[Tumores neuroendocrinos pancreáticos. Nuestra experiencia]. / Pancreatic neuroendocrine tumors. Our experience.

Pancreatic neuroendocrine tumors are rare. It is a heterogeneous group of neoplasms with very different behavior and prognosis. They can appear sporadically or associated with genetic syndromes. They are divided into functioning and non-functioning. A descriptive retrospective study of patients diagnosed with pancreatic neuroendocrine tumor was performed. The incidental diagnosis of these tumors is increasingly. The only curative treatment for these tumors is surgical excision, depending on the location and characteristics of the tumor and the patient. In selected cases a conservative attitude is recommended.

Los tumores neuroendocrinos pancreáticos son poco frecuentes. Es un grupo heterogéneo de neoplasias con comportamiento y pronóstico muy diferentes. Pueden aparecer de manera esporádica o asociados a síndromes genéticos. Se dividen en funcionantes y no funcionantes. Se realizó un estudio retrospectivo descriptivo de los pacientes diagnosticados de tumor neuroendocrino pancreático. Como se refleja en nuestra serie, cada vez es más frecuente el diagnostico incidental de estos tumores. El único tratamiento curativo de estos tumores es la exéresis quirúrgica, según la localización y las características del tumor y del paciente. En casos seleccionados se puede optar por una actitud conservadora.

Detection of pancreatic neuroendocrine tumors: 23 years of experience. / Detección de tumores neuroendocrinos pancreáticos: 23 años de experiencia.

INTRODUCTION AND AIMS: Neuroendocrine tumors are of great scientific interest, given that they are difficult to diagnose and treat. Despite being relatively rare (< 1/100,000 individuals, 1-2% of the gastrointestinal neoplasias) and indolent, their potential malignancy must not be forgotten. An increase in the number of diagnosed tumors has been observed in recent years. The aim of the present study was to update a published case series of 19 patients suspected of presenting with pancreatic neuroendocrine tumor with 51 current cases, to study and compare the new results with those of the previous case series, as well as with other recent publications from Spain, the United States, China, and India. MATERIALS AND METHODS: A retrospective, multicenter case series was conducted on 70 patients (19 cases published in 2011), whose data has been collected over a period of 23 years. The variables analyzed were: age, sex, symptomatology, tumor size, location, metastasis, final diagnosis, and surgery, among others. RESULTS: Mean patient age was 55 years and 60% of the patients were men. Disease location was the pancreatic head in 28.5% of the patients and the tail in 27.1%, mean tumor size was 3.9cm (0.2-10cm), 71.4% of the patients had non-functioning tumors, 32.8% had metastases (100% to the liver), 74.2% of the patients were operated on, and actuarial survival was 75%. CONCLUSIONS: Differences were observed between the previously published case series and the current results. There was an increase in incidentalomas and non-functioning tumors, but no variation in the overall survival rate. The differences with other case series (age, sex, and tumor location) were dependent on the country where the cases were compiled. The increase in tumors could be related to a higher number of diagnoses made through imaging studies and to the greater sensitivity of the devices employed.

[Pancreatic neuroendocrine tumours. What do we know of their history?]. / Tumores neuroendocrinos pancreáticos. ¿Qué conocemos de su historia?

Starting with Paul Langerhans, who first described pancreatic islets in 1869, this article reviews the various protagonists who, in the last century and a half, have contributed to the discovery of the main hormones originating in the pancreas, the analytical methods for their measurement, the imaging techniques for identifying tumoural location, and the various pancreatic neoplasms.

Costo Efectividad del Tratamiento de Tumores Neuroendócrinos Pancreáticos Avanzados no Operables con Sunitinib en México.

OBJECTIVES: Sunitinib had showed a substantial clinical benefit in patients with non-resectable pancreatic Neuroendocrine Tumors (NET). The objective of this study was to estimate the cost-effectiveness of sunitinib in the treatment of non-resectable pancreatic NET, from the perspective of the Social Security Mexican Institute (IMSS). METHODS: A Markov model (2-week cycles) was used to estimate the health and economic consequences of sunitinib 37.5mg/day+best supportive care (BSC) regarding placebo+BSC (ten-years horizon, discount rate: 5%). Effectiveness measures were: overall survival (OS), progression-free survival (PFS) and quality adjusted life years (QALY). Resource utilization (BSC, adverse events management, medical follow-up) was estimated through a survey with Mexican oncologists (n=10). Unit costs of medication and medical resources were obtained from institutional sources. Sensitivity analyses were performed and acceptability curves were constructed. RESULTS: Sunitinib+BSC gained 0.49 years (PFS), 1.18 years (OS) and 0.70 QALY against placebo+BSC. Sunitinib+BSC increased medical direct costs (2011 US$) per patient in $20,854, which was driven by acquisition costs of sunitinib and medical follow up before progression. ICER's were $42,157, $17,662 and $29,808 per progression-free year, life-year and QALY gained, respectively, which remained robust through±25% changes in main parameters. At willingness to pay higher than $40,000, $22,400 and $37,600 sunitinib+BSC becomes the most cost-effective alternative in regards to PFS, OS and QALYs, respectively. CONCLUSION: At IMSS, sunitinib+BSC would provide substantial clinical benefits to patients suffering unresectable pancreatic NET, although the latter would increase medical costs of treatment and clinical follow up.

Tumores neuroendocrinos gastroenteropancreáticos / Gastroenteropancreatic neuroendocrine tumors (GEP-NETS)

Los tumores neuroendocrinos gastroenteropancreáticos son neoplasias raras distribuidas a lo largo del tubo digestivo y poseen características peculiares, como la captación de sales de plata, la expresión de marcadores de célula neuroendocrina y los gránulos secretorios de contenido hormonal. Según su tamaño, localización anatómica y la presencia de metástasis, estos tumores debutan con distintas características clínicas y pronóstico. El diagnóstico temprano, que requiere de un alto grado de sospecha y una confirmación con estudios especializados, resulta invaluable para tratar estas lesiones a tiempo y aumentar la sobrevida de los pacientes. El tratamiento quirúrgico es la herramienta de primera mano, y otras terapias médicas ayudan a mejorar los síntomas y la calidad de vida de aquellos pacientes con lesiones irresecables. En esta revisión, se tratan los aspectos más relevantes en cuanto a la clasificación, morfología, localización, diagnóstico y tratamiento de estas neoplasias gastrointestinales, y al final, se expone la única experiencia colombiana sobre la epidemiología y el manejo de los tumores neuroendocrinos.

Gastroenteropancreatic neuroendocrine tumors (GEP-NETS) are rare neoplasms which can occur anywhere in the gastrointestinal tract. Their particular characteristics include uptake of silver salts, neuroendocrine cell marker expression and hormonal secretory granules. Depending on their size, anatomical location and upon whether or not metastasis has occurred, these tumors can show different clinical patterns and have different prognoses. Early diagnosis is essential for treating these lesions and improving the patients’ prognoses, but it requires a high degree of suspicion and confirmation by special testing. Surgical treatment is the first choice, but other medical therapy can be helpful for patients who have unresectable disease. This review presents the most relevant aspects of classification, morphology, methods of locating tumors, diagnosis and treatment of GEP-NETS. It presents only the Colombian experience in the epidemiology and management of these tumors.