Metástasis ganglionar mesentérica de neoplasia neuroendocrina: una causa infrecuente de obstrucción de intestino delgado / Small bowel obstruction caused by lymph nodes of a neuroendocrine tumor: a clinical case report

Small intestine neuroendocrine tumors (NET-SI) are relatively rare neoplasms. If encountered, the most common location is the ileum. Symptoms are usually non-specific, delaying the tumors diagnosis. NET-SI are often small in size and can be challenging to recognize on imaging studies. However, they have a tendency to induce a pronounced fibrotic reaction in the mesentery, often accompanied by large calcified mesenteric adenopathies. In some cases, the fibrotic reaction can produce rare complications, such as intestinal obstruction or vascular congestion with occasional secondary ischemia. This case report presents a 79-year-old male with a partial small bowel obstruction caused by a fibrotic reaction and mesenteric adenopathies of a well-differentiated neuroendocrine tumor of the ileum. The patient also presented multiple peritoneal metastases at diagnosis. Characteristic imaging findings of the tumor, allowed an accurate and early diagnosis. Once the acute episode was resolved, the diagnosis was confirmed with an image guided biopsy.

Role of Imaging in Early Therapeutic Response Assessment for Neuroendocrine Neoplasm with Liver Metastasis:from Anatomical to Functional View / 中国医学科学院学报

Liver metastasis is not rare during the course of neuroendocrine neoplasms.The methods for treating neuroendocrine neoplasm with liver metastasis(NENLM)are diversifying,which exposes the limitations of the early therapeutic response assessment based on only morphological changes.The emerging imaging biomarkers can sensitively describe changes in response to treatment from the functional level,providing new ideas for the therapeutic response evaluation of NENLM.In this paper,we reviewed the status quo and the latest research progress of imaging assessment for early therapeutic response of NENLM,aiming to provide reference for assessing the response and further exploring the treatment-related biomarkers.

Application of molecular probes in nuclear imaging of neuroendocrine tumors / 浙江大学学报·医学版

Neuroendocrine tumors are a type of heterogeneous tumors originating from neuroendocrine cells derived from the neural crest，which can secrete a variety of amines and peptide hormones.Based on different molecular biomarkers，histologic types and differentiation degrees，individualized nuclear imaging can provide information for the early diagnosis，clinical staging，treatment guidance，and detection of the recurrence and metastasis of neuroendocrine tumor. In this paper，we review the development and application of nuclear medicine molecular imaging probes such as glucose analogs，somatostatin analogues，amine precursors，hormone analogs and enzyme inhibitors in the diagnosis and treatment of neuroendocrine tumors.

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.

Utilidad de la elastografía guiada por ultrasonografía endoscópica en la evaluación de lesiones sólidas pancreáticas / Usefulness of endoscopic ultrasound guided elastography in the assessment of solid pancreatic lesions

Introducción: La elastografía guiada por ultrasonografía endoscópica es considerada una herramienta útil en la evaluación de las lesiones solidas pancreáticas (LSP). Objetivo: El objetivo del estudio fue evaluar el rendimiento diagnóstico de la elastografia en pacientes con LSP. Material y métodos: Se realizó un estudio transversal prospectivo en el hospital Rebagliati durante julio 2017 a junio 2018. Se incluyeron pacientes con diagnóstico de LSP y estudio ecoendoscópico, elastografía y toma de PAAF. Se realizó elastografia cualitativa y elastografia cuantitativa (SR e histograma) y se analizó con resultados histopatológicos para determinar la sensibilidad, especificidad, valor predictivo positivo (VPP), valor predictivo negativo (VPN) y exactitud diagnostica en la detección de malignidad. Resultados: De 846 ecoendoscopías, se estudiaron 46 pacientes con LSP con una edad promedio de 64,6 años, 29 (63%) sexo femenino. El adenocarcinoma pancreático fue diagnosticado en 36 casos (78,3%). En elastografía cualitativa predominó el score 3 (n=39, 84,8%) con una sensibilidad, especificidad y exactitud de 88.9%, 30% y 76,1% respectivamente para predecir adenocarcinoma. Elastografía cuantitativa de SR≥ 15 (sensibilidad 100%, especificidad 66,7% y exactitud 97,8%) y un valor de histograma menor de 49 (sensibilidad 66,7%, especificidad 97,6% y exactitud 95,6%) predice malignidad en una LSP con área bajo de la curva ROC de 0,941 (IC 95%, 0,82 - 1,0). Conclusiones: La elastografía brinda información para predecir la naturaleza maligna de la lesión. En nuestro estudio la detección elastográfica de un score 3, SR≥ 15 o un histograma < 49 predice la presencia de malignidad en la LSP estudiada.

Introduction: endoscopic ultrasonography (EUS) elastography is considered a useful tool for the evaluation of solid pancreatic lesions (SPL). Objective: The aim of our study was to evaluate the diagnostic performance of elastography in patients with SPL. Material and methods: A prospective, cross-sectional study was performed at the Rebagliati Hospital between July 2017 and June 2018. Patients with a diagnosis of SPL and echoendoscopic study, elastography and FNA were included. Qualitative and quantitative elastography: strain ratio (SR) and strain histogram, were performed and analyzed with histopathological results. The diagnostic accuracy of EUS elastography in detecting malignancy was calculated using receiver operating curve analysis. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy for the detection of malignancy were calculated. Results: Out of 832 EUS examinations performed, 46 patients with SPL (mean age, 64.6 years; 29 women) were included in the study. Pancreatic adenocarcinoma was diagnosed in 36 cases. In qualitative elastography, score 3 was most frequent (n = 39, 84.8%) with sensitivity, specificity and accuracy of 88.9%, 30% and 76.1%, respectively, for predict adenocarcinoma. A strain ratio of 15 or higher (100% sensitivity, 66.7% specificity and 97.8% accuracy) and a histogram of less than 49 (66.7% sensitivity, 97.6% specificity and 95.6% accuracy) predicts malignancy in SPL, with area under a ROC curve of 0.941 (95% CI, 0.82 - 1.0). Conclusions: EUS elastography provides information to predict the malignant nature of the pancreatic lesion. In our study, the elastographic detection of a score 3, SR≥15 or a histogram <49 predicts the presence of malignancy in LSP.

Carcinoid syndrome: update on the pathophysiology and treatment

Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.

Tumores neuroendocrinos de origen pulmonar: reporte de un caso / Neuroendocrine tumors of lung’s origin, a case report

Introduction or case story: Young female patient (24 years-old), without known morbid precedents. She comes for a ten-days period of symptoms characterized by fever of up to 38.5 °C and a dyspnoea grade III. Physical exam showed decreased vesicular murmur on the right pulmonary base with dullness and positive vocal vibrations. Exams: Thorax X-ray: Atelectasis condensation on the right pulmonary base. CT chest scan without contrast: Nodule located in an intermediate bronchus which generates atelectasis in the basal bronchi. Fibro-bronchoscopy (FOB): A tumour-like injury blocking 100% of the right intermediate bronchus’ duct. Biopsy: Pulmonary tissue with haemorrhagic areas, granular tissue with small cellular clusters of lobular disposal and glandular shape with eccentric central nuclei cells, with homogenous chromatin and without atypical mitosis. Immunohistochemistry: Intensely positive cells to synaptophysin and CD56. Diagnosis: Neuroendocrine Typical Carcinoid Tumor. Comments: The patient evolves without progression of dyspnoea, she is waiting for a surgical resolution of tumour at National Institute of Thorax.

Introducción o historia del caso: Mujer joven de 24 años de edad, sin antecedentes mórbidos, acudió por cuadro de 10 días de evolución, de fiebre de hasta 38,5 °C y disnea grado III. Al examen físico destacó a nivel pulmonar: murmullo pulmonar disminuido en base pulmonar derecha, matidez de la misma zona y vibraciones vocales positivas. Exámenes: Radiografía de Tórax: Condensación atelectásica en base pulmonar derecha. TAC de Tórax sin contraste: Imagen nodular a nivel de bronquio intermedio, que genera atelectasia en bronquios basales. Fibrobroncoscopía (FBC): Lesión tumoral que ocluye el 100% del lumen para bronquio intermedio derecho. Biopsia: Tejido pulmonar con áreas de hemorragia, tejido granulatorio y tumor con acúmulos celulares de disposición lobular y glanduliforme, con núcleos centrales excéntricos, cromatina homogénea, sin atipias. Inmunohistoquímica: Células intensamente positivas para sinaptofisina, y CD-56. Diagnóstico: Tumor Neuroendocrino Carcinoide típico Comentarios: Paciente evoluciona sin progresión de su disnea, esperando resolución quirúrgica del tumor en Instituto Nacional del Tórax.

Experiencia de una institución Argentina en tumores neuroendocrinos de cérvix / Experience of an Argentine institution in neuroendocrine tumors of the cervix

El cáncer de cuello uterino es de alta prevalencia en nuestra comunidad. El subtipo escamoso tiene directa relación con factores socioeconómicos. Los tumores neuroendocrinos son la variedad menos frecuente y no presentan un claro agente causal. En este trabajo se analizan los casos identificados en nuestro instituto en la última década, enfocándonos en los aspectos de presentación de la enfermedad y rasgos sociales (AU)

The cervical cancer has a high prevalence in our comunity. It is a disease with a direct correlation to socio-economic factors in cases of squamous subtype. Neuroendocrine tumors are the least frequent subtype, and do not present a clear cause. In this study, we present the cases identified in our institution in the last decade, focusing on the clinical presentation aspects of the disease and its social traits (AU)

Uso del análogo de somatostatina 99m TC-HYNIC-TYR-3-Octreotida 20 µg en gammagrafias de receptores de somatostatina para la detección de tumores neuroendocrinos / Use of the somatostatin 99m TC-HYNIC-TYR-3-Octreotide analogue 20 µg in somatostatin receptor scans for the detection of neuroendocrine tumors

INTRODUCCIÓN: Antecedentes: El presente dictamen expone la evaluación de tecnología del uso del análogo de somatostatina 99m TC-HYNIC-TYR-3-Octreotida (99mTc-HYNIC-TOC) em gammagrafias de receptores de somatostatina para la detección de tumores neuroendocrinos.Aspectos Generales: Los tumores neuroendocrinos (TNE) surgen a partir de células neurales, las cuales pueden diferenciarse de cualquier otro tipo de célula, llegando así a distribuirse en todo el cuerpo. Este tipo de neoplasias es poco común ya que constituyen menos del 5% de todos los carcinomas de sitio primario desconocido. Este grupo de neoplasias es considerablemente heterogéneo, ya que incluye varios sub-tipos, los cuales difieren en sintomatologia, apariencia histológica y respuesta a tratamiento. Los subtipos incluyen, por ejemplo, los TNE bien diferenciados del tracto gastrointestinal, pancreáticos, tumores medulares de la tiroides y cáncer de pulmón de células pequeñas. Tecnología Sanitaria de Interés: El Tecnecio-99m es un isótopo radioactivo que posee menor energia de radiación que In-11, el cual al unirse con un análogo de somatostaina (i.e.: Octreotida) constituye un radiofármaco que es adminitrado vía intravenosa para la realización de pruebas por imágenes diagnósticas tales como PET, SPECT, RMN entre otras. METODOLOGÍA: Estratégia de Búsqueda: Se realizó una estratégia de búsqueda sistemática de la evidencia científica con respecto al uso del análogo de somatostatina 99mTC-HYNIC-TYR-3-Octreotida (99mTC-HYNIC-TOC) en gammagrafías de receptores de somatostatina para la detección de tumores neuroendocrinos. Para la búsqueda primaria se revisó la información disponible por entes reguladoras y normativas como la Food and Drug Administration (FDA), y la Dirección General de Medicamentos y Drogas (DIGEMID). Posteriormente se buscaron Guías de Práctica Clínica a través de los metabuscadores: Translating Research into Practice (TRIPDATABASE)< National Library of Medicine (Pubmed-Medline), The National Guideline of Clearinghouse, y Helath Systems Evidence. Finalmente, se realizo una búsqueda dentro de la información generada por grupos internacionales que realizan revisiones sistemáticas, evaluación de tecnologías sanitarias y guías de práctica clínica, tales como The Cochrane Library, The National Institute for Health and Care Excellence (NICE), The Canadian Agency for Drugs and Technologies en Health (CADTH), The Scottish Medicines Consortium (SMC), que a su vez fue complementada con una búsqueda em www.clinicaltrials.gov, para indentificar estudios primarios en elaboración o que no hayan sido publicados aún. Se realizó además una búsqueda manual con una estrategia de "bola de nieve" mediante revisión de listas de referencias de las guías, evaluaciones de tecnologías, estudios primarios y revisiones narrativas seleccionados. RESULTADOS: Tras la búsqueda se encontró evidencia científica acerca del uso del análogo de somatostatina 99mTC-HYNIC-TOC en gammagrafias de receptores de somatostatina para la detección de tumores neuroendocrinos. Sinopsis de la Evidencis: A continuación, se detalla la evidencia científica encontrada acerca del uso del análogo de somatostatina 99mTC-HYNIC-TOC en gammagrafias de receptores de somatostatina para la detección de tumores neuroendocrinos. No se encontraron Guías de Práctica Clínica (GPC), Evaluaciones de Tecnología Sanitarias (ETS), Revisiones Sistemáticas que respondan a la pregunta PICO de interés. CONCLUSIONES: La presente evaluación de tecnología sanitaria presenta la evidencia científica encontrada acerca del uso de análogo de somatostatina 99mTC-HYNIC-TYR-3-Octreotida (99mTC-HYNIC-TOC) en gammagrafías de receptores de somatostatina para la detección de tumores neuroendocrinos (TNE). El 99mTC-HYNIC-TYR-3-Octreotida es la única alternativa disponible en el mercado peruano para la realización de gammagrafías de tumores neuroendocrinos. A diferencia de otro tipo de células oncológicas, las neuroendocrinas, carecen de alta actividad metabólica y expresan en su lugar, receptores de somatostaina, siendo necesario el uso de radiofármacos análogos de somatostatina com el 99mTC-HYNIC-TYR-3-Octreotida para la oportuna detección de TNE. El Instituto de Evaluación de Tecnologías en Salud e Investigación-IETSI, aprueba el uso del análogo de somatostatina 99mTC-HYNIC-TYR-3-Octreotida en gammagrafias de receptores de somatostatina para la detección de tumores neuroendocrinos. El presente Dictamen Preliminar tiene una vigencia de dos años a partir de la fecha de publicación.

Tratamento de tumores neuroendócrinos por medicina nuclear: revisão sistemática da eficácia e segurança do radiofármaco Dotatate-177Lu / Treatment of neuroendocrine tumors by nuclear medicine: a systematic review of efficacy and safety of the radiopharmaceutical 177Lu-Dotatate

O DOTATATE-177Lu é um análogo de somatostatina radiomarcado que guia a radioatividade para tumores neuroendócrinos (NETs) que superexpressam receptores de somatostatina (SSTRs), promovendo o seu tratamento por terapia radionuclídica receptor-específica (PRRT). O objetivo deste trabalho foi avaliar a eficácia e a segurança da PRRT dos NETs com o DOTATATE-177Lu, através de uma revisão sistemática da literatura. A PRRT com o DOTATATE-177Lu foi eficaz no tratamento de NETs metastáticos ou inoperáveis que expressam SSTR tipo 2. Observou-se estabilização ou até regressão da doença em 20 a 50 % dos casos. Já a prevalência de remissão total da doença, apesar de numericamente baixa, foi satisfatória, principalmente levando-se em conta o estágio avançado dos tumores. Os efeitos adversos mais comuns da PRRT foram toxicidade renal e hematológica. Apesar dos estudos evidenciarem a eficácia e a segurança da PRRT com o radiofármaco, essa terapia ainda é considerada em desenvolvimento.

The DOTATATE-177Lu is a radiolabeled somatostatin analogue that guides the radioactivity to neuroendocrine tumors (NETs) that overexpress somatostatin receptors (SSTRs), allowing their treatment by receptor- specific radionuclide therapy (PRRT). The aim of this study was to evaluate the efficacy and safety of PRRT of NETs with DOTATATE-177Lu through a systematic literature review. PRRT with 177Lu - DOTATATE was effective in the treatment of inoperable or metastatic NETs that express SSTR type 2. Stabilization or regression of the disease was observed in 20 to 50% of the cases . The prevalence of total remission, although numerically low, was satisfactory, especially taking into account the advanced tumor stage. The most common adverse events in the PRRT were hematological toxicity and renal failure. Despite studies demonstrate the efficacy and safety of PRRT with the radiotracer , this treatment modality is still considered under development.

Neuroendocrine tumors of the liver / 대한간학회지

No abstract available.