A rare cause of chronic dysphagia: pulmonary inflammatory myofibroblastic tumor with distal esophagus invasion

Background Inflammatory myofibroblastic tumor (IMT) is rare intermediate tumor, which happens mostly in children and young adults. Case presentation Reported is the successful treatment of a 29-year-old man presented with progressively dysphagia and weight loss. No other abnormal symptoms were observed. The contrast enhanced computed tomography (CT) revealed a dumbbell-shaped lesion between lung and esophagus. Finally, it was pathologically diagnosed as pulmonary IMT invading to the distal esophagus after operation. The patient underwent partial esophagectomy and left lower lobectomy, and was discharged on 10th postoperative day. Conclusions IMT is a rare lesion that usually occurs in the lung, but pulmonary IMT with distal esophagus invasion has not been described previously. Discriminating untypical symptom, completed resection, pathological expertise and closed follow-up will reach the successful diagnosis and treatment.

Incidental Gastrointestinal Stromal Tumors – What can we learn from them?

Introduction/Objective Gastrointestinal stromal tumors (GIST), albeit very rare overall, with approximately 5,000 new diagnoses yearly in the US, represent the most common mesenchymal tumor involving the GI tract. particularly the stomach and small bowel. Much progress has been made in the past two decades in terms of classification and targeted therapy of GIST, however, their behavior is still quite challenging and unpredictable, and their prognosis remains guarded. Methods/Case Report We reviewed our institutional experience with GIST over the past decade and identified 80 GIST in total, 5 of which were incidental findings, meaning that they were detected as a secondary diagnosis in a specimen that was obtained for a different pathology. All 5 tumors were less than 2 cm in greatest dimension and showed less than 5 mitoses/ 50HPF, which put them into the “no risk of malignant progression category”. As a matter of fact, all five tumors were less than 1 cm (ranging from 0.2-0.7cm), while the mean size overall for GIST is 6 cm, ranging from 0.2-40 cm. They were all c-kit and DOG-1 positive, and 3 out of 5 incidental GIST were diagnosed in association with an adenocarcinoma of the GI tract (gastric or distal esophagus). Three of the 5 incidental GIST were located in the stomach, one in the distal esophagus and one in the sigmoid; unsurprisingly, two gastric and one esophageal GIST were associated with poorly differentiated adenocarcinoma of the stomach or distal esophagus. The mean age at diagnosis in our series was 72.4 yo, while reported mean age for GIST ranges between 60-65yo. All but one of the patients with incidental GIST were males, while there seems to be no gender predilection in GIST in general. The two benign cases associated with incidental, “no risk” GIST were perforated diverticulitis and sleeve gastrectomy for morbid obesity. Review of the stained slides showed no apparent increase of interstitial cells of Cajal in the blocks containing the tumor. Results (if a Case Study enter NA) NA Conclusion In summary, we report a small series of 5 incidental GIST diagnosed over a span of 10 years in our institution. Although a rare tumor overall, with an annual incidence of 10-20 new cases/ million, incidental GIST is even more rare, in our case representing 6.25% out of the total number of cases diagnosed in a decade. Increased awareness of the incidental GIST and closer pathological assessment may throw more light into the pathogenesis of GIST in general.

Challenges in Determining the Role of Microbiome Evolution in Barrett’s Esophagus and Progression to Esophageal Adenocarcinoma

Esophageal adenocarcinoma (EAC) claims the lives of half of patients within the first year of diagnosis, and its incidence has rapidly increased since the 1970s despite extensive research into etiological factors. The changes in the microbiome within the distal esophagus in modern populations may help explain the growth in cases that other common EAC risk factors together cannot fully explain. The precursor to EAC is Barrett’s esophagus (BE), a metaplasia adapted to a reflux-mediated microenvironment that can be challenging to diagnose in patients who do not undergo endoscopic screening. Non-invasive procedures to detect microbial communities in saliva, oral swabs and brushings from the distal esophagus allow us to characterize taxonomic differences in bacterial population abundances within patients with BE versus controls, and may provide an alternative means of BE detection. Unique microbial communities have been identified across healthy esophagus, BE, and various stages of progression to EAC, but studies determining dynamic changes in these communities, including migration from proximal stomach and oral cavity niches, and their potential causal role in cancer formation are lacking. Helicobacter pylori is negatively associated with EAC, and the absence of this species has been implicated in the evolution of chromosomal instability, a main driver of EAC, but joint analyses of microbiome and host genomes are needed. Acknowledging technical challenges, future studies on the prediction of microbial dynamics and evolution within BE and the progression to EAC will require larger esophageal microbiome datasets, improved bioinformatics pipelines, and specialized mathematical models for analysis.

720 CONVENTIONAL AND SIMPLE METHODS OF MEASURING MEAN NOCTURNAL BASELINE IMPEDANCE SHOW EXCELLENT AGREEMENT

Mean nocturnal baseline impedance (MNBI) is a measurement that shows promise in investigating reflux disease by reflecting esophageal mucosal integrity. The conventional method of measuring MNBI is time-consuming and operator-dependent. A new, simple method of measuring MNBI has been suggested. This study aimed to report MNBI measurements by both conventional and simple methods in a cohort of laryngopharyngeal reflux (LPR) and gastro-esophageal reflux disease (GERD) patients to validate the simple measurement method. Methods 187 patients were divided into LPR (n = 105) or GERD (n = 82) groups by predominant symptom profile, and underwent off-therapy impedance-pH monitoring. MNBI was blindly measured by both conventional and simple methods, and values were correlated. Bland–Altman plots were constructed to assess mean differences and to identify bias between the two measurement methods. Results For the two measurement methods, mean difference in the distal esophagus was −89 (±328) ohms, in the proximal esophagus was −6 (±653) ohms, and in the pharynx was 128 (±577) ohms. There was strong correlation between conventional and simple MNBI values, with r = 0.940 in the distal esophagus, r = 0.463 in the proximal esophagus, and r = 0.712 in the pharynx (all p < 0.001). Conclusion There was excellent agreement between conventional and simple methods of measuring MNBI, with no evidence of proportional bias. Conventional and simple MNBI values correlated excellently in the distal esophagus, and moderately well in the proximal esophagus and pharynx. This study supports the use of the simple method of measuring MNBI to enhance diagnosis of reflux disease.

500 THE PATHOLOGICAL GASTROESOPHAGEAL REFLUX IN PATIENTS WITH CYSTIC FIBROSIS

The presence of gastroesophageal reflux in patients after lung transplantation rises the risk of posttransplantant complications. The aim of our study was to evaluate the severity of gastroesophageal reflux (GER) in patients with cystic fibrosis before lung transplantation. Methods 14 patients with cystic fibrosis, who refferred to our Centre, were included in this study, mean age 28,86 ± 2,5 (6 males and 8 females). The diagnosis of cystic fibrosis was established at the age of 1–5 y.o. according to clinic, sweet test and genetic tests. All patients were performed upper endoscopy, X-ray, esophageal manometry and 24 h impedance monitoring. PPI treatment was excluded for 7 days before monitoring. Pathologic GER was established when total number of refluxes in distal esophagus was more than 75, the total number of refluxes in proximal esophagus was more than 20% of those in the distal part. Results According to endoscopy, none of patients had erosions of the esophagus. Five patients had ineffective motility and one—diffuse esophageal spazm. 56% of patients had pathologic number of refluxes in distal esophagus. 80% subjects had pathologic refluxes in proximal esophagus. The mean number of total refluxes in distal part was 54,93 ± 5,7, in proximal part—27,64 ± 2,6 (it is 40% of those in distal part). The distribution of acid and non-acid refluxes in patients with pathologic refluxes was 2:1. We didn’t reveal any correlation between number of refluxes, ineffective motility and gastric emptying. Conclusion despite normal upper endoscopy, 56% of patients with cystic fibrosis have pathologic gastroesophageal reflux. Besides, the study showed high propagation of GER to proximal esophagus in 80% of subjects, which may play role in posttransplantation period.

Symptomatic Heterotopic Gastric Mucosa in Distal Esophagus

Introduction Heterotopic gastric mucosa (HGM) in esophagus is commonly noted as an inlet patch at endoscopy. We describe a rare patient with symptomatic distal esophageal HGM. Case Report A 40-year-old male presented with retrosternal pain and marked odynophagia for the last 4 weeks without any history of ingestion of antibiotics, foreign body, or corrosive. Endoscopy showed abrupt circumferential transition to salmon pink mucosa at 35 cm from incisors. From 35 to 41 cm, there were areas of polypoid edematous thickening with few superficial ulcers of 1 to 3 mm. Squamous epithelium was visualized at narrow band imaging from 41 cm to the Z-line at 43 cm with no hiatus hernia. Biopsy showed gastric-type mucosa with parietal cells without dysplasia. Serology for cytomegalovirus and human immunodeficiency virus was negative. He was managed with proton pump inhibitors (PPIs) and prokinetics and improved symptomatically. Follow-up endoscopy at 3 months demonstrated healing of ulcers with persistence of HGM and pseudopolyps. He remains well on maintenance with PPI at 1-year follow-up. Conclusion Symptomatic HGM in distal esophagus is rare and can be differentiated from Barrett’s esophagus histologically and by presence of squamous epithelium between HGM and stomach. Inflammatory mass lesions may develop and mimic esophageal malignancy. Symptoms are largely due to acid production and usually respond to PPI.

Correlation of SOX-2 and NOTCH1 copy numbers with vimentin expression in orthotopic patient-derived xenografts of cardioesophageal cancer in immunodeficient mice.

e15034 Background: Cardioesophageal cancer is one of the most common tumors affecting the mucosa of the cardiac stomach and distal esophagus. Despite the variety of treatment strategies and chemotherapy agents, the prognosis for the patients remains poor. The purpose of the study was to analyze the relative copy numbers of SOX-2 and NOTCH1 and vimentin expression in orthotopic patient-derived xenografts of cardioesophageal cancer. Methods: The model of cardioesophageal cancer was created in Balb/c Nude mice with surgical bioptates of moderately differentiated adenocarcinoma obtained from a donor with infiltrative ulcerative cancer of the lower third of the esophagus with a transition to the cardiac stomach into the distal esophagus of Balb/c Nude mice. The index of proliferative activity in the bioptates was assessed by IHC. The relative copy numbers of SOX-2 and NOTCH1 and vimentin expression were determined by Real-Time qPCR. Results: Expression of vimentin was absent in tissues of the donor tumor. The levels of vimentin expression statistically significantly increased in xenografts (1+ and 3+). The SOX-2 and NOTCH1 relative copy numbers were statistically significantly increased in tissues of the donor tumor (0.9 and 0.7), compared to xenografts (1.5±0.03 and 1.7±0.03). Molecular genetic analysis demonstrated an association between an increased vimentin expression and changes in the relative copy numbers of SOX-2 and NOTCH1 (p = 0.013 and p = 0.0001). Conclusions: The relative copy numbers of SOX-2 and NOTCH1 genetic loci was associated with increased expression of the epithelial-mesenchymal transition marker vimentin in tumor tissue samples and changed with each new generation of orthotopic xenografts.