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.

Difference in survival between right- versus left-sided colorectal neuroendocrine neoplasms / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors that arise from neuroendocrine cells, and in some cases are capable of producing agents that may cause characteristic hormonal syndromes (Cives and Strosberg, 2018). Such tumors were previously thought to be rare, but the rate of detection of NENs, especially from the gastrointestinal tract, is increasing with the widespread use of colonoscopy, cross-sectional imaging, and biomarkers (Gu et al., 2019). A study based on the Surveillance, Epidemiology, and End Results (SEER) database showed that the age-adjusted incidence of NENs increased 6.4-fold from 1973 (1.09 per 100 000) to 2012 (6.98 per 100 000) (Dasari et al., 2017), while there was a progressive increase in the incidence of colorectal NENs (Starzyńska et al., 2017).

Primary neuroendocrine neoplasm of the esophagus – Report of 14 cases from a single institute and review of the literature / Neoplasia neuroendócrina primária de esôfago - Relato de 14 casos e revisão de literatura

ABSTRACT BACKGROUND Most prevalent esophageal neoplasm is squamous cell carcinoma and adenocarcinoma. Other tumors are uncommon and poorly studied. Primary neuroendocrine esophageal neoplasm is a rare carcinoma and most of its therapy management is based on lung neuroendocrine studies. Neuroendocrine tumors can be clustered in the following subtypes: high grade (small cell carcinoma or large cell carcinoma) and low grade (carcinoids). OBJECTIVE The present study aims to assess clinical and pathological neuroendocrine esophageal tumors in a single oncologic center. METHODS A retrospective analysis of patients and review of the literatures was performed. RESULTS Fourteen patients were identified as neuroendocrine tumors, 11 male and 3 female patients. Mean age was 67.3 years old. Ten patients were classified as small cell, 3 as large cell and 1 as carcinoid. Four patients presented squamous cell carcinoma simultaneously and 1 also presented adenocarcinoma. Main sites of metastasis were liver, peritoneum, lung and bones. Most patients died before 2 years of follow-up. Patient with longer survival died at 35 months after diagnosis. CONCLUSION Neuroendocrine esophageal tumors are rare; affect mainly men in their sixties or seventies. High grade tumors can be mixed to other subtypes neoplasms, such as adenocarcinoma and squamous cell carcinoma. Most of these patients have poor overall survival rates.

RESUMO CONTEXTO As neoplasias esofágicas mais prevalentes são o adenocarcinoma e o carcinoma espinocelular. Outros subtipos histológicos são incomuns e pouco estudados. Neoplasia neuroendócrina esofágica é uma patologia rara e seu manejo atualmente se baseia nos conhecimentos prévios de tumores neuroendócrinos de pulmão. Tumores neuroendócrinos podem ser divididos nas seguintes formas: alto grau (pequenas células ou grandes célula) e baixo grau (carcinoides). OBJETIVO Avaliar clínica e patologicamente os tumores de esôfago em um centro oncológico referenciado. MÉTODOS Foi realizada análise retrospectiva e revisão da literatura de neoplasias neuroendócrinas de esôfago. RESULTADOS Foram identificados 14 pacientes com tumores neuroendócrino, sendo 11 homens, 3 mulheres. Idade média foi de 67,3 anos de idade. Desses pacientes, 10 foram classificados como pequenas células, 3 como grandes células e 1 como carcinoide. Foram encontrados quatro casos de tumor misto neuroendócrino e carcinoma espinocelular, e um caso de tumor misto adenoneuroendócrino. Principal sítio de metástases foi fígado, peritônio, pulmão e ossos. A maioria dos pacientes foi a óbito em até 2 anos de seguimento. Paciente com sobrevida mais longa foi a óbito após 35 meses do diagnóstico. CONCLUSÃO Neoplasias neuroendócrinas de esôfago são raras, afetam principalmente o sexo masculino na 7ª ou 8ª década de vida. A maioria dos pacientes com tumores de alto grau tem sobrevida curta.

Tumores neuroendocrinos del páncreas: resultados quirúrgicos y sobrevida alejada / Pancreatic neuroendocrine tumors: surgical outcomes and overall survival

Background: pancreatic neuroendocrine tumors (PNET) account for 1-2percent of pancreatic neoplasms. Its incidence has increased in recent years probably due to improved imaging studies. Aim: to analyze the clinical characteristics, surgical outcomes and overall survival of patients with PNET who underwent resective surgery. Methods: case series study. Data was collected from the central database and clinical records from patients with biopsy-proven PNET's who underwent surgical resection from june 2005 to june 2012. Results: twenty patients were included (10.6 percent of all pancreatic resections), 12 female, with a median age of 44 [20-77] years. Abdominal pain was the most common symptom. Two patients had a type 1 multiple endocrine neoplasia (MEN-1) syndrome. Pre-operative work up included CT, MR and/or PET/CT. Five patients had functional tumors. Five Whipple procedures, 14 distal pancreatectomies and 1 enucleation were performed. Among the postoperative complications, there were 5 type B and 1 type C pancreatic fistulas. There was no mortality. At 31 [5-90] month median follow-up, overall survival was 100%. Conclusion: PNETs represent an increasing reason for pancreatic resection in our center. Surgical resection of the tumor with negative microscopic margins is the treatment of choice.

Introducción: los tumores neuroendocrinos del páncreas (TNEP) representan el 1-2 por ciento de las neoplasias pancreáticas. Su incidencia ha aumentado en los últimos años debido probablemente a la mayor capacidad diagnóstica de los estudios por imágenes. Objetivos: analizar las características clínicas, resultados quirúrgicos y sobrevida alejada de los pacientes con TNEP operados en nuestro centro. Material y Métodos: estudio descriptivo de una serie de casos. Análisis de base de datos y registros clínicos de los pacientes con diagnóstico histológico de TNEP sometidos a cirugía resectiva en nuestro centro entre junio de 2005 y junio de 2012. Resultados: serie compuesta por 20 pacientes (10,6 por ciento de las resecciones pancreáticas), 12 de sexo femenino, con una mediana de edad de 44 (20-77) años. El dolor abdominal fue el síntoma de presentación más frecuente. Dos pacientes eran portadores del síndrome de neoplasia endocrina múltiple tipo 1 (NEM-1). La evaluación diagnóstica se realizó con CT, RM y/o PET/CT. Los tumores fueron funcionantes en 5 enfermos. Se realizaron 5 pancreatoduodenectomías (PD), 14 pancreatectomías corporocaudales (PC) y 1 enucleación. Entre las complicaciones postoperatorias, hubo 5 fístulas pancreáticas tipo B y una tipo C. No hubo mortalidad. En el seguimiento (mediana de 31 [5-90] meses), ningún paciente falleció por progresión de la enfermedad. Conclusión: los TNEP representan una patología en aumento en nuestro centro. La cirugía resectiva con márgenes microscópicos negativos es la alternativa terapéutica de elección.

Neuroendocrine tumors: a registry of 1000 patients / Tumores neuroendócrinos: registro de 1000 pacientes

Neuroendocrine tumors (NET) can originate diffusely in most organs, with varying clinical presentations. The relative rarity of these tumors, previously referred to as carcinoids, encouraged several centers worldwide to study NET. Since 2003, a similar group was established in , the GETNE - Grupo de Estudo de Tumores Neuroendocrinos (Neuroendocrine Tumors Study Group) that included 32 centers, from all regions of Brasil. A patient database was initiated, collecting information about NET, regardless of the site of origin. OBJECTIVES: The present study shows initial results of 1000 patients included. METHODS: Descriptive statistical analyses, as well as overall survival rates for different groups of NET patients registered by GETNE. RESULTS: Most patients presented with thoracic NET (71.6 percent), while 20.2 percent had gastro-entero-pancreatic tumors. Median follow-up of all patients included was 33.7 months (range 1-141 months). At the time of the analysis, 29.3 percent of the patients were still alive (of these, 45.7 percent were alive with no evidence of disease). CONCLUSION: This is the largest database of NET in, and further accrual of new patients, as well as individual study results are expected in the near future.

Os tumores neuroendócrinos (TNE) podem se originar da maioria dos órgãos com apresentação clínica variável. A relativa raridade destes tumores, previamente classificados como carcinóides, levou vários centros no mundo a realizar estudos específicos dos TNE. A partir de 2003, um grupo similar foi criado no , GETNE- Grupo de Estudo de Tumores Neuroendocrinos, que incluiu 32 centros médicos de várias regiões do Brasil. Um arquivo de pacientes foi criado, registrando informações individuais sobre TNE, independente do órgão de origem. OBJETIVOS: O presente estudo apresenta os resultados dos primeiros 1000 pacientes incluídos. MÉTODOS: Análise estatística descritiva, assim como análises de sobrevida global dos pacientes registrados no GETNE. RESULTADOS: A maioria dos pacientes foi admitida com TNE torácicos (71,6 por cento), enquanto 20,2 por cento tiveram TNE gastro-entero-pacnreáticos. O seguimento mediano foi de 33,7 meses (variando entre 1-141 meses). Ao término desta análise, 29,3 por cento dos pacientes ainda estavam vivos (destes, 45,7 por cento vivos sem evidência de doença). CONCLUSÃO: Este representa o maior arquivo de TNE no , e a inclusão de novos pacientes, assim como estudos específicos, são esperados no futuro próximo.