Síndrome de Hedinger secundario a Tumor neuroendocrino bronquial. Presentación de un caso

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.

Tumor neuroendocrino primario de hígado / Primary neuroendocrine tumor of the liver

RESUMEN Los tumores neuroendocrinos generalmente se originan en el tracto digestivo o páncreas, desarrollando metástasis hepática a lo largo de su evolución. La presencia de un tumor neuroendocrino primario de hígado es motivo de controversia y de muy escasa casuística. Presentamos el caso de un tumor neuroendocrino primario de hígado confirmado por estudio anatomo-patológico e imágenes. La paciente fue sometida a dos resecciones hepáticas mayores, preservando sólo el segmento IV.

ABSTRACT Neuroendocrine tumors generally originate in the digestive tract or pancreas, developing liver metastases throughout their evolution. The presence of a primary neuroendocrine tumor of the liver is still controversial and there are very few cases. We present a case of a primary neuroendocrine tumor of the liver confirmed by anatomo-pathological study and images. The patient underwent two major liver resections, lastly preserving only segment IV.

Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

Carcinoma neuroendocrino de canal anal: presentación de un caso y revisión bibliográfica

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.

Divertículo de Meckel con tumor neuroendocrino: presentación inusual / Meckel's diverticulum with neuroendocrine tumor: unusual presentation

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)

Carcinoma neuroendocrino de la piel: Carcinoma de células de Merkel. Revisión de la Literatura, a propósito de un caso / Neuroendocrine skin carcinoma: Merkel cell carcinoma. Review of literature, about a case

El carcinoma de células de Merkel, también llamado carcinoma neuroendocrino de la piel, es un tipo de cáncer de piel muy poco frecuente que generalmente aparece como un nódulo de color carne o rojo azulado, más frecuentemente en región facial, cabeza y cuello. El carcinoma de células de Merkel se desarrolla principalmente en personas mayores ya que la exposición al sol a largo plazo o un sistema inmunitario débil pueden aumentar el riesgo de desarrollarlo. Las células de Merkel se encuentran en la base de la capa más externa de la piel (epidermis) y están conectadas a las terminaciones nerviosas que son responsables del sentido del tacto. Tiende a crecer rápido y diseminarse a otras partes del cuerpo. Por tanto, las opciones de tratamiento para el carcinoma de células de Merkel dependen de si el cáncer se ha diseminado más allá de la piel

Merkel cell carcinoma, also called neuroendocrine skin of the skin, is a very rare type of skin cancer that generally appears as a bluish meat or red color nodule, more frequently in the facial, head and neck region. Merkel cell carcinoma develops mainly in older people since long -term exposure or a weak immune system can increase the risk of developing it. Merkel cells are at the base of the outermost layer of the skin (epidermis) and are connected to nerve endings that are responsible for the sense of touch. It tends to grow quickly and spread to other parts of the body. Therefore, the treatment options for Merkel cell carcinoma depend on whether cancer has spread beyond the skin

Carcinoma neuroendocrino de vulva, una entidad poco frecuente. Reporte del primer caso en Venezuela / Neuroendocrine carcinoma of the vulva, a rare entity. Report of the first case in Venezuela

El cáncer de vulva representa 1 % de los cánceres en mujeres; el carcinoma neuroendocrino de vulva es extremadamente raro, con menos de 20 casos publicados. Su comportamiento es muy agresivo produciendo recurrencias locales tempranas, metástasis a ganglios linfáticos y a distancia. Se describe el caso de una paciente de 60 años de edad, quien consultó por lesión vulvar de 6 meses de evolución, con extensión a tercio inferior de uretra y vagina y plastrón ganglionar inguinal izquierda. La biopsia de vulva reportó neoplasia maligna de células redondas y el resultado de la inmunohistoquímica fue compatible con carcinoma neuroendocrino. Recibió quimioterapia y radioterapia concurrente, evidenciando respuesta completa en vulva y parcial en región inguinal, posteriormente se realizó linfadenectomía inguinofemoral bilateral. El carcinoma neuroendocrino de vulva debe ser considerado al momento de manejar patología vulvar maligna, el diagnóstico oportuno requiere histología e inmunohistoquímica para establecer un pronóstico y tratamiento adecuados(AU)

Vulvar cancer represents 1% of cancers in women; vulvar neuroendocrine carcinoma is extremely rare, with less than 20 published cases. Its behavior is very aggressive, producing early local recurrences, lymph node and distant metastases. The case of a 60-year-old patient who consulted for a vulvar lesion of 6 months of evolution, with extension to the lower third of the urethra and vagina and left inguinal nodal plastron is described. The vulvar biopsy reported malignant round cell neoplasm and the immunohistochemistry result was compatible with neuroendocrine carcinoma. She received concurrent chemotherapy and radiotherapy, evidencing a complete response in the vulva and a partial response in the inguinal region. Later, bilateral inguinofemoral lymphadenectomy was performed. Neuroendocrine carcinoma of the vulva should be considered when managing malignant vulvar pathology, timely diagnosis requires histology and immunohistochemistry to establish an adequate prognosis and treatment(AU)

Tumores neuroendócrinos pancreáticos: revisão histopatológica / Pancreatic neuroendocrine tumors: histopathological review

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.

Tumor neuroendócrino em coração de cão / Neuroendocrine tumor involving the heart of a dog

Background: Neuroendocrine tumors are epithelial tumors with predominant neuroendocrine differentiation. Ectopic thyroid carcinoma develops in the ectopic thyroid parenchyma, in the anterior mediastinum, being an important differential diagnosis of paraganglioma, although occurring less frequently than the same. The cells invade the mediastinal fat reaching the adventitia of the aorta. There is a possibility of metastatic formations in the lung, kidneys, and pancreas. A supporting treatment was provided. The present study proposes the exposition and description of a case of neuroendocrine tumor in the heart, considering the rarity of the involvement of this tumor in the canine species. Case: A 9-year-old American Bulldog male canine was treated at home in the city of Rio de Janeiro. On clinical examination, dyspnea, edema of the hind limbs and an increase in abdominal volume were observed. With the suspicion of congestive heart failure, mainly on the right, cardiac exams were requested. Because it was an aggressive animal, sedation was performed with a combination of tiletamine and zolazepam. During cardiac auscultation, the presence of S4 was noticed, characterizing a gallop rhythm. No alterations were observed on the electrocardiographic examination. The echocardiogram showed a tumor mass in the atrium and dilation of the venous return vessels. The tumor enveloped 80% of the atrium of a 9-year-old male American Bulldog. The abdominocentesis procedure was performed to drain abdominal free fluid. Furosemide, enalapril and digoxin were prescribed as a treatment. Three months after the start of treatment, the animal presented intense dyspnea that culminated in the death of the patient, who later had the body sent for necropsy and subsequent tissue collection for histopathological analysis. Based on the immunohistochemical markers chromogranin and synaptophysin, the work here presented describes a case of a neuroendocrine tumor suggestive of paraganglioma involving a dog's heart. Discussion: Brachycephalic dogs, such as Boxers, Bulldogs and Boston Terriers, are predisposed to the development of paragangliomas. The genetic predisposition is related to hyperplasia of the carotid bodies generated by chronic hypoxia, resulting from obstructive processes in the airways. As the reported dog is an American Bulldog, the possibility of the tumor in question was reinforced. Paragangliomas are more frequent in males from nine to thirteen years of age, commonly associated with other endocrine neoplasms. It is also an important marker present in paragangliomas. In contrast to the literature, the report points to a neuroendocrine tumor suggestive of paraganglioma, without eliminating the possibility of an ectopic thyroid tumor, because although TTF-1 appears in most thyroid tumors, not all of them are present, although it is very suggestive. Treatment in question, attributed to the control of right congestive heart failure, although with little effect, denotes that the obstructive process of the return pathway, even with the use of drugs that prevent congestion, did not delay the evolution of the disease. However, it is observed that the treatment resources for these tumors, whether by surgical or chemotherapy option, also have few answers. The lack of literature on ectopic thyroid tumors influences the diagnosis of paraganglioma. Thus, clinical and complementary findings, mainly along with immunohistochemistry, were essential in the diagnosis of neuroendocrine tumor. After 3 months of the start of the treatment, the animal died.

Manifestaciones endocrinológicas en la enfermedad de Von Hippel-Lindau: revisión narrativa

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.

Presentación polipoide de un tumor neuroendocrino: reporte de caso y revisión de la literatura / Neuroendocrine Tumor Polypoid Presentation: Case Report and Literature Review

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.

Incidental pulmonary carcinoid tumorlet associated with lung cancer / Tumorlet carcinoide pulmonar incidental asociado a cáncer de pulmón

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.

Métodos de diagnóstico de laboratorio e imagenológicos en el síndrome de Zollinger-Ellison

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.

Paciente positiva a la COVID-19 operada de un insulinoma

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.

O prognóstico dos diferentes subtipos de carcinomas de esôfago neuroendócrinos: um estudo de base populacional / The prognosis of the different esophageal neuroendocrine carcinoma subtypes: a population-based study

ABSTRACT Background Neuroendocrine neoplasms are extremely rare and account for 0.4% to 2% of all malignant esophageal neoplasms. The burden of the neuroendocrine histological type on the patients' prognosis and survival is poorly debated. This study aimed to compare the survival rates of primary neuroendocrine neoplasms compared with adenocarcinoma and squamous cell carcinoma of the esophagus. Methods This is a retrospective cohort from the Surveillance, Epidemiology, and End Results Program database. Overall survival and cancer-specific survival were evaluated with Kaplan-Meier curves and logrank tests. Proportional Cox regression models were used to evaluate variables related to overall survival. Results After eligibility criteria, 66,528 patients were selected. The mean follow-up was 22.6 months (SD 35.6). Adenocarcinoma was predominant (62%), followed by squamous cell carcinoma (36%). Large cell carcinoma, small cell carcinoma, and mixed adenoneuroendocrine carcinoma each account for less than 1% each. On the long-term overall survival analysis, esophageal adenocarcinoma showed a better prognosis than all the other histologic types (P-value for logrank test <0.001). With adenocarcinoma as a reference, HR was 1.32 for large cell carcinoma (95%CI 1.2 to 1.45) and 1.37 for small cell carcinoma (95%CI 1.23 to 1.53). The HR was 1.22 for squamous cell carcinoma (95%CI: 1.2 to 1.24); and 1.3 for adenoneuroendocrine carcinoma (95%CI 1.01 to 1.66). For multivariate Cox regression analysis, besides age and stage, the neuroendocrine subtypes large cell carcinoma and small cell carcinoma were considered independent prognostic variables. Conclusion In the esophagus, large cell carcinoma and small cell carcinoma show poorer long-term survival rates than squamous cell carcinoma and adenocarcinoma.

RESUMO Contexto As neoplasias neuroendócrinas são extremamente raras e representam 0,4% a 2% de todas as neoplasias malignas do esôfago. A determinação prognóstica e avaliação de sobrevida para o tipo histológico neuroendócrino é pouco debatida. Este estudo teve como objetivo comparar as taxas de sobrevida de neoplasias neuroendócrinas primárias comparadas com adenocarcinoma e carcinoma espinocelular de esôfago. Métodos Este é um estudo coorte retrospectivo do banco de dados do Surveillance, Epidemiology, and End Results Program. A sobrevida global e a sobrevida específica do câncer foram avaliadas com curvas de Kaplan-Meier e testes de logrank. Modelos de regressão de Cox proporcional foram utilizados para avaliar as variáveis relacionadas à sobrevida global. Resultados Após critérios de elegibilidade, foram selecionados 66,528 pacientes. O seguimento médio foi de 22,6 meses (DP 35,6). O adenocarcinoma foi predominante (62%), seguido pelo carcinoma espinocelular (36%). Carcinoma de grandes células, carcinoma de pequenas células e carcinoma adenoneuroendócrino misto representam menos de 1% cada. Na análise de sobrevida global, o adenocarcinoma de esôfago apresentou um prognóstico melhor do que todos os outros tipos histológicos (P valor para teste de logrank < 0,001). Com adenocarcinoma como referência, HR foi de 1,32 para carcinoma de grandes células (IC95% 1,2 a 1,45) e 1,37 para carcinoma de pequenas células (IC95% 1,23 a 1,53). O HR foi de 1,22 para carcinoma espinocelular (IC95%: 1,2 a 1,24); e 1,3 para carcinoma adenoneuroendócrino (IC95% 1,01 a 1,66). Para a análise multivariada da regressão de Cox, além da idade e do estadiamento, os subtipos neuroendócrinos carcinoma de grandes células e carcinoma de pequenas células foram considerados variáveis prognósticas independentes. Conclusão No esôfago, o carcinoma de grandes células e o carcinoma de pequenas células apresentam menores taxas de sobrevida a longo prazo do que o carcinoma espinocelular e o adenocarcinoma.

68Ga-DOTATATE PET/CT versus 111In-octreotide scintigraphy in patients with neuroendocrine tumors: a prospective study / PET/CT com 68Ga-DOTATATE versus cintilografia com 111In-octreotide em pacientes com tumores neuroendócrinos: estudo prospectivo

Abstract Objective: To compare 68Ga-DOTA-DPhe1,Tyr3-octreotate (68Ga-DOTATATE) positron-emission tomography/computed tomography (PET/CT) findings with those of conventional 111In-octreotide scintigraphy in patients with neuroendocrine tumors (NETs). Materials and Methods: This was a single-center prospective study including 41 patients (25 males; mean age, 55.4 years) with biopsy-proven NETs who underwent whole-body 111In-octreotide scintigraphy and whole-body 68Ga-DOTATATE PET/CT. The patients had been referred for tumor staging (34.1%), tumor restaging (61.0%), or response evaluation (4.9%). Images were compared in a patient-by-patient analysis to identify additional lesions, and we attempted to determine the impact that discordant findings had on treatment planning. Results: Compared with 111In-octreotide scintigraphy, 68Ga-DOTATATE PET/CT revealed more lesions, the additional lesions typically being in the liver or bowel. Changes in management owing to the additional information provided by 68Ga-DOTATATE PET/CT occurred in five patients (12.2%), including intermodal changes in three (7.3%) and intramodal changes in two (4.9%). In addition, 68Ga-DOTATATE PET/CT yielded incidental findings unrelated to the primary NET in three patients (7.3%): Hürthle cell carcinoma of the thyroid, bowel non-Hodgkin lymphoma, and a suspicious breast lesion. Conclusion: We conclude that 68Ga-DOTATATE PET/CT is superior to conventional 111In-octreotide scintigraphy for the management of NETs because of its ability to determine the extent of the disease more accurately, which, in some cases, translates to changes in the treatment plan.

RESUMO Objetivo: Comparar os achados da PET/CT com 68Ga-DOTATATE em relação aos da cintilografia com 111In-octreotide em pacientes com tumores neuroendócrinos (TNEs). Materiais e Métodos: Estudo prospectivo unicêntrico incluindo 41 pacientes (25 homens; média de idade: 55,4 anos) com TNEs comprovados por biópsia submetidos a cintilografia de corpo inteiro com 111In-octreotide e PET/CT com 68Ga-DOTATATE. Os pacientes incluídos foram encaminhados para estadiamento do tumor (34,1%), reestadiamento (61,0%) ou avaliação da resposta (4,9%). As imagens foram comparadas para identificar lesões adicionais e o impacto dos achados discordantes no planejamento terapêutico. Resultados: Na comparação com a cintilografia com 111In-octreotide, a PET/CT com 68Ga-DOTATATE revelou mais lesões, mais frequentemente localizadas no fígado e intestino. Mudanças no tratamento devidas às informações adicionais reveladas pela PET/ CT ocorreram em 5/41 pacientes (12,2%), incluindo mudanças intermodalidade em três casos (7,3%) e intramodalidade em dois casos (4,9%). A PET/CT também identificou achados incidentais não relacionados ao TNE em 3/41 pacientes (7,3%), incluindo um carcinoma de células de Hürthle da tireoide, um linfoma não Hodgkin de intestino e uma lesão mamária suspeita. Conclusão: A PET/CT com 68Ga-DOTATATE é superior à cintilografia convencional com 111In-octreotide para o manejo de pacientes com TNEs, em virtude da sua capacidade de detectar a extensão da doença com mais precisão, o que se traduz, em alguns casos, em alterações terapêuticas.

Crisis carcinoide como una causa poco frecuente de choque distributivo / Carcinoid crisis as a rare cause of distributive shock

Los tumores neuroendocrinos son neoplasias que suelen tener un comportamiento clínico maligno, son provenientes de células entero cromafines y/o células productoras de gastrina. Según su origen anatómico, se clasifican en tumores del intestino anterior (comprometen estómago, duodeno, pulmones y páncreas), intestino medio (parte distal del duodeno) e intestino posterior (colon transverso hasta el recto). El síndrome carcinoide, presente en la mitad de los casos al momento del diagnóstico, se caracteriza por desencadenar episodios de diarrea, taquicardia, hipotensión, rubor (por el desarrollo de telangiectasias), y según la gravedad, valvulopatías cardiacas. Por otro lado, la crisis carcinoide, una complicación infrecuente, está relacionada con episodios de choque, que cuando ocurren son consecuencia de la liberación en la circulación sistémica de aminas vasoactivas, posterior a un evento desencadenante. A continuación, se presenta el caso de un paciente masculino con antecedente de un tumor neuroendocrino de intestino delgado, quien luego de ser llevado a embolización de metástasis hepáticas, presentó una crisis carcinoide, y finalmente un choque distributivo refractario a tratamientos convencionales, incluyendo octreotide y vasopresores, que culminó con la muerte del paciente. Se realiza la discusión del caso clínico y la presentación de la literatura disponible, donde se describe la epidemiología, patogénesis, diagnóstico, clínica y tratamiento de esta entidad

Neuroendocrine tumors are neoplasms that usually have a malignant clinical behavior. They arise from enterochromaffin-like and/or gastrin-producing cells. According to their anatomical location they can be classified as foregut tumors (af- fecting stomach, duodenum, lungs and pancreas), midgut tumors (affecting distal portion of the duodenum) and hindgut tumors (affecting transverse colon to rectum). Carcinoid syndrome, occurring in half of the cases at the time of diagnosis, is characterized by episodes of diarrhea, tachycardia, hypotension, flushing (due to telangiectasia), and heart valve disease depending on their severity. On the other hand, the carcinoid crisis, a rare complication that is related to episodes of shock, occur as a consequence of the release of vasoactive amines into the systemic circulation after a triggering event. Here we describe a case of a male patient with a history of neuroendocrine neoplasm, who after embolization of hepatic metastatic lesions presented a carcinoid crisis, ending with a vasodilatory shock, refractory to conventional treatment including octreotide and vasopressors, which resulted in the death of the patient. A discussion of the clinical report and a review of the available literature are presented, including the epidemiology, pathogenesis, diagnosis, clinical manifestations and treatment of this entity

Linfoma de Hodgkin anal primario y tumor neuroendocrino intestinal sincrónicos: a propósito de un caso y revisión de la literatura / Primary rectal Hodgkin's lymphoma and synchronous small bowel neuroendocrine tumor: Case report and literature review

Resumen La localización extranodal gastrointestinal del linfoma de Hodgkin comprende el 5% de todos los linfomas. Dentro de este grupo, el linfoma anal primario representa menos del 0,05%, siendo por tanto una entidad extremadamente rara. Por otro lado, los tumores neuroendocrinos son un grupo heterogéneo de neoplasias relativamente poco frecuentes, pero de localización fundamentalmente digestiva. La asociación entre un linfoma de Hodgkin de localización anal y un tumor neuroendocrino intestinal no ha sido descrita previamente en la literatura, pero no es en absoluto raro que los tumores neuroendocrinos puedan coexistir con otro tipo de neoplasias. Los autores presentan el caso infrecuente de presentación de linfoma Hodgkin de localización anal asociado a un tumor neuroendocrino intestinal en una paciente mujer de 74 años, describiéndose la clínica, resultados de pruebas complementarias y tratamiento recibido.

Abstract Extranodal gastrointestinal Hodgkin's lymphoma comprises 5% of all lymphomas. In this group, primary rectal lymphoma represents less than 0.05%; thus, it is an extremely rare entity. On the other hand, neuroendocrine tumors are a heterogeneous group of infrequent neoplasms, mainly of digestive location. The association between a rectal Hodgkin's lymphoma and an intestinal neuroendocrine tumor has not been previously described in the literature, but it is not at all uncommon for neuroendocrine tumors to coexist with other types of neoplasms. The authors present a rare case of rectal Hodgkin's lymphoma associated with an intestinal neuroendocrine tumor in a 74-year-old female patient, describing the symptoms, complementary test results, and treatment.

Dermatosis paraneoplásica asociada a tumor pancreático neuroendócrino / Paraneoplastic dermatosis associated with pancreatic neuroendocrine tumor

RESUMEN 19- Las dermatosis paraneoplásicas son un grupo heterogéneo de manifestaciones cutáneas que tienen fuerte asociación con patología maligna interna. Su patogenia es poco clara y no se conoce su prevalencia exacta. El tratamiento consiste en el abordaje terapéutico de la enfermedad subyacente. La importancia del conocimiento de las mismas radica en la posibilidad de realizar un diagnóstico temprano de una neoplasia. 20- Se presenta el caso clínico de un paciente con dermatosis paraneoplásica asociada a tumor neuroendócrino de páncreas.

ABSTRACT 24- Paraneoplastic dermatoses are an heterogeneous group of cutaneous manifestations that have a strong association with internal malignancy. Their pathogenesis is unclear and prevalence is unknown. Treatment consists of a therapeutic approach to the underlying disease. The importance of recognizingthem lies in the possibility of making an early diagnosis oftheneoplasm. 25- We report theclinical case of a patient with paraneoplastic dermatosis associated with a pancreatic neuroendocrine tumor.