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El síndrome carcinoide es un síndrome paraneoplásico que se presenta en tumores neuroendocrinos. Aunque es una entidad infrecuente suele ser la primera manifestación de la enfermedad. La baja incidencia junto a la presentación inespecífica genera retrasos diagnósticos importantes. Se presenta el caso de una paciente con síntomas digestivos y tuforadas que posteriormente agrega insuficiencia cardíaca, logrando mediante un ecocardiograma típico y marcadores analíticos el diagnóstico de síndrome carcinoide. Posteriormente se evidencia que su origen en un tumor neuroendocrino bronquial. Conocer las características de este síndrome es fundamental para mantener una alta sospecha clínica en pacientes con síntomas sugestivos logrando un diagnóstico precoz y adecuado.

Carcinoid syndrome is a paraneoplastic syndrome that occurs in neuroendocrine tumors. Although It is an uncommon entity, it is usually the first manifestation of the disease. The low incidence besides the non-specific presentation generates important diagnostic delays. We present the case of a patient presenting digestive symptoms and flushing that subsequently adds heart failure, achieving though a typical echocardiogram and analytical markers the diagnosis of carcinoid syndrome. Later it is discovered its origin in a bronchial neuroendocrine tumor. Knowing the characteristics of this syndrome is essential to maintain a high clinical suspicion in patients with suggestive symptoms, in order to achieve an early and adequate diagnosis.

El síndrome carcinoide é um síndrome paraneoplásico que ocorre em tumores neuroendócrinos. Embora seja uma entidade rara, geralmente é a primeira manifestação da doença. A baixa incidência, juntamente com a apresentação inespecífica, resulta em atrasos importantes no diagnóstico. Apresentamos o caso de uma paciente com sintomas digestivos e ruborização cutânea, que posteriormente desenvolve insuficiência cardíaca. O diagnóstico de síndrome carcinoide foi estabelecido por meio de um ecocardiograma característico e marcadores analíticos. Posteriormente, foi evidenciada a origem em um tumor neuroendócrino brônquico. Conhecer as características deste síndrome é fundamental para manter uma alta suspeita clínica em pacientes com sintomas sugestivos, permitindo um diagnóstico precoce e adequado.

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OBJECTIVE: The aim of the study was evaluate the diagnostic performance of [68Ga]Ga-DOTA-TOC and [18F]FDG PET/CT in patients with histologically proven neuroendocrine tumors (NETs), as well as the correlation of the visualized findings with the tumor grade. MATERIAL AND METHODS: We included 50 patients with NETs who underwent both [68Ga]Ga-DOTA-TOC and [18F]FDG PET/TC. The pooled sensitivity of both scans was compared, as well as [68Ga]Ga-DOTA-TOC and [18F]FDG for each tumor grade (grade 1/G1, grade 2/G2 and grade 3/G3). Also, the sensitivity of [68Ga]Ga-DOTA-TOC and [18F]FDG as a function of the continuous variable Ki-67 was investigated. Finally, the number of lesions detected by both PET radiopharmaceuticals for each tumor grade was compared. RESULTS: The pooled sensitivity of both PET/CT (96%) was higher than [68Ga]Ga-DOTA-TOC (84%) and [18F]FDG (44%) separately, with statistically significant differences. The sensitivity of [68Ga]Ga-DOTA-TOC was higher than [18F]FDG in both G1 (p = 0.004) and G2 (p < 0.001). In G3 the performance of both scans detected disease in 100% of this subgroup. The sensitivity of [68Ga]Ga-DOTA-TOC and [18F]FDG PET/CT correlated significantly with the Ki-67 proliferative index. In G2 patients the number of lesions detected with [68Ga]Ga-DOTA-TOC was higher than [18F]FDG. CONCLUSIONS: The performance of both PET/CT, particularly in G2 and G3, demonstrates the molecular heterogeneity of metastatic NETs and contributes to the selection of a more appropriate treatment, particularly in those high-grade patients who may benefit from radionuclide therapy (PRRT).

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Introducción: Los carcinomas neuroendocrinos (NEC) de canal anal son neoplasias extremadamente raras, representando del 1 a 1,6% de la totalidad de los tumores neuroendocrinos (NET). Suelen ser poco diferenciados, muy agresivos y con alta tendencia a metastatizar. Caso clínico: Mujer de 52 años diagnosticada de fisura anal. Durante la esfinterotomía lateral interna (ELI) se evidencia un pólipo milimétrico aparentemente hiperplásico. Biopsia: NEC de alto grado. En el estudio de extensión se observa engrosamiento de la mucosa del canal anal que invade el esfínter interno, sin enfermedad a distancia. Se realiza amputación abdominoperineal laparoscópica donde se objetiva infiltración del tabique rectovaginal, por lo que se realiza resección y vaginoplastia. AP: NEC con estadio PT4B N2A, por lo que se indica quimioterapia adyuvante. Discusión: La presentación clínica de los NEC de canal anal es inespecífica, diferenciándose de otros tumores colorrectales en que hasta el 67% de los pacientes presentan metástasis al diagnóstico, siendo la supervivencia media de 11 meses. Si diagnosticamos un NEC localizado de forma incidental, es fundamental la celeridad en su tratamiento, dada su agresividad.

Introduction: Neuroendocrine carcinomas (NEC) of the anal canal are extremely rare neoplasms, representing 1 to 1.6% of all neuroendocrine tumors (NET). They are usually poorly differentiated, very aggressive and with a high tendency to metastasize. Clinical case: A 52-year-old woman diagnosed with anal fissure. During the LIS, an apparently hyperplastic millimetric polyp is evidenced. Biopsy: high-grade NEC. The imaging study shows thickening of the mucosa of the anal canal that invades the internal sphincter, without metastases. We performed a laparoscopic abdominoperineal amputation, and noticed an infiltration of the rectovaginal septum, so resection and vaginoplasty was performed. Pathology: NEC with stage PT4B N2A, for which adjuvant chemotherapy is indicated. Discussion: The clinical presentation of NEC of the anal canal is nonspecific, differing from other colorectal tumors in that up to 67% of patients have metastases at diagnosis, with a median survival of 11 months. When an incidentally localized NEC is diagnosed, prompt treatment is essential, given its aggressiveness.

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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)

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Introdução: Os tumores neuroendócrinos pancreáticos são considerados raros. Eles são classificados em funcionantes e não funcionantes. Objetivo: Definir e classificar tumores neuroendócrinos pancreáticos de acordo com sua avaliação histopatológica e imunoistoquímica, associado aos critérios diagnósticos. Método: Trata-se de revisão narrativa sobre publicações encontradas no PubMed, SciELO e Google Acadêmico. Resultados: Esses tumores podem ser bem ou pouco diferenciados e apresentam características microscópicas distintas. As células bem diferenciadas têm formato pequeno, núcleos uniformes redondos ou ovais, citoplasma finamente granular indicando forte capacidade secretória e mantêm a estrutura organoide. A presença de necrose tumoral, atividade mitótica aumentada e índice de Ki-67 elevado indicam alta probabilidade de neoplasia neuroendócrina. Cromogranina A e sinaptofisina favorecem o diagnóstico do bem diferenciado. Já a marcação positiva do BCL 10 em conjunto com a ausência de expressão da cromogranina A e da sinaptofisina mostram a precária diferenciação tumoral. A presença de marcação positiva para as expressões hormonais não define o tumor como funcionante. Conclusão: Houve aumento do diagnóstico de tumores neuroendócrinos pancreáticos com o uso de técnicas de imagem e a conscientização sobre a doença. A análise histopatológica com imunoistoquímica, especialmente quando há sintomas consumptivos, podem indicar o tipo do carcinoma e induzir ao mais adequado tratamento.

Introduction: Pancreatic neuroendocrine tumors are considered rare. They are classified into functioning and non-functioning. Objective: To define and classify pancreatic neuroendocrine tumors according to their histopathological and immunohistochemical evaluation, associated with diagnostic criteria. Method: This is a narrative review of publications found in PubMed, SciELO and Google Scholar. \Results: These tumors can be well or poorly differentiated and have distinct microscopic characteristics. Well-differentiated cells are small in shape, have uniform round or oval nuclei, finely granular cytoplasm indicating strong secretory capacity, and maintain the organoid structure. Presence of tumor necrosis, increased mitotic activity and high Ki-67 index indicate a high probability of neuroendocrine neoplasia. Chromogranin A and synaptophysin favor the diagnosis of well-differentiated. The positive staining of BCL 10 together with the absence of expression of chromogranin A and synaptophysin show poor tumor differentiation. The presence of positive staining for hormone expressions does not define the tumor as functioning. Conclusion: There was an increase in the diagnosis of pancreatic neuroendocrine tumors with the use of imaging techniques and awareness of the disease. Histopathological analysis with immunohistochemistry, especially when there are consuming symptoms, can indicate the type of carcinoma and lead to the most appropriate treatment.

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Proliferative activity examined by Ki67 labeling index (LI) plays pivotal role for managing gastrointestinal neuroendocrine tumor (GI-NET). Few reports indicated the intra-patient heterogeneity of Ki67-LI among metastatic tumor sites. We report a case of brain and orbital metastases from GI-NET that showed discrepancy of the Ki67-LI. A 71 year-old woman who was diagnosed as GI-NET with liver and bone metastases and performed medical therapy, had headache, right exophthalmos, and pain of right eye and was referred to our department. Magnetic resonance image revealed that tumors in the left occipital region and right orbit. We diagnosed as metastatic brain and orbital tumors from GI-NET. Surgical removal of both symptomatic lesions was performed and the diagnosis was pathologically confirmed. Immunohistochemical studies revealed the discrepancy of the Ki67-LI of the lesions (brain tumor: 8% versus orbital tumor: 22%). Sampling of multiple metastatic sites may prevent underestimate tumor proliferative activity (AU)

La actividad proliferativa examinada por el índice de etiquetado Ki67 (LI) desempeña un papel fundamental en el tratamiento del tumor neuroendocrino gastrointestinal (GI-NET). Pocos informes indican la heterogeneidad intrapaciente del Ki67-LI entre las localizaciones de los tumores metastásicos. Presentamos un caso de metástasis cerebrales y orbitales de GI-NET que mostró discrepancia del Ki67-LI. Una mujer de 71 años a la que se le diagnosticó un GI-NET con metástasis hepáticas y óseas y que realizó tratamiento médico, presentó cefalea, exoftalmos derecho y dolor de ojo derecho, y fue remitida a nuestro departamento. La imagen de resonancia magnética reveló que los tumores en la región occipital izquierda y la órbita derecha. Diagnosticamos como metástasis tumores cerebrales y orbitales de GI-NET. Se realizó la extirpación quirúrgica de ambas lesiones sintomáticas y se confirmó patológicamente el diagnóstico. Los estudios inmunohistoquímicos revelaron la discrepancia del Ki67-LI de las lesiones (tumor cerebral: 8% frente a tumor orbitario: 22%). El muestreo de múltiples focos metastásicos puede evitar que se subestime la actividad proliferativa del tumor (AU)

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La enfermedad de Von Hippel-Lindau es un síndrome neoplásico, autosómico dominante, caracterizado por una mutación germinal del gen VHL que codifica para la proteína VHL en el cromosoma 3. Esta mutación predispone al desarrollo de tumores benignos y malignos que afectan diferentes órganos, a causa de una ausencia de la inhibición de la vía de la tumo-rigénesis mediada por el factor inducible por hipoxia. La prevalencia de esta enfermedad es de 2 a 3 por 100 000 personas y las neoplasias se localizan con mayor frecuencia en retina, sistema nervioso central, cabeza y cuello, páncreas, riñón, glándula suprarrenal y órgano reproductor. Se clasifica en 2 tipos dependiendo de la presencia o ausencia de feocromocitoma. El feocromocitoma y las neoplasias pancreáticas constituyen las manifestaciones endocrinas más frecuentes. El feocromocitoma se presenta entre el 10-30% de los casos. Puede cursar desde una entidad asintomática hasta una sintomatología variable que incluye la triada clásica de cefalea, palpitaciones y diaforesis. El diagnóstico se realiza mediante pruebas bioquímicas o sus metabolitos que confirman niveles elevados de catecolaminas, y estudios imagenológicos. Las lesiones pancreáticas son con frecuencia asintomáticas y se detectan de forma incidental en estudios de imagen realizados en los pacientes con VHL. Aunque las características clínicas y bioquímicas de estas neoplasias no son patognomóni-cas, pueden ser útiles para sugerir la enfermedad VHL como la etiología subyacente.

Von Hippel-Lindau disease is an autosomal dominant neoplastic syndrome characterized by a germline mutation of the VHL gene encoding the VHL protein on chromosome 3. This mutation predisposes to the development of benign and malignant tumors that affect different organs, due to an absence of inhibition of the hypoxia-inducible factor-mediated tumorigenesis pathway. The prevalence of this disease is 2 to 3 per 100,000 people, and neoplasms are most frequently located in the retina, central nervous system, head and neck, pancreas, kidney, adrenal gland, and the organ. It is classified into 2 types depending on the presence or absence of pheochromocytoma. Pheochromocytoma and pancreatic neoplasms are the most frequent endocrine manifestations. Pheochromocytoma occurs in 1030% of cases. It can range from an asymptomatic entity to a variable symptomatology that includes the classic triad of headache, palpitations and diaphoresis. The diagnosis is made through biochemical tests that confirm high levels of catecholamines and imaging studies. Pancreatic lesions are frequently asymptomatic and are detected incidentally in imaging studies performed in VHL patients. Although the clinical and biochemical characteristics of these malignancies are not pathognomonic, they may be useful in suggesting VHL disease as the underlying etiology.

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Proliferative activity examined by Ki67 labeling index (LI) plays pivotal role for managing gastrointestinal neuroendocrine tumor (GI-NET). Few reports indicated the intra-patient heterogeneity of Ki67-LI among metastatic tumor sites. We report a case of brain and orbital metastases from GI-NET that showed discrepancy of the Ki67-LI. A 71 year-old woman who was diagnosed as GI-NET with liver and bone metastases and performed medical therapy, had headache, right exophthalmos, and pain of right eye and was referred to our department. Magnetic resonance image revealed that tumors in the left occipital region and right orbit. We diagnosed as metastatic brain and orbital tumors from GI-NET. Surgical removal of both symptomatic lesions was performed and the diagnosis was pathologically confirmed. Immunohistochemical studies revealed the discrepancy of the Ki67-LI of the lesions (brain tumor: 8% versus orbital tumor: 22%). Sampling of multiple metastatic sites may prevent underestimate tumor proliferative activity.

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Los quistes intestinales (tailgut cyst), o hamartomas quísticos retrorrectales, son lesiones benignas raras que se incluyen en la categoría de lesiones quísticas del desarrollo. Aunque existen varias hipótesis con respecto a su desarrollo, actualmente sigue sin conocerse con certeza su origen. Se localizan principalmente en el espacio presacro (retrorrectal) y afectan fundamentalmente a mujeres de edad media (40-60 años).Teniendo en cuenta su localización y características histológicas, los principales diagnósticos diferenciales incluyen quistes epidermoides, quistes de duplicación y teratomas.La transformación maligna de estas lesiones es rara, la mayoría en forma de adenocarcinoma y tumores neuroendocrinos.Nosotros presentamos un caso de un quiste intestinal asociado a tumor neuroendocrino bien diferenciado (G1) en una mujer de 63 años.(AU)

ntestinal (tailgut) cysts or retrorectal cystic hamartomas are rare benign lesions that are included in the category of developmental cystic lesions. Their origin is still uncertain, although several hypotheses have been proposed to explain their development. They are located mainly in the presacral (retrorectal) space and predominately affect middle-aged women (40-60 years).Taking into account location and histological characteristics, the main differential diagnoses include epidermoid cysts, duplication cysts and teratomas.Malignant transformation of these lesions is rare and preferentially into adenocarcinoma and neuroendocrine tumors.We present a case of an intestinal cyst associated with a well-differentiated neuroendocrine tumor (G1) in a 63-year-old woman.(AU)

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Intestinal (tailgut) cysts or retrorectal cystic hamartomas are rare benign lesions that are included in the category of developmental cystic lesions. Their origin is still uncertain, although several hypotheses have been proposed to explain their development. They are located mainly in the presacral (retrorectal) space and predominately affect middle-aged women (40-60 years). Taking into account location and histological characteristics, the main differential diagnoses include epidermoid cysts, duplication cysts and teratomas. Malignant transformation of these lesions is rare and preferentially into adenocarcinoma and neuroendocrine tumors. We present a case of an intestinal cyst associated with a well-differentiated neuroendocrine tumor (G1) in a 63-year-old woman.

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Resumen Debido a la implementación de las colonoscopias como método de tamizaje, ha aumentado el diagnóstico de tumores neuroendocrinos rectales (TNER), la mayoría tiene un tamaño menor de 1 centímetro en el momento del diagnóstico, confinado a la submucosa y bien diferenciado; generalmente tienen un curso benigno y en su mayoría se tratan por métodos endoscópicos. Las metástasis son raras y dependen del tamaño tumoral y otros factores como la invasión a la submucosa, diseminación linfática y clasificación histológica, lo cual determinará el pronóstico y tratamiento a elección. Se presenta el caso de un tumor neuroendocrino rectal presentado en forma de pólipo durante un tamizaje endoscópico de rutina y una aproximación de la literatura actual.

Abstract Diagnosis of rectal neuroendocrine tumor (NET) has increased due to the implementation of colonoscopies as a screening method. Most rectal NETs are less than 1cm at diagnosis time, confined to the submucosa, and well differentiated. They generally have a benign course and are treated mainly using endoscopic methods. Metastases are rare and depend on tumor size and other factors such as submucosal invasion, lymphatic spread, and histologic classification, which will determine the prognosis and treatment. We present a case of a rectal neuroendocrine tumor as a polyp during routine endoscopic screening and a review of the current literature.

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Abstract Lung tumorlets are rare neuroendocrine neoplasms of 0.5 cm or less in diameter that extend beyond the basement membrane. Although they are associated with bronchiectasis and fibrosis they tend to be asymptomatic and behave in a benign way, usually being diagnosed as incidental microscopic nests of neuroendocrine cells in lung tissue. We present a case of a pulmonary tumorlet finding after right upper lobectomy for lung cancer.

Resumen. Los tumorlets pulmonares son neoplasias neuroendocrinas poco frecuentes, que se extienden más allá de la membrana basal y miden 0.5 cm o menos de diámetro. Aunque suelen asociarse a bronquiectasias y fibrosis pulmonar, suelen ser asintomáticas comportándose de una manera indolente, siendo usualmente diagnosticadas de forma incidental en el estudio microscópico de una pieza pulmonar. Presentamos el caso de un tumorlet pulmonar incidental luego de una lobectomía superior derecha por cáncer de pulmón.

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RESUMEN Introducción: el síndrome de Zollinger - Ellison es un tumor neuroendocrino que produce hipersecreción de ácido gástrico y úlcera péptica. Por lo que se realizó la revisión con el objetivo de describir los principales métodos diagnósticos de laboratorio e imagenológicos en el síndrome de Zollinger-Ellison. Desarrollo: se realizó una revisión bibliográfica con los descriptores en español e inglés "síndrome de Zollinger-Ellison", "tumor neuroendocrino" y gastrinoma, sacados de los descriptores en ciencias de la salud (DeCS/MeSH), en las bases de datos Google Académico, SciELO y National Library of Medicine. Como resultados se obtuvo que los métodos de laboratorio son la gastrina sérica basal en ayunas, la cual no es confiable debido a su alteración en diferentes enfermedades, el pH gástrico, excluye hipergastrinemias secundarias, y la secreción gástrica ácida basal, que diferencia las formas de hipergastrinemia. Estos análisis de laboratorio son complementarios entre sí, y para su realización se debe suspender la toma de los inhibidores de la bomba de protones. Otros estudios son la prueba de estimulación por secretina, que confirma la hipergastrinemia, y la prueba de estimulación por calcio, que diagnostica tumores > 1 mm que expresan receptores de calcio. Los métodos imagenológicos son fundamentales para la localización del tumor. La primera técnica de imagen que se debe realizar debido a su alta sensibilidad y especificidad es la gammagrafía con 111In-octreótido, esta localiza tumores no detectados con otras exploraciones y permite realizar el diagnóstico diferencial con lesiones hipervascularizadas. Conclusiones: el síndrome de Zollinger-Ellison requiere para un diagnóstico certero la utilización de métodos de laboratorio y de imagen novedosos.

ABSTRACT Introduction: Zollinger-Ellison syndrome is a neuroendocrine tumor that produces hypersecretion of gastric acid and peptic ulcer. Therefore, the review was carried out with the objective of describing the main laboratory and imaging diagnostic methods in Zollinger-Ellison syndrome. Development: a bibliographic review was carried out with the descriptors in Spanish and English "Zollinger-Ellison syndrome", "neuroendocrine tumor" and gastrinoma, taken from the descriptors in health sciences (DeCS / MeSH), in Google databases. Academic, SciELO and National Library of Medicine. As results, it was obtained that the laboratory methods are fasting basal serum gastrin, which is not reliable due to its alteration in different diseases, gastric pH, excludes secondary hypergastrinemias, and basal acid gastric secretion, which differentiates the forms of hypergastrinemia. These laboratory tests are complementary to each other, and to perform them, the intake of proton pump inhibitors must be suspended. Other tests include the secretin stimulation test, which confirms hypergastrinemia, and the calcium stimulation test, which diagnoses tumors >1 mm that express calcium receptors. Imaging methods are essential for tumor localization. The first imaging technique to be performed due to its high sensitivity and specificity is 111In-octreotide scintigraphy, which locates tumors not detected with other examinations and allows differential diagnosis with hypervascularized lesions. Conclusions: Zollinger-Ellison syndrome requires the use of novel laboratory and imaging methods for an accurate diagnosis.

RESUMO Introdução: a síndrome de Zollinger-Ellison é um tumor neuroendócrino que produz hipersecreção de ácido gástrico e úlcera péptica. Portanto, a revisão foi realizada com o objetivo de descrever os principais métodos de diagnóstico laboratorial e de imagem na síndrome de Zollinger-Ellison. Desenvolvimento: foi realizada revisão bibliográfica com os descritores em espanhol e inglês "Síndrome de Zollinger-Ellison", "tumor neuroendócrino" e gastrinoma, retirados dos descritores em ciências da saúde (DeCS/MeSH), nas bases de dados do Google. Acadêmico, SciELO e Biblioteca Nacional de Medicina. Como resultados, obteve-se que os métodos laboratoriais são a gastrina sérica basal em jejum, o que não é confiável devido à sua alteração em diferentes doenças, pH gástrico, exclui hipergastrinemias secundárias, e secreção gástrica ácida basal, o que diferencia as formas de hipergastrinemia. Esses exames laboratoriais são complementares entre si e, para realizá-los, deve-se suspender a ingestão de inibidores da bomba de prótons. Outros exames incluem o teste de estimulação de secretina, que confirma a hipergastrinemia, e o teste de estimulação de cálcio, que diagnostica tumores >1 mm que expressam receptores de cálcio. Os métodos de imagem são essenciais para a localização do tumor. A primeira técnica de imagem a ser realizada devido à sua alta sensibilidade e especificidade é a cintilografia com 111In-octreotide, que localiza tumores não detectados com outros exames e permite o diagnóstico diferencial com lesões hipervascularizadas. Conclusões: a síndrome de Zollinger-Ellison requer o uso de novos métodos laboratoriais e de imagem para um diagnóstico preciso.

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RESUMEN Introducción: El insulinoma es una neoplasia neuroendocrina considerada una de las más raras. Surge en las células beta de los islotes de Langerhans, produce insulina de forma constante, por lo que provoca hipoglucemia. Su diagnóstico se basa en criterios clínicos, analíticos y pruebas de imagen. El tratamiento curativo es la cirugía, mediante la enucleación del tumor, la pancreatectomía parcial o total o duodenopancreatectomía cefálica. La actual pandemia dio a lugar a nuevos protocolos de actuación, con el fin de adaptar los recursos sanitarios sin comprometer la seguridad de los pacientes y el personal de salud. Objetivo: Presentar un caso clínico de una paciente con diagnóstico de insulinoma, que requirió tratamiento quirúrgico en el curso de la COVID-19. Caso clínico: Paciente femenina de 48 años de edad, con episodios de hipoglucemia intensa y dificultad para seguimiento clínico adecuado. Se le diagnosticó insulinoma y la COVID-19; esta última empeoró la sintomatología, de forma que resultó imposible el adecuado control metabólico. Se decidió tratamiento quirúrgico; se llevó a cabo la enucleación del tumor, sin complicaciones. Conclusiones: La cirugía es el tratamiento definitivo para lograr la curación en el caso de los tumores neuroendocrinos y la COVID-19 empeora el pronóstico. La decisión del tratamiento quirúrgico, debe ser tomada en discusión multidisciplinaria con el fin de evitar complicaciones postoperatorias.

ABSTRACT Introduction: Insulinoma is a neuroendocrine neoplasm considered one of the rarest, it arises in the beta cells of the islets of Langerhans, it produces insulin constantly, thus causing hypoglycemia. Its diagnosis is based on clinical, analytical and imaging criteria. Curative treatment is surgery, through enucleation of the tumor, partial or total pancreatectomy, or cephalic pancreaticoduodenectomy. The current pandemic gave rise to new action protocols, in order to adapt health resources without compromising the safety of patients and health personnel. Objective: To present a clinical case of a patient diagnosed with insulinoma who required surgical treatment in the course of COVID-19. Clinical case: 48-year-old female patient, with episodes of severe hypoglycemia and difficulty in proper clinical follow up. She was diagnosed with insulinoma and COVID-19, the latter worsened the patient's symptoms in such a way that adequate metabolic control was impossible. Surgical treatment was decided. The tumor was enucleated, without complications. Conclusions: Surgery is the definitive treatment to achieve cure in the case of neuroendocrine tumors, COVID-19 worsens the prognosis of patients with this tumor. Therefore, the decision of surgical treatment must be made in a multidisciplinary discussion in order to avoid postoperative complications.

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Esta formación pretende presentar, de forma clara y de fácil comprensión, la biología de los tumores neuroendocrinos (TNE), las características de los receptores de somatostatina, cómo debe realizarse la selección de pacientes candidatos a terapia con péptidos radiomarcados (PRRT), si el paciente cumple los criterios para beneficiarse del tratamiento con los mínimos efectos adversos posibles, el protocolo de administración, seguimiento y evaluación de respuesta. También, se desarrollan los estudios de imagen funcional necesarios para explorar la biología del tumor e individualizar el tratamiento, que constituyen la piedra angular para el desarrollo de la teragnosis.Se están desarrollando ensayos clínicos para definir mejor la posición de PRRT dentro del amplio panorama terapéutico y, entre las perspectivas de futuro, hay varias líneas de investigación para mejorar la tasa de respuesta objetiva y la supervivencia con PRRT, centradas en el desarrollo de nuevos agonistas y antagonistas del receptor de somatostatina, nuevos radionúclidos y terapias combinadas radiosensibilizantes.En conclusión, la PRRT es un tratamiento bien tolerado, seguro y con efectos secundarios agudos, generalmente leves y autolimitados, que constituye una gran herramienta terapéutica que debe secuenciarse en el mejor momento de la evolución de la enfermedad de los pacientes con TNE. Los pacientes candidatos a la PRRT deben ser valorados siempre por un comité clínico multidisciplinar (AU)

This continuing education aims to present in a clear and easy-to-understand way, the biology of neuroendocrine tumors (NETs), the characteristics of somatostatin receptors, the selection of patients for radiolabelled peptide therapy (PRRT), the inclusion criteria to benefit from treatment with the minimum possible adverse effects, the administration protocol, follow-up and response evaluation. The functional imaging studies necessary to explore the biology of the tumor and to individualize the treatment are also carried out, and constitute the cornerstone for the development of teragnosis.Clinical trials are being developed to better define the position of PRRT within the broad therapeutic options, and among the future perspectives, there are several lines of research to improve the objective response rate and survival with PRRT, focused on the development of new agonists and somatostatin receptor antagonists, new radionuclides and radiosensitizing combination therapies.In conclusion, PRRT is a great therapeutic, well-tolerated and safe tool with generally mild and self-limited acute side effects, that must be sequenced at the best moment of the evolution of the disease of patients with NET. Candidate patients for PRRT should always be evaluated by an multidisciplinary clinical committee (AU)

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RESUMEN

This continuing education aims to present in a clear and easy-to-understand way, the biology of neuroendocrine tumors (NETs), the characteristics of somatostatin receptors, the selection of patients for radiolabelled peptide therapy (PRRT), the inclusion criteria to benefit from treatment with the minimum possible adverse effects, the administration protocol, follow-up and response evaluation. The functional imaging studies necessary to explore the biology of the tumor and to individualize the treatment are also carried out, and constitute the cornerstone for the development of teragnosis. Clinical trials are being developed to better define the position of PRRT within the broad therapeutic options, and among the future perspectives, there are several lines of research to improve the objective response rate and survival with PRRT, focused on the development of new agonists and somatostatin receptor antagonists, new radionuclides and radiosensitizing combination therapies. In conclusion, PRRT is a great therapeutic, well-tolerated and safe tool with generally mild and self-limited acute side effects, that must be sequenced at the best moment of the evolution of the disease of patients with NET. Candidate patients for PRRT should always be evaluated by a multidisciplinary clinical committee.

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RESUMEN

Resumen Los tumores neuroendocrinos del colon son relativamente raros en comparación con el carcinoma colorrectal esporádico; su incidencia es muy baja y la localización más frecuente es en el ciego, colon sigmoideo y recto. Se describe con poca frecuencia la asociación de tumor neuroendocrino con la colitis ulcerativa debido a la inflamación crónica que conduce a la diferenciación de células neuroendocrinas a partir de células multipotenciales en el epitelio displásico, que puede ser responsable del desarrollo de carcinomas neuroendocrinos colorrectales. La presentación del caso se trata de un paciente de 57 años de edad con colitis ulcerativa con cuadro de dolor abdominal, pérdida de peso y diarreas líquidas con moco. En el examen físico presentó una lesión endurecida en el marco colónico derecho con tomografía de lesión neoplásica en el colon ascendente y en su hospitalización presentó un cuadro de oclusión intestinal, por lo que se le realizó una hemicolectomía más ileostomía, cuyo resultado en la biopsia fue un tumor neuroendocrino G3 de células grandes en el colon ascendente. En este artículo se revisan de forma práctica los aspectos relacionados con la fisiopatología, diagnóstico y tratamiento de la asociación de estas dos patologías.

Abstract Neuroendocrine colon tumors are relatively unknown compared with sporadic colorectal cancer; its incidence is low, and it is generally located in the cecum, sigmoid colon, and rectum. The existing relation between neuroendocrine tumors and ulcerative colitis is not frequently described due to the chronic inflammation that leads to neuroendocrine cell differentiation from multipotential cells in the dysplastic epithelium, which can be responsible for colorectal neuroendocrine carcinomas development. The study refers to the case of a patient of 57 years old with ulcerative colitis, abdominal pain, weight loss, and liquid diarrhea with mucus. Physical examination revealed a hardened lesion in the right colonic framework with the tomography of a neoplastic lesion in the ascending colon. When the patient was hospitalized, he developed an intestinal obstruction. A hemicolectomy plus ileostomy procedure was performed resulting in a large cell G3 neuroendocrine tumor in the ascending colon revealed in the biopsy. In this article, the aspects related to the pathophysiology, diagnosis, and treatment of the association of these two pathologies are reviewed in a practical way.

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