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.

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)

Ileum neuroendocrine tumor: Case report and literature review

Ileal neuroendocrine tumors are rare primary epithelial neoplasm arising from enterochromaffin cells. A 65-year-old male presented with complaints of recurrent abdominal pain of 1 year duration. The pain was more aggravated in last 7 days associated with vomiting, loss of appetite and diarrhea. On radioimaging ultrasonography finding impression of small bowel mass lesion suggestive of ileac carcinoid tumor with ischemic bowel disease was given. Computerized tomograph abdomen pelvis showed a welldefined hypodense, round, hetergenous, enhancing mass measuring 2.6x1.6x1.2 cm., showing arterial enhancement in mesentery in close relation to ileum and metastatic lymph node mass- indicating likeness of neuroendocrine tumor. The surgical resection of ileum with mass and enlarged mesenteric nodes were done. On gross examination ileum specimen on cut open showed multiple, irregular mucosal thickenings with foci of ulceration. The submocosa showed single, circumscribed, round tumor measuring 3 x 2.5x1.5 cm. Cut section of tumor was yellowish, tan, well circumscribed and solid. The mesenteric lymph nodes were enlarged. The microscopic examination ileum showed a tumor composed of uniform, round cells having to oval nuclei with salt and pepper chromatin. Tumor cells were arranged in trabecular, nests, ribbons and in areas pseudo glandular pattern. Increased mitotic activity was noted (4/10 high power field). Mesenteric lymph nodes were involved by tumor. On histopathology reported as Well-differentiated neuroendocrine tumors (NET), G2, intermediate grade of ileum. The immunohistochemical stain were positive for synaptophysin, chromogranin A. The Ki-67 expression was <1%. Herewith we are presenting rare case of well-differentiated neuroendocrine tumor NET, G2, intermediate grade of ileum for its clinical, radio imaging ,pathological features and management.

Value of predictive liver metastasis in pancreatic neuroendocrine neoplasms based on ultrasonographic radiomics / 中华超声影像学杂志

Objective:To explore the predictive value of ultrasound-based radiomics for liver metastasis in pancreatic neuroendocrine tumors (pNEN).Methods:A retrospective analysis was conducted on clinical, pathological, and ultrasound data of 269 pNEN patients confirmed by pathology at Tianjin Medical University Cancer Institute and Hospital from January 2012 to June 2022, including 94 patients with liver metastasis and 175 without liver metastasis. The regions of interest (ROI) were delineated on the maximum diameter section of the tumor using ITKSNAP software, and radiomics features were extracted using Pyradiomics. Radiomics features with an intra-group correlation coefficient greater than 0.90 were retained, and the optimal features were selected using the maximum relevance minimum redundancy (MRMR) algorithm. The dataset was randomly divided into a training set and a validation set in a ratio of 7∶3, and the random forest algorithm (Rfs) was used to predict pNEN liver metastasis. Three models were constructed, including the clinical ultrasound model, the radiomics model, and the comprehensive model that combined clinical ultrasound and radiomics features. The predictive performance of different models for pNEN liver metastasis was analyzed using the ROC curve, and the predictive performance of different models was compared using the Delong test.Results:A total of 874 features were extracted from the ROI, and 12 highly robust radiomics features were retained for model construction based on inter- and intra-observer correlation grading and feature selection. The area under curve(AUC), sensitivity, specificity, and accuracy of the radiomics model, the clinical ultrasound model, and the comprehensive model for predicting liver metastasis in pNEN patients were 0.800, 0.574, 0.789, 0.714; 0.780, 0.596, 0.874, 0.777; and 0.890, 0.694, 0.874, 0.810, respectively. The Delong test showed that the comprehensive model had the best predictive performance, with an AUC superior to that of radiomics model ( Z=3.845, P=0.000 12) and clinical ultrasound model ( Z=3.506, P=0.000 45). Conclusions:The radiomics model based on ultrasound has good performance in predicting liver metastasis in pNEN, and the comprehensive model that combines clinical ultrasound and radiomics features can further improve the predictive performance of the model.

Minutes of seminar on 2023 multi-center cooperation project of liver transplantation for metastatic liver cancer in China / 器官移植

Led by Zheng Shusen, Academician of Chinese Academy of Engineering (CAE) from Zhejiang University, the first multi-center cooperation project of liver transplantation for metastatic liver cancer in China, gathering 28 liver transplantation centers nationwide, was launched in Shanghai. All participating experts conducted in-depth exchanges and discussions regarding four topics including inclusion criteria of liver transplantation for metastatic liver cancer, the risk assessment and prognostic evaluation of liver transplantation for metastatic liver cancer, perioperative medication of liver transplantation for metastatic liver cancer, and the implementation details of multi-center cooperation project. Questionnaires were distributed to reach consensus and pinpoint the directions, aiming to carry out high-quality and standardized clinical researches on liver transplantation for metastatic liver cancer in China.

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.

Clinical and pathological profile of gastric neuroendocrine tumors

Background: Gastric neuroendocrine tumors (G-NETs) are classified into well-differentiated NETs with three grades and poorly differentiated neuroendocrine carcinomas based on morphology and the Ki-67 index. Besides, G-NETs are broadly classified into four types based on clinical and pathophysiological features. Aim: To study clinical and pathological features of different types and grades of G-NET. Materials and Method: All G-NETs, diagnosed from January 2011 to December 2020, were included. Clinical presentation, peritumoral findings, lymph node status, and liver involvement were obtained and correlated with different grades and types of G-NETs. Results: NET was diagnosed in 88 cases. Tumors were graded as I, II, III, and carcinoma in 58, 14, 12, and 4 cases, respectively. Type I NET (49.2%) in the background of chronic atrophic gastritis was the most common type followed by type III (33.3%). Type I tumors were predominantly graded I (91.1%) and limited to the mucosa and submucosa. MEN-1-associated G-NET (type II) was seen in eight cases. All except one type II tumor was associated with ZES syndrome. Remarkably, peritumoral mucosa showed atrophy and intestinal metaplasia in 52.1% and 24.6% cases, respectively. Two cases were associated with adenocarcinoma. Lymph node metastasis was seen in all carcinoma and grade III cases. All carcinoma cases and 58.3% of grade III tumors showed liver metastasis. Conclusion: Biological behavior of G-NET varies with different types and grades of tumor. Typing and grading of G-NET should be done whenever possible to predict the aggressiveness of the tumor.

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.

Aquaporin-4 protein antibody-associated optic neuritis related to neuroendocrine tumor after receiving an inactive COVID-19 vaccine

Neuromyelitis optica (NMO), also known as Devic’s disease, is a rare, autoimmune, and recurrent demyelinating disorder that primarily affects the spinal cord and optic nerve. We report a case with recurrent optic neuritis caused by the paraneoplastic NMO spectrum disorder in the setting of a gastric neuroendocrine tumor 2 weeks after receiving an inactive COVID?19 vaccine.

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.

Large cell neuroendocrine carcinoma of cervix: A case report

Neuroendocrine carcinoma of the cervix is a rare aggressive tumor with a poor prognosis. Neuroendocrine carcinomas account for 1–1.5% of cervical cancer and around 12.5% were of large cell types. We report the case of a 55-year-old woman who came with complaints of pain abdomen and was reported as large cell neuroendocrine carcinoma of the cervix on biopsy. Immunohistochemistry was done which confirmed the diagnosis.

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.

Imaging features of intraductal pancreatic neuroendocrine tumor / 中华消化外科杂志

Objective:To explore the imaging features of intraductal pancreatic neuro-endocrine tumor (PNET).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 17 patients with intraductal PNET who were admitted to the First Affiliated Hospital of Naval Medical University (Changhai Hospital of Shanghai) from January 2013 to October 2020 were collected. There were 7 males and 10 females, aged (47±13)years. Preoperative contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) of the pancreas was performed on patients. Observation indicators: (1) imaging features of intraductal PNET, including ① imaging features of CT and ② imaging features of MRI; (2) treatment and histopathological examination of intraductal PNET. Measurement data with normal distribution were described as Mean± SD and count data were described as absolute numbers. Results:(1) Imaging features of intraductal PNET. ① Imaging features of CT: 17 patients underwent preoperative contrast-enhanced CT of pancreas. There were 9 cases with tumor located in the head of the pancreas, 5 cases with tumor located in the neck of the pancreas and 3 cases with tumor located in the body and tail of the pancreas. The tumor diameter of the 17 patients was (8.7±2.5)mm, with a range of 5.2?15.5 mm. The tumor shape was round-like in the 17 patients. All the 17 patients showed isodensity on plain CT and markedly enhancement in arterial, venous and portal phases on enhanced CT. The degree of enhancement of tumor was higher than surrounding normal pancreatic parenchyma. All tumors of 17 patients were located at the truncation of main pancreatic duct (MPD) dilation, showing abrupt change in caliber of MPD without the "beak sign". The diameter of dilated MPD was (11.4±5.3)mm, with a range of 4.5?22.5 mm. Other imaging manifestations of the 17 patients included 11 cases with pancreatic parenchymal atrophy, 1 case with retention cyst, 1 case with choledochal dilation, 1 case with calcification, and all cases without cystic degeneration or hemorrhage. ② Imaging features of MRI: preoperative contrast-enhanced MRI was performed in 14 patients. Five cases showed slightly low signal but 9 cases showed unclear on T1-weighted imaging. Five cases showed low signal, 2 cases showed slightly high signal but 7 cases showed unclear on T2-weighted imaging. Of the 14 patients, 9 cases showed diffusion limited on diffusion weighted imaging and 5 cases showed unlimited diffusion. Nine cases showed marked enhancement in tumor higher than in normal pancreatic parenchyma, but 5 cases were unclear on contrast-enhanced MRI. (2) Treatment and histopathological exmination of intraductal PNET: all the 17 patients underwent surgical treatment, including 9 cases with pancreaticoduodenectomy, 4 cases with distal pancreatectomy and splenectomy, 4 cases with pancreatic segmentectomy. Postoperative histopatho-logical examination results showed 10 cases of G1 and 7 cases of G2, including 1 case of G2 with lymph node metastasis, 1 case of G2 with lymph node and liver metastasis. The pathological gross showed that the tumor body was mainly located in the pancreatic duct and blocked the pancreatic duct, with upstream pancreatic dilation. There were pancreatic acinar atrophy and fibrous tissue hyperplasia. The tumor was grayish-yellow or brownish red, solid, medium in texture and well-defined with the surrounding tissues. Microscopically, the tumor of 17 patients was mainly located in the pancreaic duct and invaded into surrounding pancreatic parenchyma. The cells of tumor were polygonal with a central nucleus, but the mitosis was rare. The cytoplasm was eosinophilic or hyaline. The tumor stroma was mainly collagen fiber with abundant capillary network.Conclusions:The imaging features of intraductal PNET are small size, marked enhancement on contrast-enhanced CT and MRI. The tumor obstructs the MPD with distal MPD dilation and pancreatic parenchyma atrophy.

Study on the risk factors of type 1 gastric neuroendocrine tumor in 123 cases of autoimmune gastritis / 中华消化杂志

Objective:To investigate the risk factors of type 1 gastric neuroendocrine tumor (g-NET) in patients with autoimmune gastritis(AIG).Methods:From September 1, 2016 to February 28, 2022, 123 patients with AIG visited the First Affiliated Hospital of Zhengzhou University were retrospectively enrolled, including 37 cases with type 1 g-NET and 86 cases without type 1g-NET. The clinical data, serological indicators, and endoscopic manifestation of all the patients were analyzed, including the age at the time of AIG diagnosis (hereinafter referred to as the age at diagnosis), levels of gastrin 17 and pepsinogen Ⅰ (PGⅠ), presence or absence of gastric fundus and gastric body polyps, etc. The independent risk factors of type 1 g-NET in AIG patients were analyzed by univariate and multivariate logistic regression. The receiver operating characteristic curve (ROC) was plotted to analyze the optimal cut-off value, sensitivity and specificity of the independent risk factors in predicting type 1 g-NET in AIG patients. Independent sample t test, Mann-Whitney U test and chi-square test were used for statistical analysis. Results:Compared with those of the AIG patients without type 1 g-NET, the age at diagnosis of AIG patients with type 1 g-NET was younger ((57.49±11.16) years old vs. (48.49±10.96) years old), the level of gastrin 17 was higher (200.21 ng/L, 121.85 ng/L to 244.40 ng/L vs. 244.40 ng/L, 182.50 ng/L to 248.02 ng/L), and the proportion of patients with gastric fundus and gastric body polyps was higher(18.6%, 16/86 vs. 56.8%, 21/37), and the differences were statistically significant( t=-4.13, Z=-3.06, χ2=17.90; P<0.001, =0.002 and <0.001). The results of univariate logistic analysis showed that the age at diagnosis ( OR=0.931, 95% confidence interval (95% CI)0.895 to 0.967), gastrin 17( OR=1.012, 95% CI 1.005 to 1.019), PGⅠ( OR=0.974, 95% CI 0.950 to 0.998)and gastric fundus and gastric body polyps( OR=5.742, 95% CI 2.461 to 13.399)were the influencing factors of type 1 g-NET in AIG patients ( P<0.001, =0.001, =0.033 and <0.001). The results of multivariate logistic regression analysis indicated that the age at diagnosis( OR=0.921, 95% CI 0.881 to 0.964), gastrin 17( OR=1.011, 95% CI 1.001 to 1.020), gastric fundus and gastric body polyps( OR=7.696, 95% CI 2.710 to 21.857)were the independent risk factors of type 1 g-NET in AIG patients ( P<0.001, =0.024 and <0.001). The results of ROC analysis demonstrated that the optimal cut-off values for the age at diagnosis and gastrin 17 in predicting type 1 g-NET were 56.50 years old and 206.40 ng/L, respectively; with sensitivity of 83.8% and 70.3%, respectively, and specificity of 54.7% for both ( P<0.001 and=0.003). Conclusion:The age at diagnosis< 56.50 years old, gastrin 17>206.40 ng/L and the presence of gastric fundus and gastric body polyps are independent risk factors of type 1 g-NET in AIG patients.

Metastatic risk assessment and related treatment progress of paraganglioma and pheochromocytoma / 中华内分泌外科杂志

Paraganglioma and pheochromocytoma (PPGL) is an uncommon neuroendocrine tumor with variable metastatic potential which is related to multiple factors, and has a clear genetic background. Metastatic PPGL requires systemic intervention after failure of surgical treatment, but the overall efficacy is poor. In this review, the related literatures were reviewed to describe PPGL from the histological origin and name evolution, genetic background and metastasis risk, metastasis risk prediction and prognosis assessment, challenges in the clinical staging and progress in the systemic treatment.

Laboratory variables as predictors of progression in gastroenteropancreatic neuroendocrine tumors in different lines of antineoplastic treatments

ABSTRACT Objective: To determine the association of red cell blood counts, and liver panel tests to predict outcomes in patients with gastroenteropancreatic neuroendocrine tumors who underwent systemic antineoplastic treatments. Methods: Patients with gastroenteropancreatic neuroendocrine tumors in systemic treatment were assessed according to laboratory tests within the same period. Progression free survival was determined by the period between the beginning of treatment and the date of progression. We used conditional models (PWP model) to verify the association between laboratory tests and tumor progression. The level of significance used was 5%. Results: A total of 30 treatments given to 17 patients in the intention-to-treat population were evaluated. Treatment included octreotide, lanreotide, everolimus, lutetium, and chemotherapy. We had statistically significant results in chromogranin A, neutrophils and platelets-to-lymphocyte ratio. The risk of progression increases by 2% with the addition of 100ng/mL of chromogranin A (p=0.034), 4% with the increase of 100 neutrophil units (p=0.006), and 21% with the addition of 10 units in platelets-to-lymphocyte ratio (p=0.002). Conclusion: Chromogranin A, neutrophils and platelets-to-lymphocyte ratio were associated with disease progression during systemic treatment in gastroenteropancreatic neuroendocrine tumors. Further prospective studies with larger cohorts are necessary to validate our findings.

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

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

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

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

Tumor neuroendocrino de colon derecho en un paciente con colitis ulcerativa: a propósito de un caso / Right colon neuroendocrine tumor in a patient with ulcerative colitis: A case study

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.

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.