Surgical options for the treatment of neuroendocrine neoplasms of the ampulla of Vater: a reference centre experience.

INTRODUCTION: The main objective of this study was to analyse the results of the surgical treatment of ampullary neuroendocrine tumours (NET) based on transduodenal ampullectomy and pancreatoduodenectomy, in a reference centre in hepatobiliopancreatic pathology. METHOD: Retrospective, observational study, including all patients operated on for pancreatic and/or duodenal NET in a reference unit of hepatobiliopancreatic pathology and prospectively registered between January 1st, 1993 and September 30th, 2021. For those parameters not present, retrospective research was performed. Demographic, clinical, analytical and pathological data were analysed. A descriptive study was carried out. Overall and disease-free survival was calculated using Kaplan-Meier curves and the Log-Rank test. RESULTS: Of 181 patients operated on for pancreatic and/or duodenal NET, only 9 were located in the ampulla of Vater, which represents 4.9% of all pancreatic and/or duodenal NET. Pancreatoduodenectomy (PD) was performed in 6 patients, while 3 patients underwent transduodenal ampullectomy (TDA). Longer surgical time and more postoperative complications were observed in the PD group. There were no differences in hospital stay. Overall and disease-free survival at 5 years in the PD group compared to ATD was 83.3% vs. 100% and 50% vs. 100%, respectively. CONCLUSIONS: Ampullary NET without locoregional involvement or risk factors, can be treated by conservative surgeries such as transduodenal ampullectomy.

Opciones en el tratamiento quirúrgico de la neoplasia neuroendocrina de la ampolla de Váter: experiencia en un centro de referencia / Surgical options for the treatment of neuroendocrine neoplasms of the ampulla of Vater: a reference centre experience

Introducción: El objetivo del estudio fue analizar los resultados del tratamiento quirúrgico de las neoplasias neuroendocrinas (NNE) ampulares mediante Ampulectomía transduodenal (ATD) y duodenopancreatectomía cefálica (DPC), en un centro de referencia en patología hepatobiliopancreática. Método: Estudio retrospectivo, observacional, incluyendo los pacientes intervenidos de NNE de páncreas y/o duodenales en una unidad de referencia en patología hepatobilipancreática y registrados prospectivamente entre el 1 de enero de 1993 y el 30 de septiembre de 2021. Para aquellos parámetros no presentes, se realizó una búsqueda retrospectiva. Se analizaron datos demográficos, clínicos, analíticos y anatomopatológicos. Se realizó un análisis descriptivo. La supervivencia global y libre de enfermedad se calculó mediante curvas de Kaplan-Meier y el test de log-rank. Resultados: De 181 pacientes intervenidos de NNE de páncreas y/o duodenales, solo nueve se localizaban en la ampolla de Váter, lo que representa 4,9% de todos los NNE pancreáticos y/o duodenales. Se realizó DPC en seis pacientes, mientras que a tres se les practicó ATD. Se observó mayor tiempo quirúrgico y más complicaciones en el grupo DPC. No hubo diferencias en la estancia hospitalaria. La supervivencia global y libre de enfermedad a cinco años del grupo DPC respecto a la ATD fue de 83,3 vs. 100% y del 50 vs. 100%, respectivamente. Conclusiones: Las NNE ampulares sin afectación locorregional ni factores de riesgo, pueden ser tratadas mediante cirugías preservadoras como la ATD. (AU)

Introduction: The main objective of this study was to analyse the results of the surgical treatment of ampullary neuroendocrine tumours (NET) based on transduodenal ampullectomy and pancreatoduodenectomy, in a reference centre in hepatobiliopancreatic pathology. Method: Retrospective, observational study, including all patients operated on for pancreatic and/or duodenal NET in a reference unit of hepatobiliopancreatic pathology and prospectively registered between January 1st, 1993 and September 30th,2021. For those parameters not present, retrospective research was performed. Demographic, clinical, analytical and pathological data were analysed. A descriptive study was carried out. Overall and disease-free survival was calculated using Kaplan-Meier curves and the Log-Rank test. Results: Of 181 patients operated on for pancreatic and/or duodenal NET, only 9 were located in the ampulla of Vater, which represents 4.9% of all pancreatic and/or duodenal NET. Pancreatoduodenectomy (PD) was performed in 6 patients, while 3 patients underwent transduodenal ampullectomy (TDA). Longer surgical time and more postoperative complications were observed in the PD group. There were no differences in hospital stay. Overall and disease-free survival at 5 years in the PD group compared to ATD was 83.3% vs. 100% and 50% vs. 100%, respectively. Conclusions: Ampullary NET without locorregional involvement or risk factors, can be treated by conservative surgeries such as transduodenal ampullectomy. (AU)

Appendiceal neuroendocrine neoplasia: analysis of 50 patients.

OBJECTIVE: The objective of this study was to investigate the clinical, surgical, and pathological findings of appendiceal neuroendocrine neoplasms (ANNs). MATERIALS AND METHODS: The demographic, clinical, surgical, and pathological characteristics of 50 patients with ANN were analyzed. The patients were also classified as Group 1 (< 40 years, n = 37) and Group 2 (≥ 40 years, n = 13), and compared each other in terms of all parameters. RESULTS: Acute appendicitis was the pre-operative clinical presentation in 48 (96%) patients. Appendectomy (94%) was the most common surgical procedure. Mean tumor size was 8.6 mm (1-70 mm). Approximately half of the tumors (46%) were T1. There was no lymphatic and distant metastasis. The patients in Group 2 (15.4 mm) had a higher mean tumor size than patients in Group 1 (6.3 mm) (p < 0.001). The two groups were similar in other characteristics (p > 0.05). CONCLUSIONS: ANNs are usually diagnosed after histopathological evaluation due to the lack of specific clinicoradiological signs. Therefore, carefull intraoperative examination of appendectomy specimens may increase the possibility of suspecting these tumors. The results also showed that ANNs were bigger in patients above 40-years-old. Although not statistically significant, ANNs tended to have higher grade and to be more located at the base of the appendix in this group of patients.

OBJETIVO: Investigar los hallazgos clínicos, quirúrgicos y patológicos de las neoplasias neuroendocrinas (RNA) apendiculares. MÉTODO: Se analizaron las características demográficas, clínicas, quirúrgicas y patológicas de 50 pacientes con RNA. Los pacientes también fueron clasificados como Grupo 1 (< 40 años, n = 37) y Grupo 2 (≥ 40 años, n = 13), y se compararon entre sí en términos de todos los parámetros. RESULTADOS: La apendicitis aguda fue la presentación clínica preoperatoria en 48 (96%) pacientes. La apendicectomía (94%) fue el procedimiento quirúrgico más común. El tamaño medio del tumor fue de 8,6 mm (1-70 mm). Aproximadamente la mitad de los tumores (46%) eran T1. No hubo metástasis linfáticas ya distancia. Los pacientes del Grupo 2 (15.4 mm) tenían un tamaño tumoral medio mayor que los pacientes del Grupo 1 (6.3 mm) (p < 0.001). Los dos grupos fueron similares en otras características (p > 0.05). CONCLUSIONES: Las RNA suelen diagnosticarse tras evaluación histopatológica debido a la falta de signos clínico-radiológicos específicos. Por lo tanto, el examen intraoperatorio cuidadoso de las muestras de apendicectomía puede aumentar la posibilidad de sospechar estos tumores. Los resultados también mostraron que las ANN eran más grandes en pacientes mayores de 40 años. Aunque no estadísticamente significativas, las ANN tendieron a tener mayor grado y estar más ubicadas en la base del apéndice en este grupo de pacientes.

Tumor neuroendócrino gástrico: relato de caso e revisão de literatura / Gastric neuroendocrine tumor: case report and literature review / Tumor neuroendocrino gástrico: reporte de caso y revisión de la literatura

Este artigo consiste em um estudo de caso de paciente do sexo masculino, 40 anos, com diarreia e perda ponderal significativa iniciada sete meses depois da sua admissão no Hospital Geral Roberto Santos. O paciente não apresentava alterações significativas no exame físico e as investigações clínicas e laboratoriais foram inexpressivas. Foram realizados estudos endoscópicos e anatomopatológicos, confirmando o diagnóstico de neoplasia neuroendócrina gástrica. Os achados histopatológicos evidenciaram um tumor bem diferenciado do tipo dois, sendo uma neoplasia rara de bom prognóstico. O paciente foi submetido a gastrectomia segmentar de corpo gástrico e evoluiu com melhora clínica da diarreia. Ele recebeu alta hospitalar e segue em acompanhamento com os serviços de gastroenterologia e endocrinologia.

This study reports the case of a 40-year-old male patient presenting with diarrhea and significant weight loss initiated seven months before admission at the Hospital Geral Roberto Santos. The patient showed no significant changes in the physical examination, and clinical and laboratory investigations were inexpressive. Gastric neuroendocrine neoplasia was diagnosed by endoscopic and anatomopathological studies. Histopathological findings showed a well-differentiated type 2 tumor ­ a rare neoplasm with a good prognosis. The patient underwent segmental gastrectomy of the gastric body, evolving with clinical improvement of diarrhea. He was discharged from the hospital and is being followed by gastroenterology and endocrinology services.

Paciente del sexo masculino, 40 años, con diarrea y pérdida significativa de peso, que inició siete meses después de su ingreso en el Hospital Geral Roberto Santos. El paciente no presentó cambios significativos en la exploración física y las investigaciones clínicas y de laboratorio fueron inexpresivas. Se realizaron estudios endoscópicos y anatomopatológicos que confirmaron el diagnóstico de neoplasia neuroendocrina gástrica. Los hallazgos histopatológicos mostraron un tumor tipo 2 bien diferenciado, que es una neoplasia poco frecuente y de buen pronóstico. El paciente fue sometido a gastrectomía segmentaria del cuerpo gástrico y evolucionó con mejoría clínica de la diarrea. Fue dado de alta del hospital y aún está en seguimiento con los servicios de gastroenterología y endocrinología.

Microcytic carcinoma of the urinary bladder: Experience over 22 years.

INTRODUCTION: Microcytic carcinoma of the urinary bladder or bladder Small Cell Carcinoma (SCC) is a rare entity, characterised by an aggressive behaviour, with a poor prognosis, elevated metastatic potential, and is commonly found in older patients and in advanced disease stages. Here we present our experiences with the behaviour of the disease and the treatments applied. MATERIAL AND METHOD: This was a retrospective study on patients diagnosed with bladder SCC in our hospital between February 1992 and February 2014. We analysed the demographic and clinical characteristics of the tumour, the applied treatments and survival. We performed a descriptive statistical analysis of the median follow-up time, Overall Survival (OS) and Cancer-Specific Survival (CSS), using the SPSS statistical package v. 15.0. RESULTS: Over 22 years, 20 patients with an average age of 75 years were diagnosed with bladder SCC (2 female). The predominant symptom was macroscopic haematuria (75%). After the first transurethral resection (TUR) of the bladder and the histological diagnosis, 35% (7 patients) did not receive additional treatment, 15% (3 patients) were treated with chemoradiotherapy (CRT), 10% (2 patients) with TUR, 15% (3 patients) with chemotherapy (QT), 5% (1 patient) with TUR associated to CRT, 5% (1 patient) with radical surgery, 5% (1 patient) with radical surgery treatment followed by adjuvant CRT, 5% (1 patient) with palliative surgery (hypogastric arteriae ligation) followed by adjuvant QT and 5% (1 patient) with hemostatic radiotherapy (RT). With a median follow-up time of 13.8 months, the OS was 14.48 months (95% CI: 6.22 - 22.75) and the CSS 18.04 months (95% CI: 6.51-29.57). Only 10% (2 patients) survived till the end of the study. CONCLUSION: Microcytic carcinoma of the urinary bladder is a rare and aggressive entity commonly diagnosed in males of advanced age and in advanced disease stages. It has a poor prognosis and reduced survival. Due to its aggressiveness previous to the initial diagnosis, a cystectomy is only possible in very few cases; therefore multimodal treatment is necessary. This treatment is yet to be defined.

[Intestinal carcinoid tumour: Case report]. / Tumor carcinoide intestinal: reporte de un caso.

BACKGROUND: Carcinoid of the small intestine, is a well-differentiated neuroendocrine tumor that rarely presents with clinical signs. This tumour can be associated with other conditions, such as inflammatory bowel disease, presenting a wide range of symptoms. In some cases they have an aggressive and highly symptomatic behaviour; thus, clinical suspicion must be high to make an early diagnosis. CLINICAL CASE: A 60 year-old male patient with Crohn's disease and gastrointestinal symptoms attributed to this disease within the last year. He presented with intestinal obstruction initially treated with conservative management with no improvement. Exploratory laparotomy was performed finding a mesenteric tumour that caused the bowel obstruction. Bowel resection with primary anastomosis was performed. The pathology report showed an intestinal carcinoid tumour with lymph node metastases. The patient recovered well, and was discharged without complications to continue medical treatment and follow-up by the Oncology department. CONCLUSION: In almost 42% of the cases, the most common site of carcinoid tumours is the small intestine, and of these, 41% are presented as locoregional disease. Patients with Crohn's disease present a higher incidence. In these cases, the most common presentation is an acute intestinal obstruction (90%). Surgery is usually curative, and follow up is important as the symptoms of Crohn's disease can hide any recurrence.

Neoplasia neuroendocrina quística múltiple: a propósito de un caso / Multiple cystic neuroendocrine neoplasm: a purpose of a case

Los tumores neuroendocrinos, independientemente del tipo de hormona que produzcan, tienen igual potencial de transformación quística. Presentamos el caso de femenino de 15 años quien presentó hace 3 años parotiditis acompañado de dolor en hemiabdomen superior y vómitos. Se realizaron estudios que diagnostican pancreatitis aguda y tomografía abdominal con evidencia de 3 lesiones ubicadas en el páncreas planteándose el diagnóstico de lesión neuroendocrina. La Gastrina en dos ocasiones reportó valores elevados de 970ng/L y 1313ng/L, es tratada con inhibidores de bomba de protones 40mg BID mejorando parcialmente los síntomas. La paciente no acudió a sus controles regulares por consulta en un lapso de 2 años. En abril de este año se realizó Ecoendoscopia superior por presentar nuevamente los mismos síntomas y se evidenció en el páncreas 5 lesiones que por las características mixtas con estructuras internas micro quístico sugieren Lesiones neuroendocrinas. Se realizó punción aspiración guiada por ecoendoscopia de 3 de las lesiones observando células epiteliales neoplásicas, sugestivos de tumores neuroendocrinos bien diferenciados. La Inmunohistoquimica reportó positiva para cromogranina, sinaptofisina y enolasa neuronal específica. Actualmente la paciente está en plan para cirugía

Neuroendocrine tumors, regardless of the type of hormone produced, have potential as cystic transformation. The case of women who presented 15 years ago 3 years accompanied mumps upper abdomen pain and vomiting. Studies were conducted to diagnose acute pancreatitis and abdominal CT with evidence of 3 lesions located one in the pancreas tail and one in considering the diagnosis of neuroendocrine injury. Gastrin twice the reported high levels of 970ng/L and 1313ng/L, is treated with proton pump inhibitors 40mg BID partially improve the symptoms. The patient did not attend regular checkups for consultation over a period of 2 years. In April this year was higher Ecoendoscopy to resubmit the same symptoms and showed 5 lesions in the pancreas of mixed characteristics internal structure micro cystic lesions suggestive of neuroendocrine. We performed EUS-guided aspiration of 3 lesions observed neoplastic epithelial cells, suggesting well-differentiated neuroendocrine tumors. The reported Immunohistochemistry positive for chromogranin, synaptophysin and neuron-specific enolase. Currently the patient is in surgical plan