“Clip coupled with an elastic ring” internal traction for endoscopic submucosal dissection of a rectal neuroendocrine tumor: a junior endoscopist experience

A 64-year-old female was found a rectal neuroendocrine tumor (NET) for cancer screening examination. Endoscopic ultrasonography (EUS) revealed a hypoechoic lesion (8.3*6.6 mm) originating from the submucosa layer. “Clip coupled with elastic ring” internal traction for endoscopic submucosal dissection (ESD) was used to remove the NET according to the procedure removal of a duodenal tumor1. The procedures are following: 1. Marking around the lesion with a margin of approximately 5 mm. 2. Submucosal injection and circumference incision around the lesion. 3. Applied clip coupled with elastic ring internal traction. 4. Submucosal injection. 5.Precise dissection was performed with the NET being en bloc resection. 5. Closed the mucosal defect. Finally, the Histopathology confirmed a neuroendocrine tumor. (AU)

Carcinoma neuroendocrino de células grandes en la unión esofagogástrica. Una entidad extremadamente infrecuente / Large cell neuroendocrine carcinoma at the esophagogastric junction. An extremely rare entity

Presentamos el tercer caso descrito hasta la fecha de carcinoma neuroendocrino de células grandes localizado en la unión esofagogástrica (CNECG). Los tumores neuroendocrinos esofágicos representan el 0,03-0,05% de todos los tumores malignos esofágicos. Dentro de los TNE esofágicos. El CNECG representa el 1% de los TNE esofágicos. Este tipo tumoral se caracteriza por elevar unos marcadores determinados: sinaptofisina, cromogranina A y CD56. De hecho, el 100% de los pacientes tendrán cromogranina o sinaptofisina, o al menos uno de estos tres marcadores. A su vez, el 78% tendrán invasión linfovascular y el 26% invasión perineural. Únicamente el 11% de los pacientes tendrán un estadio I-II, circunstancia que implica un curso agresivo y un peor pronóstico.(AU)

Gastric variceal bleeding as a form of presentation of a pancreatic neuroendocrine tumor / Sangrado por varices gástricas como forma de presentación de un tumor neuroendocrino pancreático

NETs (neuroendocrine tumors) constitute a heterogeneous group of epithelial-type neoplasms with a predominantly neuroendocrine differentiation. Although the most common locations are the pancreas, digestive tract, and lung, this type of neoplasm can arise in virtually any organ in the body. They are rare tumors with a wide variety of clinical presentations. Symptomatic tumors are more frequent in younger patients and present at more advanced pathological stages. We present the case of a 42-year-old male with idiopathic splenomegaly and bicytopenia (anaemia and thrombocytopenia) under study by haematology department who was admitted due to an episode of melena and hemoglobin of 4.5 mg/dl. Isolated gastric varices (IGV1) with red spots were confirmed at gastroscopy and endoscopic variceal obturation using cyanoacrylate was performed in two sessions. An endoscopic ultrasonography was performed, showing thrombosis of the splenic vein extending towards the splenoportal confluence with anechoic serpiginous structures outside and inside the gastric wall suggestive of collateral circulation with gastric varices (GV). An increase in portal caliber was observed, with no signs of liver cirrhosis. Computed tomography confirms the findings. Two months/week/days later he was readmitted with rebleeding signs after starting anticoagulant treatment, so it was decided to perform a splenectomy due to failure of the endoscopic treatment. Histology revealed infiltration of the spleen by a well-differentiated neuroendocrine tumor (NET). Gallium PET/CT and Octreotid scan showed uptake in the body and tail of the pancreas with positivity for somatostatin receptors previously undetected by other means. Finally, treatment was completed with distal pancreatectomy and splenoportal axis trombectomy with vascular esplenic resection and the patient was discharged from hospital.(AU)

SEOM-GETNE clinical guidelines for the diagnosis and treatment of gastroenteropancreatic and bronchial neuroendocrine neoplasms (NENs) (2022)

'Neuroendocrine neoplasms (NENs) are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise mainly due to an improvement on diagnostic techniques and awareness. Earlier detection, along with steadfast improvements in therapy, has led to better prognosis over time for advanced gastrointestinal and pancreatic neuroendocrine tumors. The aim of this guideline is to update evidence-based recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification, and therapeutic options, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are reviewed and discussed, and treatment algorithms to guide therapeutic decisions are provided (AU)

Papel de los factores inflamatorios sistémicos en los tumores neuroendocrinos gastroenteropancreáticos (TNE-GEP) tratados con péptidos marcados con radionúclidos (PRRT): de la biología a la teragnosis / Role of systemic inflammatory factors in gastroenteropancreatic neuroendocrine tumors (GEP-NETs) treated with peptide receptor radionuclide therapy (PRRT): From biology to theragnosis

Objetivo Los índices inflamatorios sistémicos se han validado como indicadores de inflamación sistémica como marcadores predictivos de mal pronóstico para diversas enfermedades oncológicas. Sin embargo, se desconoce el impacto pronóstico de los marcadores de inflamación sistémica en pacientes con tumores neuroendocrinos gastroenteropancreáticos (TNE-GEP) tratados con péptidos marcados con radionúclidos (PRRT). Métodos Realizamos un estudio observacional, retrospectivo, multicéntrico de 40 pacientes con TNEs-GEP y TNE de origen desconocido tratados con PRRT entre el 2016 y el 2020. Los marcadores inflamatorios sistémicos se calcularon de la siguiente manera: relación neutrófilos a linfocitos (NLR)=recuento de neutrófilos/recuento de linfocitos, relación de monocitos a linfocitos (MLR)=recuento de monocitos/recuento de linfocitos, relación de plaquetas a linfocitos (PLR)=recuento de plaquetas/recuento de linfocitos, relación de albúmina a linfocitos (ALR)=niveles de albúmina/recuento de linfocitos y relación derivada de neutrófilos a linfocitos (dNLR)=recuento de neutrófilos/(recuento de leucocitos – recuento de neutrófilos). Se utilizaron datos analíticos basales pretratamiento y después de la segunda dosis para el cálculo de los distintos índices. Resultados La mediana de edad fue de 63 años (rango 41-85), el 55% eran hombres. Los valores de corte de referencia para NLR fueron 2,61, para MLR 0,31, para PLR 110,14, para ALR 2,39 y para dNLR 1,71. Los valores de corte después de la segunda dosis fueron, para NLR 2,3, para MLR 0,3, para PLR 131,61, ALR 4,16 y dNLR 1,48. La mediana de la sobrevivencia libre de progresión (SLP) fue de 21,7 meses (IC del 95%: 10,7-32,8 m) y la supervivencia global (SG) fue de 32,1 meses (IC del 95%: 19,6-44,7 m), la SLP fue más corta en pacientes con NLR elevado (p=0,001), ALR (0,03) y dNLR (p=0,001) en el análisis basal. La tasa de control de enfermedad (DCR) fue del 81% y la tasa de respuesta objetiva (ORR) del 18% (AU)

Aim Systemic inflammatory factors have been validated as indicators of ongoing systemic inflammation that could be predictive markers of poor prognosis for oncological outcomes. However, the prognostic impact of systemic inflammation markers is unknown in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs) treated with peptide receptor radionuclide therapy (PRRT). Methods We conducted an observational, retrospective, multicentric study of 40 patients with GEP or unknown origin NETs treated with PRRT between 2016 and 2020. The systemic inflammatory markers were calculated as follows: neutrophil to lymphocyte ratio (NLR)=neutrophil count/lymphocyte count, monocyte to lymphocyte ratio (MLR)=monocyte count/lymphocyte count, platelet to lymphocyte ratio (PLR)=platelet count/lymphocyte count, albumin to lymphocyte ratio (ALR)=albumin levels/lymphocyte count and derived Neutrophil to Lymphocyte ratio (dNLR)=neutrophil count/(leucocytes count – neutrophils count). Baseline analysis and after the second dose were used for the calculation of different ratios. Results The median age was 63 years (range 41–85), 55% were male. The baseline cut-off values for NLR were 2.61, for MLR 0.31, for PLR 110.14, for ALR 2.39 and for dNLR 1.71. The cut-off values after the 2° dose were, for NLR 2.3, for MLR 0.3, for PLR 131.61, ALR 4.16, and dNLR 1.48. Median progression-free survival (PFS) was 21.7 months (95% CI 10.7–32.8 months) and overall survival (OS) was 32.1 months (95% CI 19.6–44.7 months), PFS was shorter in patients with elevated NLR (P=0.001), ALR (0.03), and dNLR (P=0.001) in baseline analysis. DCR was 81% and ORR 18%. Conclusions In GEP or unknown origin NETs treated with PRRT, we have identified the predictive and prognostic impact of baseline systemic inflammatory factors (AU)

PET/TC en endocrinología nuclear. Resultados de la encuesta difundida por las distintas redes sociales de la SEMNIM en España / PET/CT in nuclear endocrinology: Results of the survey diffused through different social networks of the SEMNIM in Spain

Objetivo Desde el Grupo de Trabajo de Endocrinología de la SEMNIM, se planteó la necesidad de conocer el uso actual de la tecnología PET/TC aplicada en el campo de la endocrinología. El objetivo de la encuesta era obtener una fotografía instantánea del uso de la PET/TC en endocrinología nuclear, con el fin de conocer si está siendo adecuadamente utilizada y detectar posibles necesidades. Material y métodos Durante el primer trimestre del 2022, se analizaron los datos obtenidos de una encuesta que se difundió a través de distintas redes sociales a lo largo de la segunda mitad del 2021. Se recogieron datos sobre el uso de las distintas técnicas PET/TC en el carcinoma diferenciado de tiroides, el carcinoma medular de tiroides, los tumores neuroendocrinos y el hiperparatiroidismo. Resultados Un total de 15 centros respondieron la encuesta. El 79% de los hospitales utilizan la 18F-FDG PET/TC en el diagnóstico y/o seguimiento del carcinoma diferenciado de tiroides (media de exploraciones anuales: 36,9; rango 10-100). El 85% utilizan la 18F-DOPA PET/TC para el estudio de recidiva bioquímica de carcinoma medular de tiroides (media estudios anuales: 7,8; rango 2-20). El 77% utilizan la 18F-DOPA PET/TC para el estudio de los tumores neuroendocrinos: el 77% utilizan la 18F-DOPA PET/TC (media de 10 exploraciones anuales; rango 2-30) y el 69% utilizan el 68Ga-DOTA-SA (media de 24,7 exploraciones anuales; rango 2-127). El 79% utilizan la 18F-colina PET/TC para el estudio del hiperparatiroidismo (media de 30,1 exploraciones anuales; rango 10-120). Conclusiones El uso de la técnica PET/TC en endocrinología aún no está generalizado, sin embargo, vimos que las indicaciones en las cuales se utiliza son, en general, las reportadas en los distintos consensos (AU)

Aim To know the current use of the PET/CT technology applied in the field of endocrinology, the Endocrinology Working Group of SEMNIM proposed conducting a survey. The objective was to obtain a snapshot of the use of PET/CT in nuclear endocrinology, to know if it is being used properly and detect possible needs. Material and methods During the first quarter of 2022, we analyzed the data obtained from a survey that was distributed through different social networks throughout the second half of 2021. The survey asked for the use of the different PET/CT techniques available in Spain in different endocrinological pathologies like differentiated thyroid carcinoma, medullary thyroid carcinoma, neuroendocrine tumors and hyperparathyroidism. Results A total of 15 centers responded to the survey. A percentage of 79 of hospitals used 18F-FDG PET/CT in the diagnosis and/or follow-up of differentiated thyroid carcinoma (mean annual studies: 36.9; range 10–100); 85% used 18F-DOPA PET/CT for the study of biochemical recurrence of medullary thyroid carcinoma (mean annual studies: 7.8; range 2–20); 77% used 18F-DOPA PET/CT for the study of neuroendocrine tumors: 77% used 18F-DOPA PET/CT (mean of 10 scans per year; range 2–30) and 69% used 68Ga-DOTA-SA (mean of 24.7 studies per year; range 2–127); 79% used 18F-choline PET/CT for the study of hyperparathyroidism (mean of 30.1 annual studies; range 10–120). Conclusions We detected that the use of the PET/CT technique in endocrinology is not yet widespread, however, we saw that the indications in which it is used are, in general, those reported in the different consensus (AU)

Hallazgo casual de un paraganglioma paraaórtico / An incidentally discovered paraaortic paraganglioma

Introducción: los paragangliomas son tumores neuroendocrinos derivados de células cromafi nes. Son entidadesmuy infrecuentes que se localizan típicamente en el cuello o en la base del cráneo. presentamos el caso de unparaganglioma paraaórtico, una ubicación inusual que cuenta con escasos reportes en la literatura. Caso clínico: mujer de 78 años que presenta una masa retroperitoneal hallada de forma casual durante el estudiopreoperatorio de una neoplasia ginecológica. ante la sospecha de un tumor del estroma gastrointestinal o unschawnnoma retroperitoneal, se indica su extirpación quirúrgica. Durante la operación se objetiva una masa muyvascularizada adherida a la cara anterior de la aorta, cuya resección provoca en la paciente un cuadro de crisishipertensiva y taquicardia asociadas a inestabilidad hemodinámica, que cede tras completar su exéresis. el estudioanatomopatológico posterior confi rma la sospecha diagnóstica intraoperatoria de paraganglioma paraaórtico. Discusión: los paragangliomas localizados en el abdomen suelen ser productores de catecolaminas y puedendesarrollar clínica adrenérgica de forma espontánea o inducida por fármacos, ejercicio o manipulación quirúrgica,como sucedió en nuestro caso. Un diagnóstico certero es clave para planificar un tratamiento adecuado quepermita reducir las posibles complicaciones en este tipo de pacientes.(AU)

Introduction: paragangliomas are neuroendocrine tumours derived from chromaffin cells. they are very rareentities that are typically located in the neck or at the base of the skull. We present the case of a para-aortic para-ganglioma, an unusual location that has few reports in the literature. Case report: a 78-year-old woman with a retroperitoneal mass found incidentally during the preoperative study fora gynecological neoplasm. Given the suspicion of a gastrointestinal stromal tumour or a retroperitoneal schawn-noma, surgical removal of it was indicated. Intraoperatively, a highly vascularized mass adhered to the anteriorface of the aorta was observed, whose resection caused a hypertensive crisis and tachycardia associated withhemodynamic instability, which subsided after completing the exeresis. the subsequent anatomopathologicalstudy confirmed the intraoperative diagnostic suspicion of paraaortic paraganglioma. Discussion: paragangliomas located in the abdomen are usually catecholamine producers and can develop adr-energic symptoms spontaneously or induced by drugs, exercise, or surgical manipulation, as occurred in our case.an accurate diagnosis is the key to planning an appropriate treatment that allows reducing possible complicationsin this type of patient.(au)