS: To analyze a clinical series in liver transplant of patients with neuroendocrine tumors metastases. METHODS: A retrospective descriptive study, based on the review of medical records of patients undergoing liver transplants due to neuroendocrine tumor metastases in a single center in northeast Brazil, over a period of 20 years (January 2001 to December 2021). RESULTS: During the analyzed period, 2,000 liver transplants were performed, of which 11 were indicated for liver metastases caused by neuroendocrine tumors. The mean age at diagnosis was 45.09±14.36 years (26-66 years) and 72.7% of cases were females. The most common primary tumor site was in the gastrointestinal tract in 64% of cases. Even after detailed investigation, three patients had no primary tumor site identified (27%). Overall survival after transplantation at one month was 90%, at one year was 70%, and five year, 45.4%. Disease-free survival rate was 72.7% at one year and 36.3% at five years. CONCLUSIONS: Liver transplantation is a treatment modality with good overall survival and disease-free survival results in selected patients with unresectable liver metastases from neuroendocrine tumors. However, a rigorous selection of patients is necessary to obtain better results and the ideal time for transplant indication is still a controversial topic in the literature.

RESUMO RACIONAL: Os tumores neuroendócrinos são neoplasias raras de comportamento biológico incerto. O fígado é um local comum de metástase, ocorrendo em 50% dos pacientes com doença metastática. OBJETIVOS: Analisar casuística de transplante hepático por metástases de tumores neuroendócrinos. MÉTODOS: Estudo descritivo retrospectivo com revisão de prontuários de pacientes submetidos a transplante hepático por metástases de tumores neuroendócrinos em um único centro no Nordeste do Brasil durante 20 anos (janeiro de 2001 a dezembro de 2021). RESULTADOS: Durante o período analisado, foram realizados 2.000 transplantes hepático, sendo 11 indicados por metástases hepáticas de tumores neuroendócrinos. A média de idade ao diagnóstico foi de 45,09±14,36 anos (26-66 anos) e 72,7% dos casos eram do sexo feminino. O local do tumor primário mais comum foi o trato gastrointestinal (64% dos casos). Após detalhada investigação, três pacientes não tiveram o local do tumor primário identificado (27%). A sobrevida global um mês e após um ano do transplante foi de 90 e 70%, respectivamente. A sobrevida após 5 anos foi de 45,4%. A taxa de sobrevida livre de doença foi de 72,7% no primeiro ano e 36,3% em cinco anos. CONCLUSÕES: O transplante hepático é uma modalidade de tratamento com bons resultados de sobrevida global e sobrevida livre de doença, em pacientes selecionados com metástases hepáticas irressecáveis de tumores neuroendócrinos. No entanto, a seleção rigorosa dos pacientes é necessária para obter melhores resultados e o momento ideal para a indicação do transplante ainda é um tema controverso na literatura.

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.

Seguimento de longo prazo de pacientes com tumor neuroendócrino da papila duodenal submetidos à papilectomia endoscópica: série de casos e revisão da literatura / Long-term follow-up of patients undergoing endoscopic papillectomy for papillary neuroendocrine tumor: a case series and literature review

ABSTRACT BACKGROUND: Duodenal papilla neuroendocrine tumors (DP-NET) are rare. Surgical therapy may be recommended for their treatment. However, they have high rates of morbidity and mortality. Endoscopic papillectomy (EP) is safe and effective for complete resection of tumors at this site. OBJECTIVE: This study aimed to describe a case series of DP-NETs resected by EP and perform a literature review. METHODS: A series of patients with DP-NETs underwent EP as primary treatment between Jan/2008 and Mar/2020 at a tertiary referral center. A comprehensive search was made on the MEDLINE primary electronic database. The search strategy was designed to find all articles related to DP-NETs published in the literature. RESULTS: Six patients underwent EP for presenting DP-NETs, four of whom were women (mean age, 63 years). The mean diameter of DP-NETs was 1.6±0.3 cm. Four of six patients were followed up, one of whom suffered relapse at the resection site after 3 months and was referred to surgery (pT3n1b) and the remaining three patients experienced no endoscopic or histological recurrence during follow-up periods of 10, 7, and 4 years, respectively. Eighteen articles were found in the literature search in MEDLINE. The articles included case reports of endoscopically treated DP-NETs. CONCLUSION: EP is safe and effective for DP-NETs that are ≤20 mm, confined to the submucosal layer, well-differentiated, and without local or remote metastasis. Adequate endoscopic follow-up and definitive surgical treatment in the presence of relapse are necessary.

RESUMO CONTEXTO: Tumores neuroendócrinos da papila duodenal (TNE-PD) são raros. A cirurgia deve ser recomendada para o tratamento. No entanto, apresentam altas taxas de morbimortalidade. A papilectomia endoscópica (PE) é segura e eficaz para a ressecção completa de tumores nesta região. OBJETIVO: Descrever uma série de casos de TNEs-PD ressecados por PE e realizar uma revisão da literatura. MÉTODOS: Pacientes com TNEs-PD submetidos a PE como tratamento primário com intenção curativa entre jan/2008 e mar/2020 em um centro de referência terciário foram estudados. Uma pesquisa abrangente foi feita no MEDLINE. A estratégia de busca foi desenhada para encontrar todos os artigos relacionados a TNEs-PD publicados na literatura, que haviam sido submetidos a PE. RESULTADOS: Seis pacientes foram submetidos a PE por apresentar TNEs-PD, sendo quatro mulheres, com média de idade de 66 anos (22-96). O diâmetro médio dos TNEs-PD foi de 1,8±0,4 cm. Quatro dos seis pacientes foram acompanhados, um dos quais sofreu recidiva no local da ressecção após 3 meses e foi encaminhado para cirurgia (pT3n1b), e os três pacientes restantes não apresentaram recorrência endoscópica ou histológica durante os períodos de acompanhamento de 10, 7 e 4 anos, respectivamente. Dezoito artigos foram encontrados no MEDLINE. Os artigos incluíram relatos de casos de TNEs-PD tratados pela PE. CONCLUSÃO: A PE é segura e eficaz para TNEs-PD ≤20 mm, confinados à camada submucosa, bem diferenciados e sem metástases locais ou remotas. Acompanhamento endoscópico adequado e tratamento cirúrgico definitivo na vigência de recidiva são necessários.

Ressecção endoscópica sob imersão d'água de pequenos tumores neuroendócrinos retais / Underwater endoscopic mucosal resection for small rectal neuroendocrine tumors

ABSTRACT BACKGROUND: A common site of neuroendocrine tumors (NETs) is the rectum. The technique most often used is endoscopic mucosal resection with saline injection. However, deep margins are often difficult to obtain because submucosal invasion is common. Underwater endoscopic mucosal resection (UEMR) is a technique in which the bowel lumen is filled with water rather than air, precluding the need for submucosal lifting. OBJECTIVE: This study aimed to evaluate the efficacy and safety of UEMR for removing small rectal neuroendocrine tumors (rNETs). METHODS: Retrospective study with patients who underwent UEMR in two centers. UEMR was performed using a standard colonoscope. No submucosal injection was performed. Board-certified pathologists conducted histopathologic assessment. RESULTS: UEMR for small rNET was performed on 11 patients (nine female) with a mean age of 55.8 years and 11 lesions (mean size 7 mm, range 3-12 mm). There were 9 (81%) patients with G1 rNET and two patients with G2, and all tumors invaded the submucosa with only one restricted to the mucosa. None case showed vascular or perineural invasion. All lesions were removed en bloc. Nine (81%) resections had free margins. Two patients had deep margin involvement; one had negative biopsies via endoscopic surveillance, and the other was lost to follow-up. No perforations or delayed bleeding occurred. CONCLUSION: UEMR appeared to be an effective and safe alternative for treatment of small rNETs without adverse events and with high en bloc and R0 resection rates. Further prospective studies are needed to compare available endoscopic interventions and to elucidate the most appropriate endoscopic technique for resection of rNETs.

RESUMO CONTEXTO: Um local comum de tumores neuroendócrinos (TNEs) é o reto. A técnica mais utilizada é a ressecção endoscópica da mucosa com injeção de solução salina. No entanto, as margens profundas costumam ser difíceis de ressecar porque a invasão da submucosa é comum. A ressecção endoscópica sob imersão d'água (RESI) é uma técnica em que o lúmen intestinal é preenchido com água em vez de ar, evitando a necessidade de elevação submucosa. OBJETIVO: Este estudo teve como objetivo avaliar a eficácia e segurança da RESI para a remoção de pequenos TNEs retais (rTNEs). MÉTODOS: Estudo retrospectivo com pacientes que realizaram RESI em dois centros. RESI foi realizada usando um colonoscópio padrão. Nenhuma injeção submucosa foi realizada. Patologistas certificados conduziram avaliação histopatológica. RESULTADOS: RESI foi realizada para pequenos rTNEs em 11 pacientes (nove mulheres) com média de idade de 55,8 anos e 11 lesões (tamanho médio de 7 mm, variando de 3-12 mm). Havia 9 (81%) pacientes com G1 rTNEs e dois pacientes com G2, sendo que todos os tumores invadiam a submucosa sendo apenas um restrito a mucosa. Nenhum caso mostrou invasão vascular ou perineural. Todas as lesões foram removidas em bloco. Nove (81%) ressecções tiveram margens livres. Dois pacientes tiveram envolvimento de margens profundas; um teve biópsias negativas por meio de vigilância endoscópica e o outro perdeu o acompanhamento. Não ocorreram perfurações ou sangramento tardios. CONCLUSÃO: A RESI parece ser uma alternativa eficaz e segura para o tratamento de pequenos rTNEs sem eventos adversos e com altas taxas de ressecção em bloco e R0. Mais estudos prospectivos são necessários para comparar as intervenções endoscópicas disponíveis e para elucidar a técnica endoscópica mais adequada para ressecção de rTNEs.

Surgical treatment of gastric neuroendocrine neoplasms / 中华胃肠外科杂志

Neuroendocrine neoplasms (NEN) are rare neoplasms originating from all major systems, in which gastric neuroendocrine neoplasms (G-NEN) is rarely malignant neoplasm originated in stomach. In 2019, the 5th WHO classification of digestive system tumors updated the classification of G-NEN and solved several naming problems. Since the classification of G-NEN has become more specific and more scientific, the surgical treatment of G-NEN is becoming more individual and more precise. Generally, endoscopic resection is often recommended for the treatment of type I gastric neuroendocrine tumors (NET). Type II gastric NET is mostly secondary to gastrinoma originating from the duodenum or pancreas, and thus surgical treatment of primary gastrinoma deserves enough attention. The decision of operation for type III gastric NET needs comprehensive consideration of tumor size, invasive depth and lymph node metastasis. For gastric neuroendocrine carcinomas without distant metastasis, aggressive surgery should be performed, and the resection range of primary site and lymph nodes can refer to the standard of gastric adenocarcinoma. For locally advanced gastric NEC, it has not been reported whether neoadjuvant chemotherapy or neoadjuvant chemoradiotherapy could reduce tumor stage and improve radical resection rate. In addition, for functional gastric NEN with distant metastasis, radical resection or palliative surgery can be performed to control hormone secretion and may improve the survival. In general, it is an important principle to thoroughly consider biological behavior, extent of primary and metastatic sites, resectability and function of tumor before surgery of gastric neuroendocrine neoplasm, and thus multi-disciplinary treatment (MDT) is recommended.

Endoscopic diagnosis and treatment of gastrointestinal neuroendocrine neoplasms / 中华胃肠外科杂志

The incidence of neuroendocrine neoplasms (NEN) is increasing globally, and gastrointestinal NEN (GI-NEN) is the most common type of NEN. Diagnosis and treatment of GI-NEN are quite different, according to tumor's location, size, background, cell origin, and pathogenesis. Digestive endoscopy has unique advantages in detecting of GI-NEN. However, endoscopist should not perform endoscopic resection arbitrarily, due to the high heterogeneity and complexity of GI-NEN. We need to establish the concept about comprehensive assessment for GI-NEN, including medical history and physical signs, serology, imaging, radionuclide and end·oscopic examination, to make an individualized treatment after rigorous multidisciplinary discussion.

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.

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.

Pancreatic neuroendocrine tumors: surgical resection / Tumores neuroendócrinos ressecáveis do pâncreas: abordagem cirúrgica

ABSTRACT Introduction: Pancreatic neuroendocrine tumors (pNET) correspond to about 3% of all tumors in pancreas and could be presented as a difficult diagnosis and management. Objective: To review the diagnosis and treatment of the pNET available in scientific literature. Method: A bibliographic survey was performed by means of an online survey of MeSH terms in the Pubmed database. A total of 104 articles were published in the last 15 years, of which 23 were selected as the basis for the writing of this article. Results: pNET is an infrequent neoplasia and their incidence, in USA, is about 1:100.000 inhabitants/year. Thereabout 30% of them produce hormones presenting as a symptomatic disease and others 70% of the cases could be silent disease. Magnetic Resonance Imaging (MRI) and/or Computed Tomography (CT) have similar sensitivy to detect pNET. They are very important when associated to nuclear medicine mainly Positron Emission Tomography (PET-CT) Gallium-68 to find primary tumor and its staging. The appropriate treatment should be chosen based on characteristics of the tumor, its staging and associated comorbidities. Conclusion: The surgical resection is still the best treatment for patients with ressectable pancreatic NETs. However, the size, grade, tumor functionality, stage and association with multiple endocrine neoplasia type 1 (MEN-1) are important to define who will be eligible for surgical treatment. In general, tumors bigger than 2 cm are eligible for surgical treatment, except insulinomas whose surgical resection is recommended no matter the size.

RESUMO Introdução: Os tumores neuroendócrinos pancreáticos (TNE-P) correspondem a cerca de 3% de todas as neoplasias no pâncreas e são de diagnóstico e manejo difíceis. Objetivo: Revisar o diagnóstico e tratamento desses tumores disponíveis na literatura científica atual. Método: Foi realizado levantamento bibliográfico por meio de pesquisa online de MeSH terms na base de dados do Pubmed. Ao todo foram analisados 104 artigos publicados nos últimos 15 anos, dos quais 23 foram selecionados como base para a redação deste artigo. Resultados: TNE-P é neoplasia pouco frequente com incidência estimada em 1: 100.000 habitantes/ano. Cerca de 30% produzem hormônios, ditos funcionantes, que se apresentam como doença sintomática e outros 70% podem se apresentar como doença silenciosa. Como recurso diagnóstico o uso de tomografia computadorizada ou ressonância nuclear magnética apresentam níveis de sensibilidade parecidas. Sua associação à recursos da medicina nuclear são de grande valia para localização de tumores primários e estadiamento, destacando-se o uso da tomografia por emissão de pósitrons (PET-CT) com Gálio-68. A decisão terapêutica deve ser baseada nas características tumorais, estadiamento e comorbidades associadas. Conclusão: A abordagem cirúrgica permanece como a terapia de escolha para pacientes com TNE-P ressecáveis. No entanto, a seleção de pacientes para tratamento cirúrgico deve seguir critérios baseados em funcionalidade do tumor, grau, estágio e associação com neoplasia múltipla tipo 1. De forma geral, tumores maiores de 2 cm têm indicação cirúrgica, excetuando-se os insulinomas, cuja proposta cirúrgica independe de seu tamanho.

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.

Tumores neuroendocrinos primarios de colon y recto / Primary neuroendocrine tumors of the colon and rectum

Resumen Introducción La clasificación y el manejo de los tumores neuroendocrinos (TNEs) han cambiado drásticamente en la última década. Se realiza un análisis crítico del manejo de los TENs colorrectales primarios intervenidos en nuestro centro a la luz de la nueva clasificación. Material y Método Análisis retrospectivo de los resultados del tratamiento quirúrgico radical de este tipo de lesiones en un período de 15 años. Resultados Se trata de 10 pacientes, con un promedio de 56 años (extremos 48-76), 6 ubicados cercanos a la válvula ileocecal (VIC) que se presentaron con un cuadro de obstrucción intestinal incompleto y tres en el recto (2 pólipos) y un pólipo en colon sigmoides. La cirugía efectuada incluye cuatro resecciones anteriores y seis colectomías derechas (con resección de una metástasis hepática en 1 caso). La mediana de seguimiento fue de 78,3 meses (extremos 8-180), durante el cual dos pacientes fallecen por metástasis a distancia. Conclusión Los TNEs ubicados en el colon habitualmente se presentaron como grandes masas tumorales cercanos a la VIC, lo que proporcionalmente no ocurre con las lesiones del rectosigmoides que son diagnosticados más precozmente como pólipos o lesiones submucosas. Algunos tumores de bajo grado muestran un compromiso avanzado en la pared y/o los linfonodos regionales lo que justifica la resección radical y/o la quimioterapia adyuvante. A la luz de esta experiencia, es necesario complementar el grado del tumor OMS 2010 (Ki-67 y número de mitosis) con el estadio TNM para caracterizar adecuadamente los TNEs, lo que influye en el manejo multidisciplinario.

Background The nomenclature and staging classification of neuroendocrine tumors (NETs) has changed drastically in the past decade. Objective To do a critical analysis of management of colorectal NETs in our institution in the light of the new classification. Methods We retrospectively reviewed the records of consecutive patients operated on with radical intention due to a colorectal NET in the last 15 years. Results There were 10 patients, median age was 56 years (range 48-76), six of them located near the ileocecal valve, three in the rectum (2 of them polyps) and one polyp in the sigmoid colon. Surgical procedure included four anterior resections and six right colectomy (one with hepatic resection). The median follow up was 78.3 months (range 8-180). Two patients died due to metastatic disease. Conclusion NETs located near de ileocecal valve were diagnosed usually as a big tumor with obstructing symptoms, while NETs of the rectum and sigmoid colon more frequently were detected as polyps or submucosal lesions. Some low grade TENs may invade the colonic wall and/or have metastasis in the regional lymph nodes and those cases need radical resection and/or adjuvant therapy. Combine the grade (Ki-67 and number and/or number of mitosis) of 2010 WHO classification with TNM showed prognostic value for classification and staging colorectal NETs with important therapeutic implications.

Tumores neuroendócrinos quísticos no funcionantes de páncreas. a propósito de un caso / Non-fuctional cystic neuroendocrine tumors of pancreas. a case report

Constituyen un grupo heterogéneo de tumores que pueden originarse virtualmente en todos los órganos; su etiopatogenia, diagnóstico y tratamiento aún generan discusiones, aunque se sabe que todos tienen algo en común: células neuroendócrinas como origen de los tumores, con similitud bioquímica e histológica con las células de la cresta neural. Se presenta el caso de una paciente, que fue operada en nuestro servicio y cuyo diagnóstico final fue tumor neuroendocrino quístico no funcionante de agresividad intermedia.

They constitute a heterogeneous group of tumors that can originate in virtually all organs; Its etiopathogenesis, diagnosis and treatment still generate arguments, although it is known that they all have something in common: neuroendocrine cells as the origin of tumors, with biochemical and histological similarity with neural crest cells. We present the case of a patient, who was operated on in our service and whose final diagnosis was non-functioning cystic neuroendocrine tumor of intermediate aggressiveness.

Laparoscopic uncinatectomy: a more conservative approach to the uncinate process of the pancreas / Uncinectomia laparoscópica: uma operação mais conservadora que pode ser a melhor opção para acesso ao processo uncinado do pâncreas

ABSTRACT Background: The isolate resection of the uncinate process of the pancreas is a rarely described procedure but is an adequate surgery to treat benign and low grade malignancies of the uncinate process of the pancreas. Aim: To detail laparoscopic uncinatectomy technique and present the initial results. Method: Patient is placed in supine position with the surgeon between legs. Three 5-mm, one 10-mm and one 12-mm trocars were used to perform the isolated resection of the uncinate process of the pancreas. Parenchymal transection is performed with harmonic scalpel. A hemostatic absorbable tissue is deployed over the area previously occupied by the uncinate process. A Waterman drain is placed. Result: This procedure was applied to an asymptomatic 62-year-old male with biopsy proven low grade neuroendocrine tumor of the pancreatic uncinate process. A laparoscopic pancreaticoduodenectomy was proposed. During the initial surgical evaluation, intraoperative sonography was performed and disclosed that the lesion was a few millimeters away from the Wirsung. The option was to perform a laparoscopic uncinatectomy. Postoperative period until full recovery was swift and uneventful. Conclusion: Laparoscopic uncinatectomy is a safe and efficient procedure when performed by surgical teams with large experience in minimally invasive biliopancreatic procedures.

RESUMO Racional: A ressecção isolada do processo uncinado do pâncreas é procedimento raramente relatado na literatura, mas consiste em operação adequada para o tratamento de tumores benignos e malignidades não-invasivas de baixo grau do processo uncinado do pâncreas. Objetivo: Detalhar a técnica da uncinectomia laparoscópica e apresentar os resultados iniciais. Método: Paciente é colocado em posição supina com o cirurgião entre as pernas. São utilizados três trocárteres de 5 mm, um de 10 mm e um de 12 mm para realizar a ressecção isolada do processo uncinado. A transecção parenquimatosa é realizada com bisturi harmônico. Tecido absorvível hemostático é implantado sobre a área anteriormente ocupada pelo processo uncinado. Dreno de Waterman é colocado sobre a área de superfície cruenta pancreática. Resultado: Este procedimento foi utilizado em um caso de tumor neuroendócrino de processo uncinado de pâncreas de baixo grau comprovado por biópsia guiada por ultrassonografia endoscópica. Ultrassonografia intra-operatória evidenciou lesão distando alguns milímetros do ducto de Wirsung, e assim optou-se pela realização de uncinectomia laparoscópica. A evolução pós-operatória até recuperação completa foi rápida e sem intercorrências. Conclusão: A uncinectomia por via laparoscópica é procedimento eficaz e seguro em equipes com experiência em cirurgia biliopancreática minimamente invasiva.

Tumor neuroendocrino primario de mama en un hombre / Primary neuroendocrine breast tumor found in a man

El cáncer de mama en hombres es una entidad poco frecuente y muy poco estudiada. Su pronóstico y manejo aun distan de lo ideal y de lo que se ha logrado en cáncer mamario en mujeres. Los tumores neuroendocrinos de la mama son aún más raros. Su comportamiento tiende en la mayoría de los casos a ser incierto y su manejo controversial. El reporte de caso se trata de un paciente masculino de 67 años, con historia de aparición de masa en mama derecha, con diagnóstico inicial de tumor mal diferenciado, con posterior inmunohistoquimica que informa tumor neuroendocrino primario de la mama. El diagnóstico temprano de cáncer de mama en hombres implica un verdadero reto para los sistemas de salud. Debemos conocer más sobre su fisiopatología y factores de riesgo(AU)

The early diagnosis of breast cancer in men represents a real challenge for the health systems. Therefore, our knowledge about its physiopathology and risk factors must be expandedBreast cancer in men is a rare and poorly studied malady. The prognosis and management is far from being ideal and from the achievements of the female breast cancer treatment. Even rare are neuroendocine breast tumors. In most of cases, its behavior is uncertain and its management is controversial. This was the report of a male patient aged 67 years, with history of right breast mass, initially diagnosed as a poorly differentiated tumor with later immunohistochemical test that reported the existence of a primary neuroendocrine breast tumor(AU)

Somatostatinoma duodenal / Duodenal somastotinoma

Alrededor del 70%de los tumores endocrinos bien diferenciados asientan en el tracto gastrointestinal. Los tumores duodenales bien diferenciados constituyen solo el 2,6 % de todos los tumores neuroendocrinos (NET).El somatostatinoma es un tumor raro que se localiza en páncreas o duodeno con una incidencia de 1:40 millones.La neurofibromatosis tipo I es una enfermedad autosómica dominante, la mutación en el gen supresor de tumores NF1 favorece la aparición de neoplasias en estos pacientes.

About 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated tumors account for only 2.6% of all neuroendocrine tumors. Somatostatinomas are rare neuroendocrine tumors (NETs) with an incidence of 1 in 40 million. These unusual tumors arise predominantly in the pancreas and peripancreatic duodenum. Neurofibromatosis type I is an autosomal dominant disease, the mutation in the tumor suppressor gene NF1 favors the appearance of neoplasms in these patients.

Tumor neuroendócrino de reto: relato de caso / Rectal neuroendocrine tumor: case report

Introdução e objetivo: o tumor neuroendócrino de reto é uma patologia rara que apresenta incidência de 0,86 por 100.000 habitantes. Cerca de 50% dos pacientes não apresenta sintomas específicos e sua lesão é semelhante a pólipos intestinais, sendo, a única opção de cura, a completa ressecção da lesão ainda localizada. O presente relato tem por objetivo apresentar uma neoplasia rara e ressaltar a importância de seu diagnóstico e ressecção precoces, sendo por muitas vezes diagnosticado apenas em colonoscopias de rotina ao acaso. Relato de caso: paciente de 40 anos, feminina, apresentando há três meses quadro de azia acompanhada de epigastralgia e dor abdominal difusa de fraca intensidade, sem outras comorbidades. Realizada endoscopia com hérnia hiatal e esofagite erosiva leve. A colonoscopia evidenciou lesão submucosa de 1 a 1,5 centímetros. Como tratamento foi realizado a mucosectomia e o laudo anatomopatológico de tumor neuroendócrino de reto grau 1. Paciente realizou exames laboratoriais e tomografia computadorizada de abdome, sendo normais, e colonoscopia de controle após três meses com completa cicatrização do local e sem novas lesões. Conclusão: quanto mais precoce é o diagnóstico, melhor é o estadiamento da lesão, podendo ser feito o tratamento apenas com ressecção endoscópica, apresentando ótimo prognóstico. Deve-se conhecer as características e classificações deste tipo de tumor que prevalece no trato gastrointestinal para melhor abordagem diagnóstica e terapêutica do paciente.

Introduction and Aim: The rectal neuroendocrine tumor is a rare patology present in scale of 0,86 per 100.000 in habitants. About 50% of the cases do not present specific symptoms. The lesion looks like an intestinal polips. The only option of the guaranteed cure is the complete resection of the localized lesion. The present report has an objective to present one rare neoplasm and to highlight the importance of the diagnosis and the procosis resection. The tumor is diagnosis most of the times, only in a routine colonoscopy. Case report: 40-years-old female patient, presenting by 3 months heartburn, with epigastralgia and low intensity of diffuse abdominal pain, without others comorbidity. It was realized one endoscopy showing one hernia hiatal and low erosive esophagitis. The colonoscopy showed submucosal lesion with 1 to 1,5 centimeters. For the treatment it was accomplished a mucosectomy and a pathology report of a rectal neuroendocrine tumor with degree one. Patient realized laboratory exams and abdominal computerized tomography. The result was normal and control colonoscopy after 3 months with completed healing of the local and no new lesions. Conclusion: the more precosis the diagnosis better the staging of the lesion, it may be realized the treatment only with the endoscopy resection and presents good prognosis. Must know the characteristics and classifications about this type of tumor that prevails at the intestinal tract for the better approach diagnosis and therapeutic.

A propósito de un caso de paraganglioma / A propos of a case of paraganglioma

Los paragangliomas son tumores neuroendocrinos que surgen de los paraganglios autonómicos extraadrenales, los cuales son pequeños órganos formados por células derivadas de la cresta neural embrionaria con capacidad de secretar catecolaminas. Los paragangliomas están estrechamente relacionados con los feocromocitomas porque son indistinguibles a nivel celular, y a menudo comparten las mismas manifestaciones clínicas, como hipertensión, cefalea episódica, sudoración y taquicardia. El diagnóstico de estos tumores es importante por su riesgo de malignización, por las implicaciones de otras neoplasias asociadas, y para la posibilidad de realizar estudios genéticos para detección de otros casos dentro de una misma familia. El objetivo de este artículo es desarrollar un resumen sobre la epidemiología, manifestaciones clínicas, pruebas diagnósticas y tratamiento de estos tumores. Se presenta el caso de un joven de raza negra diagnosticado de un paraganglioma(AU)

Paragangliomas are neuroendocrine tumors emerging from the extra-adrenal autonomic paraganglia, which are small organs formed by embryonic neural crest-derived cells with catecholamine-secreting capacity. Paragangliomas are closely linked to pheochromocytomas because they cannot be differentiated at cell level and often share the same clinical manifestations such as hypertension, episodic headache, sweating and tachycardia. The diagnosis of these tumors is important because of risk of becoming malignant, the implications of other related neoplasias and the possibility of making genetic studies to detect other cases in the same family. The objective of this article was to make an abstract about epidemiology, clinical manifestations, diagnostic tests and treatment of these tumors. This is the case of a young Black female who was diagnosed with paraganglioma(AU)

Hipoglicemia hiperinsulínica: a propósito de un caso / Hyperinsulinemic hypoglycemia: report of a case

El diagnóstico de hipoglicemia se confirma con la triada de Whipple: síntomas de hipoglicemia, concentración plasmática baja de glucosa, y desaparición de los síntomas con la elevación de los niveles de glucosa. Se presenta el caso de una paciente de 26 años de edad, que consultó a nuestro servicio en julio de 2013 por episodios intermitentes que se iniciaron en octubre del año precedente, consistentes en visión borrosa, cambios conductuales y discurso incoherente, sin relación con la ingesta o el ayuno, que aumentaron en frecuencia los meses previos a la consulta y llegaron a asociarse a pérdida de conocimiento y relajación de esfínteres, requiriendo asistencia médica prehospitalaria. Ocasionalmente hubo registros bajos de glicemia capilar, y hubo mejoría sintomatológica con la administración de glucosa e.v. Durante una prueba de ayuno presentó una hipoglicemia sintomática con glicemia 48 mg/dl, insulinemia 21,1 µU/ml, péptido C 2.257 ng/ml. La ecografía de abdomen identificó una imagen sólida en la unión de cuerpo y cola del páncreas. La lesión fue enucleada quirúrgicamente, y la anatomía patológica confirmó su origen neuroendócrino .

The diagnosis of hypoglycemia is confirmed by Whipple’s triad: symptoms of hypoglycemia, low plasma glucose concentrations, and disappearance of symptoms after glucose administration. The case of a 26-year-old woman is presented. She consulted our Service in July 2013 due to intermittent episodes that had begun in October of the preceding year, characterized by blurred vision, slurred speech, and behavioral changes, unrelated to food ingestion. They increased in frequency, and were associated eventually with loss of consciousness and loss of sphincters control, requiring medical care. Occasionally, low capillary glucose levels were recorded, and there was improvement in symptoms with i.v. glucose administration ev.During a fasting test she had symptomatic hypoglycemia with serum glucose of 48 mg/dl, serum insulin 21.1 µU/ml, and serum C-peptide 2.257 ng/ml. Abdominal ultrasound identified a rounded image in the union of the body and tail of the páncreas; this was confirmed by MRI. The lesion was surgically enucleated, and the pathology report confirmed its neuroendocrine origin .