Endoscopic vacuum assisted closure therapy for esophagopericardial fistula in a 16-year-old male: A case report.

BACKGROUND: Esophagopericardial fistula (EPF) is a rare, life-threatening condition with limited scientific literature and no established management guidelines. This case report highlights a successful multidisciplinary approach and the innovative use of endoscopic vacuum assisted closure (endoVAC) therapy in treating this complex condition. CASE SUMMARY: A 16-year-old male with a history of esophageal atresia and colon interposition presented with progressive chest pain, fever, and dyspnea. Imaging revealed an EPF with associated pleural and pericardial effusions. Initial management with an esophageal stent failed, prompting the use of an endoVAC system. The patient underwent multiple endoVAC device changes and received broad-spectrum antibiotics and nutritional support. The fistula successfully closed, and the patient recovered, demonstrating no new symptoms at a 6-month follow-up. CONCLUSION: EndoVAC therapy can effectively manage EPF, providing a minimally invasive treatment option.

Primary adenoid cystic carcinoma in the esophagus.

Esophageal adenoid cystic carcinoma (EACC) is an exceedingly rare malignant tumor constituting only 0.2% of all esophageal tumors. The tumor exhibits aggressive behavior, composed histologically of ductal and modified myoepithelial cells. We report a case of a 69-year-old female with a diagnosis of an EACC by preoperative endoscopic biopsy. Thoracoscopy esophagectomy was carried out. However, pleural metastasis was found. Therefore, surgical resection of the esophageal tumor was not carried out. The patient underwent an uneventful recovery, followed by palliative treatment and ongoing chemoradiotherapy. EACC is uncommon but exhibits a more aggressive nature compared to its counterparts in the head and neck region. Dysphagia associated with gastroesophageal reflux disease is a common symptom. The duration from symptom onset to diagnosis is typically short. Treatment options include surgical resection, chemotherapy, and radiotherapy, with surgery being the preferred initial approach despite high operative mortality. Prognosis remains inconclusive, with some studies associating poor outcomes with lymph node metastasis and vascular invasion, while others report better survival rates. EACC presents diagnostic and therapeutic challenges due to its rarity and aggressive nature. Prognostic considerations remain unclear, emphasizing the need for further research and accumulated cases to delineate optimal treatment. The presented case demonstrates a 1-year survival with systemic palliative care, contributing to the evolving knowledge surrounding EACC.

El carcinoma adenoide quístico primario de esófago (EACC) es un tumor maligno excepcionalmente raro que constituye solo el 0.2% de todos los tumores esofágicos. El tumor exhibe un comportamiento agresivo, compuesto histológicamente por células ductales y mioepiteliales modificadas. Presentamos el caso de una mujer de 69 años con diagnóstico de un EACC mediante biopsia endoscópica preoperatoria. Se realizó una esofagectomía por toracoscopia. Sin embargo, se encontró metástasis pleural. Por lo tanto, no se llevó a cabo la resección quirúrgica del tumor esofágico. La paciente tuvo una recuperación sin complicaciones, seguida de tratamiento paliativo y radioquimioterapia continua. El EACC es poco común, pero exhibe una naturaleza más agresiva en comparación con sus contrapartes en la región de la cabeza y el cuello. La disfagia asociada con la enfermedad por reflujo gastroesofágico es un síntoma común. La duración desde el inicio de los síntomas hasta el diagnóstico suele ser corta. Las opciones de tratamiento incluyen la cirugía, quimio y radioterapia, siendo la cirugía la preferida a pesar de la alta mortalidad operatoria. El pronóstico es inconcluso, algunos estudios asocian resultados pobres con metástasis e invasión vascular, mientras que otros informan mejores tasas de supervivencia. El EACC presenta desafíos diagnósticos y terapéuticos debido a su rareza y naturaleza agresiva. El pronóstico sigue siendo poco claro, lo que enfatiza la necesidad de más investigación para delinear el tratamiento óptimo. El caso presentado demuestra una supervivencia de un año con cuidados paliativos sistémicos, contribuyendo al conocimiento en evolución sobre el EACC.

Endoscopic submucosal dissection vs. endoscopic mucosal resection in the treatment of early Barrett's neoplasia: Systematic review and meta-analysis.

OBJECTIVES: Endoscopic resection is the preferred approach to treat early Barrett's neoplasia, reducing the need for surgical interventions. However, the best choice between endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) remains unclear. The study aimed to compare the efficacy and safety of EMR vs. ESD for early Barrett's neoplasia. METHODS: An electronic search was conducted in MEDLINE, Central Cochrane, EMBASE, and LILACS until November 2023. Studies comparing ESD vs. EMR in the treatment of patients with early Barrett's neoplasia were included. This study was performed according to the Preferred Report Items for Systematic Reviews and Meta-Analyses guidelines. The ROBIN-I tool was used to analyze the risk of bias and GRADE to measure the quality of the evidence. RESULTS: A total of 9352 patients from 15 observational studies were included. Patients undergoing ESD had significantly higher rates of en-bloc (odds ratio [OR] 25.96, 95% confidence interval [CI] 13.82, 48.74; I2 = 52%; P < 0.00001) and R0 (OR 5.10, 95% CI 3.29, 7.91; I2 = 73%; P < 0.00001) with a higher risk of adverse events, including bleeding, stricture formation, and perforation. In a subgroup analysis of patients who did not receive radiofrequency ablation, ESD had a lower recurrence rate than EMR (OR 0.22, 95% CI 0.05, 0.94; I2 = 88%; P = 0.04). CONCLUSION: Endoscopic submucosal dissection is more effective than EMR in treating early Barrett's neoplasia at the expense of higher adverse events rates.

Swallowing Safety after Remote sub-total Esophagectomy: How Important is Tongue Pressure?

The factors related to oropharyngeal dysphagia after remote esophagectomy (greater than five months) remain unclear. This study aimed to assess patient perception of dysphagia, maximum anterior isometric pressure (MAIP), maximum posterior isometric pressure (MPIP), lingual swallowing pressure (LSP) and radiographic physiological components of the oral and pharyngeal phases of swallowing in patients who are post remote sub-total esophagectomy (SE). Patient perception of dysphagia was assessed using the Eating Assessment Tool (EAT-10). MAIP, MPIP, and LSP were measured using the Iowa Oral Performance Instrument. Videofluoroscopy was used to assess the physiologic components of swallowing with the Modified Barium Swallow Impairment Profile (MBSImP) and the Penetration-Aspiration Scale (PAS). Ten patients were included in the study (53.2% male; mean age 54.5 ± 18.0). The mean postoperative time was 30 months (range, 5.0-72 months). Seven patients had elevated EAT-10 scores (> 3). All patients demonstrated impaired oropharyngeal swallowing on at least three MBSImP components (range 3-12) and two patients aspirated (PAS 8). There was a significant difference in MAIP values when comparing patients with normal versus impaired laryngeal elevation and epiglottic movement (p < 0.001). MPIP values were significantly different in patients with normal versus impaired epiglottic movement as well as normal versus elevated PAS scores (p < 0.001). Decreased lingual pressure and physiological changes in swallowing coexist after SE. Our results indicate that the decrease in tongue strength may be one of the factors related to unsafe swallow. The assessment of lingual pressure provides diagnostic value and should be incorporated as part of a comprehensive assessment in this population.

Knockdown of WISP1/DKK1 restrains phenotypic plasticity in esophageal squamous cell carcinoma by suppressing epithelial-mesenchymal transition and stemness.

OBJECTIVE: Wnt-induced signaling protein 1 (WISP1) and Dickkopf-1 (DKK1) are highly expressed in esophageal squamous cell carcinoma (ESCC), but no direct connection was identified between them. Phenotypic plasticity is a hallmark of ESCC. This research intended to identify the association between WISP1 and DKK1 and their roles in the phenotypic plasticity of ESCC. METHODS: Genes differentially expressed in esophageal carcinoma were analyzed in the GEO database, followed by analyses of GO and KEGG enrichment to screen the hub gene. WISP1 expression and DKK1 secretion was assessed in ESCC tissues and cells. The tumor xenograft and in vivo metastasis models were established by injecting ESCC cells into nude mice. Functional deficiency and rescue experiments were conducted, followed by assays for cell proliferation, migration/invasion, stemness, epithelial-mesenchymal transition (EMT), and apoptosis, as well as tumor volume, weight, proliferation, stemness, and lung metastasis. The binding relationship and co-expression of WISP1 and DKK1 were determined. RESULTS: WISP1 and DKK1 were upregulated in ESCC cells and tissues, and WISP1 was enriched in the cell stemness and Wnt pathways. WISP1 knockdown subdued proliferation, migration/invasion, EMT activity, and stemness but enhanced apoptosis in ESCC cells. WISP1 knockdown restrained ESCC growth, proliferation, stemness, and metastasis in vivo. WISP1 bound to DKK1 in ESCC. DKK1 overexpression abolished the repressive impacts of WISP1 knockdown on the malignant behaviors of ESCC cells in vitro and of ESCC tumor in vivo. CONCLUSION: Knockdown of WISP1/DKK1 restrains the phenotypic plasticity in esophageal squamous cell carcinoma by suppressing epithelial-mesenchymal transition and stemness.

Esophageal achalasia: A case report in an adolescent. / Acalasia esofágica: a propósito de un caso en una adolescente.

Esophageal achalasia is an uncommon disease in pediatrics. With an insidious clinical presentation, diagnosis is delayed. Here we describe a case of esophageal achalasia in a 16-year-old girl, with the typical delay in consultation and diagnosis. Although pneumatic balloon dilatation has been described as the best therapeutic option for type II achalasia, it was ineffective in our patient and she required Heller extramucosal myotomy with gastroesophageal fundoplication for reflux.

La acalasia esofágica es una patología infrecuente en la edad pediátrica. Su presentación clínica es insidiosa, lo que causa un retraso en el diagnóstico. Se presenta un caso de acalasia esofágica en una niña de 16 años, que tuvo la demora característica en la consulta y el diagnóstico. A pesar de que se describe la dilatación neumática con balón como la mejor opción terapéutica para la acalasia tipo II, en nuestra paciente fue inefectiva y requirió miotomía extramucosa de Heller con funduplicatura gastroesofágica antirreflujo.

Disparities in esophageal cancer care: a population-based study.

BACKGROUND: Vulnerable populations potentially have a worse prognosis for cancer. The present study aimed to identify individual and municipal characteristics of access to health, including education, use of health insurance, gross domestic product per capita (GDPpc), and urban aspects, which could impact the prognosis of patients with esophageal cancer. METHODS: Data on urban concentration, administrative hierarchy, GDPpc, individual patient characteristics, and access to healthcare were collected from national and state public databases spanning between 2013 and 2022. The study included cities in the state of Sao Paulo, Brazil. Independent variables such as GDPpc, urban concentration, municipal administrative hierarchy, health insurance status, education level, and individual cancer and patient characteristics were evaluated against the outcomes of overall survival (OS), likelihood of undergoing surgical treatment, and time-to-treatment initiation. RESULTS: A total of 9280 patients with esophageal cancer (85% squamous cell carcinoma and 15% adenocarcinoma) treated in 42 cities were included in the study. In univariate analysis, higher education (hazard ratio [HR] = 0.6; P < .001), female gender (HR = 0.85; P < .001), and having private health insurance (HR = 0.65; P < .001) were identified as protective factors for OS in esophageal cancer. After adjusting for other variables in multivariate analysis, higher education (HR = 0.77; P = .009), female gender (HR = 0.82; P < .001), and private insurance (HR = 0.65; P < .001) remained protective factors. GDPpc was not associated with OS. Urban concentration and hierarchy influenced the likelihood of receiving surgical treatment. Patients from high urban concentrations had shorter time-to-treatment initiation intervals. CONCLUSION: Populations at risk, particularly those with limited access to education and healthcare, face a worse prognosis for esophageal cancer.

Nomograms for prognosis prediction in esophageal adenocarcinoma: realities and challenges.

Prognostic assessment is of great significance for individualized treatment and care of cancer patients. Although the TNM staging system is widely used as the primary prognostic classifier for solid tumors in clinical practice, the complexity of tumor occurrence and development requires more personalized probability prediction models than an ordered staging system. By integrating clinical, pathological, and molecular factors into digital models through LASSO and Cox regression, a nomogram could provide more accurate personalized survival estimates, helping clinicians and patients develop more appropriate treatment and care plans. Esophageal adenocarcinoma (EAC) is a common pathological subtype of esophageal cancer with poor prognosis. Here, we screened and comprehensively reviewed the studies on EAC nomograms for prognostic prediction, focusing on performance evaluation and potential prognostic factors affecting survival. By analyzing the strengths and limitations of the existing nomograms, this study aims to provide assistance in constructing high-quality prognostic models for EAC patients.

Esophageal Mucosal Impedance Assessment for the Diagnosis of Gastroesophageal Reflux Disease.

Background/Aims: Diagnosing gastroesophageal reflux disease (GERD) is sometimes challenging because the performance of available tests is not entirely satisfactory. This study aims to directly measure the esophageal mucosal impedance during upper gastrointestinal endoscopy for the diagnosis of GERD. Methods: Sixty participants with typical symptoms of GERD underwent high-resolution esophageal manometry, 24-hour multichannel intraluminal impedance-pH monitoring, upper gastrointestinal endoscopy, and mucosal impedance measurement. Mucosal impedance measurement was performed at 2, 5, 10, and 18 cm above the esophagogastric junction during gastrointestinal endoscopy using a specific catheter developed based on devices described in the literature over the last decade. The patients were divided into groups A (acid exposure time < 4%) and B (acid exposure time ≥ 4%). Results: The mucosal impedance was significantly lower in group B at 2 cm (2264.4 Ω ± 1099.0 vs 4575.0 Ω ± 1407.6 [group A]) and 5 cm above the esophagogastric junction (4221.2 Ω ± 2623.7 vs 5888.2 Ω ± 2529.4 [group A]). There was no significant difference in the mucosal impedance between the 2 groups at 10 cm and 18 cm above the esophagogastric junction. Mucosal impedance value at 2 cm > 2970 Ω resulted in a sensitivity of 96.4% and a specificity of 87.5% to exclude GERD. Conclusions: Direct measurement of mucosal impedance during endoscopy is a simple and promising method for diagnosing GERD. Individuals with an abnormal acid exposure time have lower mucosal impedance measurements than those with a normal acid exposure time.

Construction of prognostic nomogram based on the SEER database for esophageal cancer patients.

Currently, the incidence of esophageal cancer continues to rise around the world. Because of its good early prognosis, it is of great significance to establish an effective model for predicting the survival of EC patients. The purpose of this study was to predict survival after diagnosis in Esophageal Cancer (EC) patients by constructing a valid clinical nomogram. In this study, 5037 EC patient samples diagnosed from 2010 to 2015 were screened by accessing the SEER database, and 8 independent prognostic factors were screened by various methods, and Cox multivariate regression was included to construct a prognostic model and nomogram for esophageal cancer. to estimate esophageal cancer recurrence and overall survival. Calibration of the nomogram predicted probabilities of 1-year, 3-year and 5-year survival probability, which were closely related to actual survival. In conclusion, this study validated that the column-line graphical model can be considered an individualized quantitative tool for predicting the prognosis of patients with EC in order to assist clinicians in making therapeutic decisions.

Diagnostic differences in high-resolution esophageal motility in a large Mexican cohort based on geographic distribution.

High-resolution esophageal manometry [HRM] has become the gold standard for the evaluation of esophageal motility disorders. It is unclear whether there are HRM differences in diagnostic outcome based on regional or geographic distribution. The diagnostic outcome of HRM in a diverse geographical population of Mexico was compared and determined if there is variability in diagnostic results among referral centers. Consecutive patients referred for HRM during 2016-2020 were included. Four major referral centers in Mexico participated in the study: northeastern, southeastern, and central (Mexico City, two centers). All studies were interpreted by experienced investigators using Chicago Classification 3 and the same technology. A total of 2293 consecutive patients were included. More abnormal studies were found in the center (61.3%) versus south (45.8%) or north (45.2%) P < 0.001. Higher prevalence of achalasia was noted in the south (21.5%) versus center (12.4%) versus north (9.5%) P < 0.001. Hypercontractile disorders were more common in the north (11.0%) versus the south (5.2%) or the center (3.6%) P.001. A higher frequency of weak peristalsis occurred in the center (76.8%) versus the north (74.2%) or the south (69.2%) P < 0.033. Gastroesophageal junction obstruction was diagnosed in (7.2%) in the center versus the (5.3%) in the north and (4.2%) in the south p.141 (ns). This is the first study to address the diagnostic outcome of HRM in diverse geographical regions of Mexico. We identified several significant diagnostic differences across geographical centers. Our study provides the basis for further analysis of the causes contributing to these differences.

A new formula proposal for placing pH-impedance catheters in pediatric patients.

INTRODUCTION: Esophageal pH-impedance monitoring is a tool for diagnosing gastroesophageal reflux in children. The position of the pH catheter is essential for a reliable reading and the current formulas for calculating catheter insertion length are not completely accurate. The aim of the present study was to develop a new formula for adequate insertion of the pH catheter. MATERIAL AND METHODS: A cross-sectional study was conducted on children that underwent pH-impedance monitoring and later radiographic control, to calculate the correct catheter insertion length. The documented variables were age, sex, weight, height, naris to tragus distance, tragus to sternal notch distance, sternal notch to xiphoid process distance, and initial insertion length determined by the Strobel and height interval formulas. A multivariate regression analysis was carried out to predict the final insertion length. Regression ANOVA and Pearson's adjusted R-squared tests were performed. RESULTS: Forty-five pH-impedance studies were carried out, 53% of which were in males. The age and weight variables were not normally distributed. In the initial regression model, the variables that did not significantly correlate with the final insertion length were: sex (P 0.124), length determined by the Strobel or height interval formulas (P 0.078), naris to tragus distance (P 0.905), and tragus to sternal notch distance (P 0.404). The final equation: 5.6 + (height in cm * 0.12) + (sternal notch to xiphoid process distance * 0.57) produced an R2 of 0.93 (P 0.000). CONCLUSIONS: This formula can be considered a valid option for placement of the pH-impedance monitoring catheter in pediatrics.

Baveno VII Criteria for the Exclusion of Esophageal Varices in a Peruvian Population: Cross-Sectional Study / Criterios de Baveno VII para la exclusión de várices esofágicas en una población peruana: estudio transversal

Abstract Introduction: Esophageal varices represent one of the main complications in patients with liver cirrhosis. The main objective was to determine the diagnostic performance of the new Baveno VII criteria to exclude the presence of esophageal varices in compensated advanced chronic liver disease (cACLD), in an independent Peruvian population. Materials and methods: A cross-sectional study, including patients with cACLD, upper digestive endoscopy, and transient hepatic elastography from January 2017 to December 2019. Results: The mean age was 59.4 (12.9) years, while the mean measurement of liver stiffness was 27.21 (14.6) kPa. The prevalence of esophageal varices was 85.6%; non-alcoholic fatty liver disease (NAFLD) (63.6%) was the most prevalent etiology, followed by viral hepatitis (14.4%). For esophageal varices exclusion, Baveno VII criteria for all etiologies demonstrated adequate sensitivity and negative predictive value (sensitivity: 96.7%; 95% confidence interval [CI]: 92.3%-98.8%; negative predictive value: 76.9%, 95% CI: 56.4% -91%). However, better diagnostic performance was found when applying the Baveno VII criteria without considering NAFLD patients (sensitivity: 98.4%, 95% CI: 79.2% -99.2%; negative predictive value: 90.9%, 95% CI: 79.2% -99.2%). This would prevent 14% of endoscopic studies with a 9% risk of failing to detect esophageal varices. Conclusions: The Baveno VII criteria present good diagnostic performance for the exclusion of esophageal varices, especially in patients with cACLD without NAFLD, in an independent Peruvian population.

Resumen Introducción: Las várices esofágicas representan una de las principales complicaciones en pacientes con cirrosis hepática. El objetivo principal fue determinar el rendimiento diagnóstico de los nuevos criterios de Baveno VII para excluir la presencia de várices esofágicas en la enfermedad hepática crónica compensada (cACLD), en una población peruana independiente. Materiales y métodos: Estudio transversal que incluyó a pacientes con cACLD, endoscopia digestiva alta y elastografía hepática transitoria desde enero de 2017 hasta diciembre de 2019. Resultados: La edad media fue de 59,4 (12,9) años, mientras que la medición media de rigidez hepática fue de 27,21 (14,6) kPa. La prevalencia de várices esofágicas fue del 85,6%; la enfermedad metabólica asociada al hígado graso (MASLD) (63,6%) fue la etiología más prevalente, seguida de la hepatitis viral (14,4%). Para la exclusión de várices esofágicas, los criterios de Baveno VII para todas las etiologías demostraron una sensibilidad y un valor predictivo negativo adecuados (sensibilidad: 96,7%; intervalo de confianza [IC] del 95%: 92,3%-98,8%; valor predictivo negativo: 76,9%, IC 95%: 56,4%-91%). Sin embargo, se encontró un mejor rendimiento diagnóstico al aplicar los criterios de Baveno VII sin considerar a los pacientes con MASLD (sensibilidad: 98,4%, IC 95%: 79,2%-99,2%; valor predictivo negativo: 90,9%, IC 95%: 79,2%-99,2%). Se evitaría el 14% de estudios endoscópicos con un riesgo del 9% de no detectar várices esofágicas. Conclusiones: Los criterios de Baveno VII presentan buen rendimiento diagnóstico para la exclusión de várices esofágicas, especialmente en pacientes con cACLD sin MASLD, en una población peruana independiente.

Esophageal Melanocytosis.

Esophageal melanocytosis is a rare entity defined by the proliferation of a melanocytic basal layer of the esophageal squamous lining and deposition of melanin in the esophageal mucosa. Esophageal melanocytosis is considered a benign entity of unknown etiology; however, it has been reported as a melanoma precursor. We report a case of esophageal melanocytosis in a diabetic and hypertensive 67-year-old male with recurrent dizziness and syncope for the past 6 months. Given his complaint of dyspepsia, he underwent an upper gastrointestinal endoscopy, in which an esophageal biopsy revealed the diagnosis of esophageal melanocytosis. The definitive diagnosis of esophageal melanocytosis can only be made by histological analysis. The histologic differential diagnoses include melanocytic nevi and malignant melanoma. Therefore, they need to be ruled out.

Hipertensión portal no cirrótica por enfermedad vascular porto-sinusoidal: presentación de un caso y revisión de la literatura / Non-cirrhotic portal hypertension due to porto-sinusoidal vascular disease: Case presentation and literature review

La enfermedad vascular porto-sinusoidal es una causa infrecuente de hipertensión portal no cirrótica, fue descrita recientemente y es poco diagnosticada por el desconocimiento entre los médicos. Se considera en casos de hipertensión portal clínicamente significativa, en ausencia de cirrosis. El diagnóstico se basa en los hallazgos de la biopsia. El pronóstico de la enfermedad es mejor que el de los pacientes cirróticos, y el tratamiento es similar al de la hipertensión portal y al de las complicaciones que presentan los pacientes con cirrosis. Se presenta el caso de una paciente con várices esofágicas con estudios de imágenes no compatibles con cirrosis y hallazgos específicos en la biopsia de enfermedad vascular porto-sinusoidal. Este caso muestra el ejercicio diagnóstico en un caso de enfermedad vascular porto-sinusoidal de una paciente de Colombia, así como el resultado de las intervenciones terapéuticas y la evolución en el tiempo.

Porto-sinusoidal vascular disease is an uncommon cause of non-cirrhotic portal hypertension. It was recently described and is rarely diagnosed due to lack of knowledge among doctors. It is considered in cases of clinically significant portal hypertension in the absence of cirrhosis, and the diagnosis is based on biopsy findings. The prognosis of the disease is better than that of cirrhotic patients, and the treatment is similar to that of portal hypertension, including the management of complications associated with cirrhosis. We present the case of a patient with esophageal varices, whose imaging studies were not compatible with cirrhosis, alongside specific biopsy findings of porto-sinusoidal vascular disease. This case illustrates the diagnostic process in a patient from Colombia with portosinusoidal vascular disease, as well as the outcomes of therapeutic interventions and the patient´s evolution over time.

How appropriate is treating patients diagnosed with advanced esophageal cancer with anticancer drugs? A multicenter retrospective cohort Spanish study.

AIM: To assess the appropriateness of systemic oncological treatments (SOT) provided to patients diagnosed with advanced esophageal cancer (EC) across a group of participating hospitals. METHODS: Multicenter, retrospective cohort study in five Spanish hospitals including newly confirmed advanced EC cases between July 1, 2014, and June 30, 2016, with a 5-year follow-up. RESULTS: We identified 157 patients fulfilling the inclusion criteria (median age: 65 years, 85.9% males). Most patients, 125 (79.6%) were treated at least with one active treatment, and 33% received two or more lines of SOT. The 1-, 2- and 5-year overall survival rates were 30.3% [95%CI: 23.8, 38.7], 14.0% [95%CI: 9.3, 21.0], and 7.1% [95% CI: 3.8, 13.1] respectively, and the median survival time 8 months (95% CI: 6, 19) for stages IIIb IIIc and 7 months (95% CI: 5, 9) for stage IV. Clinical stage, receiving more than one line of SOT, and treatment with radiotherapy accelerated the time to death (0.4, 0.9-, and 0.8-times shorter survival respectively, p < 0.05). Better performance status (ECOG < 2) extended survival time by 2.2 times (p = 0.04). Age < 65 years (OR 9.4, 95% CI 3.2, 31.4, p < 0.001), and being treated in one particular hospital (OR 0.2, 95% CI 0.0, 0.8, p < 0.01) were associated with the administration of two or more lines of SOT. Altogether, 18.9% and 9.0% of patients received chemotherapy in the last four and two weeks of life, respectively. Moreover, 2.5% of patients were prescribed a new line of chemotherapy during the last month of life. The proportion of all patients who did not have access to palliative care reached 29.3%, and among those who had access to it, 34.2% initiated it in the last month of life. CONCLUSION: A high proportion of advanced EC patients receive many treatments not based on sound evidence and they do not benefit enough from palliative care services. The most accepted appropriateness indicators point out that some of the analyzed patients could have been overtreated. This study provides important insights into the quality of care provided to advanced EC, and furthermore, for giving valuable insight and opportunities for improvement.

Genomic Contributors to Esophageal Atresia and Tracheoesophageal Fistula: A 12 Year Retrospective Review.

OBJECTIVE: To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management. STUDY DESIGN: A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed. RESULTS: There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive). CONCLUSIONS: EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF.

Clinical features and treatment outcomes of PD-1 inhibitor therapy in elderly patients (≥ 65 years) with advanced esophageal squamous cell carcinoma: a real-world study.

PURPOSE: This study aims to determine the clinical features and outcomes of PD-1 inhibitor therapy as the initial treatment in patients aged 65 years or older with locally advanced or metastatic esophageal squamous cell carcinoma (ESCC). MATERIALS AND METHODS: The retrospective study conducted a comprehensive analysis of elder patients diagnosed with locally advanced or metastatic ESCC who underwent combined immunochemotherapy in the first affiliated hospital of Nanchang University from January 2019 to January 2023. The main efficacy measures were the objective response rate (ORR) and progression-free survival (PFS). The secondary endpoints were disease control rate (DCR) and overall survival (OS). The evaluation of safety was based on the assessment of adverse events (AEs). RESULTS: A total of 88 patients were enrolled in the study. All patients received PD-1 inhibitors combined with chemotherapy including taxane and platinum as the first-line treatment. The median PFS was 6.2 months (95% CI: 5.1-7.3), and the median OS was 15.3 months (95% CI: 12.9-17.7). The ORR and DCR were 42.0% and 72.7%, correspondingly. 68 (77.3%) patients experienced treatment-related adverse events (TRAEs) of various degrees, with neutrophil count decreased (21, 23.9%) being the most frequent. TRAEs of grade 3 or 4 occurred in 13 (14.8%) patients. CONCLUSION: The study demonstrated that individuals older than 65 years with locally advanced or metastatic ESCC have a survival benefit from the first-line treatment of PD-1 inhibitors combined therapy, with a manageable safety profile.