bluuebunny/crossref_metadata_2025_split huggingface dataset:
1797 Intramural Esophageal Dissection: A Rare Cause of Hematemesis
Authors: Al-Jashaami Layth , Rezaie Aida , Abidali Hussein , Alishahi Yasmin 
Published: October 2019

INTRODUCTION:
Intramural esophageal dissection (IED) was first described by Williams in 1957. It is a rare form of acute esophageal trauma defined as an injury in the esophageal submucosal layer extending between the mucosa and muscular layer. Patients may present with acute chest pain, epigastric pain, dysphagia, retching or hematemesis. It most cases, IED is associated with trauma during food ingestion.

CASE DESCRIPTION/METHODS:
A 91-year-old Veteran presented to the Phoenix VA Health Care System’s Emergency Department ED with a 1-day history of retching followed by dysphagia after pill ingestion. He subsequently developed massive hematemesis described as a ‘basin full of bright red blood’ and lightheadedness. His medications were notable for aspirin 81 mg daily, but no anticoagulant. A computed tomography scan of the chest showed severely thickened esophageal wall without gas or fluid in the mediastinum. Urgent esophagogastroduodenoscopy (EGD) was performed revealing a blood clot in the oropharynx and around the Endotracheal tube, and a large organized hematoma without active bleeding in the mid to distal esophagus adherent to the esophageal wall. Multiple attempts to remove the blood clot were made by snare and endoscopic basket without success. A repeat follow-up EGD was performed demonstrating the same hematoma with successful removal via distal attachment cap. Beneath the hematoma, a 10-cm, non-circumferential, deep submucosal tear was seen from mid- to distal esophagus associated with small spots of bleeding (Figure 1). Nine endoscopic clips were placed (Resolution clip, Boston Scientific) (Figure 2). The patient did not develop any further hematemesis, and his dysphagia improved throughout admission.

DISCUSSION:
We present a case of spontaneous IED involving 10cm of the esophagus in a 91-year-old Veteran treated with endoscopic zipper clipping to achieve successful hemostasis. While conservative management is reported in most cases in the form of bowel rest, this case demonstrates the significance of endoscopic evaluation when hemodynamic instability is present. For this Veteran, the hematoma provided a natural form of hemostasis until endoscopic clips could be placed to stop bleeding.
1798 An Endoscopic Vacuum-Assisted Closure Technique Modified Through the Overtubes for Patients With Esophageal Anastomotic Leaks
Authors: Kwon Hyeonwoo , Kim Seongmin , Lee Jun Su , Cho Byungha , Choi Jongwook , Kim Ki Bae , Kim Si-Wook , Kim Dohun , Kim Hyoung Woo , Han Joung-Ho , Yoon Soon Man , Youn Sei Jin , Chae Hee Bok , Park Seon Mee 
Published: October 2019

INTRODUCTION:
Endoscopic vacuum-assisted closure (E-VAC) therapy is safe and effective for esophageal anastomotic leaks. However, the repetitive exchange of sponge tubes is technically demanding.

CASE DESCRIPTION/METHODS:
We designed an E-VAC technique, modified through the use of overtube, to make E-VAC therapy safer and more comfortable for both endoscopists and patients. Intraluminal E-VAC therapies were used for the treatment of post-operative thoracic esophageal leaks in five cases of esophageal cancer. After overtube insertion, sponge tube was inserted through the overtube, while keeping biopsy forcep inside the nasogastric tube (NGT), and was positioned at intraluminal leakage site under endoscopic guidance. NGT was repositioned from the mouth to the nostrils using carrier tube and a controlled negative pressure was applied. Sponge tube was regularly changed twice a week until the complete closure of leaks. Technical and clinical success rates were analyzed to evaluate the safety and efficacy of this technique. The modified E-VAC therapy was applied in five selected cases for a mean of 13.8 days (range, 7-28 days) with a mean of 3.2 sponge tube changes (2-6 changes) for anastomotic leaks that were, on average, 1.0 cm (0.5-2 cm). The mean procedural time was 14.9 min (12-30 min) and no E-VAC therapy-related complications occurred. All patients were discharged, on average, after 44.4 days (range, 14-95 days) of hospitalization and two cases were treated with additional esophageal metal stents after six and two sponge tube changes, respectively.

DISCUSSION:
Our E-VAC therapy technique, modified via inserting sponge tubes through overtube, is effective and safe for patients with thoracic esophageal anastomotic leaks.
1805 AFP Value of 2400 With No HCC; Metastatic Esophageal Cancer With Neuroendocrine Differentiation With Rare Increase of AFP
Authors: Pacha Faris , Skrove Jamie L. , Sobrado Javier , Rana Zaid 
Published: October 2019

INTRODUCTION:
Adenocarcinoma (AC) accounts for 60 percent of all esophageal cancers in the USA. Dysphagia and weight loss are among the most common symptoms. In the US, an estimated 17,290 cases of esophageal cancer will be diagnosed each year, and 15,850 deaths are expected from the disease. In the US, smoking and excessive alcohol consumption, account for approximately 90 percent of the total cases of esophageal CA. The majority of cases are located near the GEJ and are associated with endoscopic evidence of Barrett's Esophagus (BE). AC arising in BE may present as an ulcer, a nodule, an altered mucosal pattern, or no visible endoscopic abnormality. Early AC not associated with BE arises from an ulcer, plaque, or nodule near the GEJ.

CASE DESCRIPTION/METHODS:
Patient is a 67-year-old male who presented to the E.D. for worsening epigastric pain and dysphagia to solids with no history of EGD. The patient is a 40 pack year smoker and non-drinker. The patient denies other symptoms other than epigastric dull pain. During his hospital stay imaging demonstrated severe lymphadenopathy near the aorta along with multiple liver lesions with the largest being 3.5cm. The patient received a CT Chest that demonstrated pulmonary nodules with speculated appearance suggested of metastatic disease, mediastinal lymphadenopathy, and multiple liver lesions greatest of which being 3.5cm. The patient then had an EGD that demonstrated friable, mottled, irregular mass extending from 40cm in the esophagus to 45cm. The patient also had an elevated CEA at greater than 949, CA 19-9 at 221 and an AFP of 2400. The patient’s esophageal mass biopsy resulted in AC with neuroendocrine differentiation. The patient then received a liver biopsy that showed metastatic Adenocarcinoma not HCC. A repeat liver biopsy was done and the pathology was read again as AC.

DISCUSSION:
This is a rare case of esophageal cancer resulting in a marked increase of AFP without HCC or testicular involvement. Also the patient had esophageal cancer with neuroendocrine differentiation with liver masses. The patient is without active hepatitis, liver disease or testicular involvement. Very elevated AFP level and esophageal cancer with no relation has only been described in few cases and if there is elevation it is moderate. A lesson to be learned from this case is the patient should have had EGD sooner as most adenocarcinomas arise from Barrett’s esophagus. Had the patient had and EGD the diagnosis of and treatment of Barrett’s could have been initiated sooner.
1807 Esophageal Melanocytosis: An Uncommon Finding
Authors: Patel Milan , Shyu Irene , Mochel Mark , Smallfield George B. 
Published: October 2019

INTRODUCTION:
Esophageal melanocytosis is a benign, rare melanocytic proliferation in the basal layer of the squamous epithelium. The etiology of the melanocytosis is not clear; however, it has been suggested that aberrant migration during embryogenesis and chronic irritation such as GERD could lead to its occurrence. While largely asymptomatic, these proliferations raise concern for mucosal melanoma.

CASE DESCRIPTION/METHODS:
A 69-year-old Trinidadian female with past medical history of gastroesophageal reflux disease (GERD) presented with a two week history of dysphagia. Physical examination did not reveal any cutaneous or mucocutaneous lesions. Neurological exam also was normal. A computerized tomography (CT) scan showed circumferential wall thickening and moderate patulous proximal esophagus. A barium esophagram revealed severe esophageal dysmotility and GERD. Given dysphagia and CT findings, an esophagogastroduodenoscopy (EGD) was performed revealing hyperpigmented mucosa in the lower third of the esophagus (Figure 1A). Biopsies revealed melanin within the basal squamous epithelium with evenly spaced melanocytes and pigmented dendritic processes (Figure 1B). Immunohistochemistry for Melan-A and SOX10, a nuclear marker of melanocytes, highlighted these melanocytes without nesting or pagetoid scatter (Figure 1C). The patient was scheduled for a follow-up EGD for monitoring in 12 months.

DISCUSSION:
Endocscopically, anal and colonic lesions have been discovered along with esophageal lesions. Esophageal lesions largely present in the middle and lower third of the esophagus. Initial visualization of these lesions should be managed with a biopsy to determine if melanoma is present. Should esophagael melanocytosis be discovered on biopsy, repeat EGD with biopsy is recommended as early melanoma-in-situ may closely mimic esophageal melanocytosis.
1808 Dysphagia: A Rare Case of Neuroendocrine Carcinoma of the Esophagus
Authors: Zaver Himesh B. , Devani Kalpit H. , McKinney Jason 
Published: October 2019

INTRODUCTION:
Neuroendocrine carcinoma (NEC) of the esophagus is a rare, poorly understood disease with high mortality rates. We present a de novo case of NEC of the esophagus responsive to multi-modality therapy facilitated by early endoscopic evaluation and clinical suspicion. Our objective is to raise awareness on rarer causes of dysphagia in order to improve time to treatment and patient outcomes.

CASE DESCRIPTION/METHODS:
A 63-year-old male with a past medical history significant for GERD and tobacco use, presented with a complaint of a one month history of progressive dysphagia to solids. He denied any history of presenting symptoms and had no prior endoscopic examination in the past. Vitals, laboratory workup, and physical exam including chest and abdominal exam were normal. In the setting of progressive dysphagia the patient underwent Esophagogastroduodenoscopy (EGD) revealing a distal esophageal mass (Figure 1). Subsequent Computed Tomography (CT) and Positron Emission Tomography (PET) scan, showed an esophageal mass approximately 4.7 cm x 8.5 cm without any distant metastasis (Figure 2). Biopsy revealed poorly differentiated, discohesive cells indicative of malignant, high-grade neoplasm with neuroendocrine features. Immunohistochemical testing was positive for CD56, synaptophysin, AE1/AE3, and CK8/18. Our patient was referred to a multidisciplinary team and underwent local radiation and chemotherapy of Cis-platinum and Etoposide with excellent response. Surveillance EGD with random biopsies and PET-CT following treatment at 1 year from diagnosis showed no evidence of residual malignancy (Figure 3). However, surveillance EGD at year 2, showed irregular Z lines and biopsy revealed small nests of atypical cells with neuroendocrine features. PET-CT and EUS were negative for any metabolically active neoplasm, and the patient declined further treatment.

DISCUSSION:
Esophageal NEC is rare with only 0.4 and 2% of all diagnosed NETs being found in the esophagus. Our case presents a patient with limited disease surviving past the median survival time of 17-23 months. Our patient was responsive to local radiation and Cis-platinum and Etoposide management. However, surveillance EGD two years after initial diagnosis showed recurrent malignancy. Poor patient outcomes and the limited number of reported cases have made it difficult to define first-line therapeutic strategies. Albeit rare, increased clinical suspicion and early endoscopic evaluation can hasten time to treatment and improve patient outcomes.
1809 Gurvits Syndrome: A Rare Cause of Upper Gastrointestinal Bleed
Authors: Ali Sara I. , Tariq Saad , Patel Jigar , Cherian Sujith , Larson Scott 
Published: October 2019

INTRODUCTION:
Acute Esophageal Necrosis (AEN), also known as Black Esophagus or Gurvit’s syndrome, is a rare disorder that can present as an acute gastrointestinal (GI) bleed. Given the high risk of perforation and mortality, a GI bleed in the correct clinical context should put AEN on the differential. We discuss clinical, endoscopic and management of AEN.

CASE DESCRIPTION/METHODS:
57-year-old female with hypertension and gastroesophageal reflux disease presented after a pre-syncopal event. She was admitted for septic shock secondary to urinary tract infection. She was hypothermic with a blood pressure of 76/48 mm Hg on arrival to the ICU. She was started on broad spectrum antimicrobials, in addition to, norepinephrine and vasopressin to maintain mean atrial pressures above 60 mmHg. On ICU day two, she had multiple episodes of coffee-ground emesis and was intubated for airway protection. Her hemoglobin acutely dropped from 8 g/dl to 6.8 g/dl over 12 hours. EGD revealed circumferential ulceration of the lower esophagus with areas of necrosis. Patient was started on high-dose intravenous proton pump inhibitor and supportive therapy with treatment of septic shock. Three days later an endoscopic nasojejunal tube was placed for enteral feeding which revealed increasing amounts of viable mucosa with a decrease in the area of necrosis. There were no further GI bleed and patient was discharged 30 days later.

DISCUSSION:
AEN is multifactorial with a combination of an ischemic insult, corrosive injury to the mucosa from gastric contents and impaired reparative mechanism in setting of acute illness. Risk factors include male sex, older age and multiple chronic medical conditions. Men are four times more commonly affected than women. Diagnosis is made via direct visualization with findings of circumferential black discoloration of the distal esophagus that abruptly ends at the gastroesophageal junction. With comparatively less dense vasculature, the distal esophagus is the water-shed area of the esophagus. Treatment of AEN focuses on aggressive treatment of underlying disease, supportive therapy, and acid suppression. Complications of AEN include esophageal perforation, mediastinitis, and abscess formation. Long term complications include stricture formation and stenosis. Insertion of nasogastric tube should be avoided given the high risk of perforation secondary to the friability of the esophageal mucosa. In our patient, endoscopically placed nasojejunal tube was a safer alternative to restoring enteral feeding.
1813 A Killer in the Dark: The Black Esophagus
Authors: Zahir Fathima Z. , Buffie Charlie , Wan David , Betesh Andrea 
Published: October 2019

INTRODUCTION:
Acute esophageal necrosis, also known as “black esophagus” is a rare condition of unclear etiology. Only 88 patients were diagnosed over a period of 40 years. It is diagnosed by endoscopy showing darkly pigmented necrotic esophageal mucosa. Prognosis is poor, with a mortality rate of 31.8-50%. We report a case of this condition in an elderly woman who presented with hematemesis and sepsis.

CASE DESCRIPTION/METHODS:
A woman aged 72 presented after a fall with head strike without loss of consciousness. CT head was unremarkable. That evening, she had an episode of coffee-ground emesis associated with hypotension, tachycardia, and worsening mental status. Hemoglobin was 6.1, raising suspicion of GI bleed. However, she developed leukocytosis without abdominal pain or melena, suggesting septic shock. She received fluids and antibiotics with no improvement. CTPA showed bilateral pulmonary embolism. Anticoagulation was held given concern for GI bleed. Later a massive transfusion protocol was activated when hemoglobin dropped to 4.8; 400 cc of blood was aspirated from the stomach. She received 3U pRBCs, 1U FFP, 1U platelets, 500 cc albumin and vasopressors with improvement of hemoglobin to 11. She then became increasingly acidotic and hypothermic for which a bair hugger was placed. An EGD revealed a severely inflamed esophagus with black material extending from the mid esophagus to the gastroesophageal junction, consistent with esophageal necrosis, the likely source of her GI bleed. The patient stopped following commands, moving extremities or responding to pain. Despite maximal measures, the patient’s hemodynamic instability worsened and she died.

DISCUSSION:
Acute necrotizing esophagitis is a fatal condition that commonly presents with hematemesis or melena. It is associated with multiple factors including diabetes, hemodynamic instability, trauma, hypercoagulable state, gastric volvulus, and some infections. While the pathophysiology is not well known, it is hypothesized that the condition results from an ischemic insult. Esophageal perforation, stricture, infection and death are the main complications. As with our patient, the most likely cause of death are underlying comorbidities such as infections, sepsis, hypovolemic shock or pulmonary embolism, while underlying esophageal disease as the cause of death have been reported in only less than 10% of patients. Elderly patients with comorbidities like diabetes and vascular disease, presenting with upper GI bleed must always get an upper GI endoscopy.
1817 Blastic Mantle Cell Lymphoma of the Gastroesophageal Junction: A Unique Presentation
Authors: Ahmed Ahmed , Vossough Sima 
Published: October 2019

INTRODUCTION:
Though vast, the medical literature is deficient on reports of gastroesophageal junction (GEJ) involvement of mantle cell lymphoma (MCL). We present a unique case of a 76-year-old male who was found to have a blastic variant of MCL in the GEJ. This case provides additional distinct features to the various clinical presentations associated with this rare proliferative disorder, thereby enhancing the medical literature on MCL.

CASE DESCRIPTION/METHODS:
A 76-year-old male with a history of prostate and bladder cancer presented with worsening dysphagia, weight loss, and heartburn for over 6 months. He had extensive workup in the past for an ulcerative, gastric/GEJ lesion, found on 4 separate esophagogastroduodenoscopies (EGD) and 2 esophageal ultrasounds (EUS), whose biopsies ranged from mild gastritis to intestinal metaplasia, but were repeatedly negative for malignancy. Physical exam and labs were unremarkable except for anemia. Computerized tomography (CT) scan showed an irregular mass extending from the proximal fundus to the GEJ, and diffuse lymphadenopathy (LAD). EGD on admission showed a large friable, ulcerated lesion with heaped up margins involving the cardia, with extension into the GEJ. GEJ biopsies showed histological and immunohistochemical (IHC) findings consistent with blastic MCL. Further workup of a positron emission tomography (PET) scan showed a fluorodeoxyglucose (FDG) avid mass involving the GEJ and stomach as well as FDG avid regional LAD. A bone marrow biopsy showed minimal involvement (<5%) of CD5+/CD23+ B cells and was negative for both Cyclin D1 and t (11;14). He was started on a chemoimmunotherapy regimen (CITR) with a good response and is currently progression free after 2 years.

DISCUSSION:
The EGD biopsy was diffusely infiltrated by atypical lymphocytes with prominent nucleoli and IHC stains positive for CD-20, Cyclin D1, BCL-2, and BCL-6, and a Ki-67 proliferative index >90%; all consistent with blastic MCL; a rare and aggressive subtype of MCL. He was not a candidate for stem cell transplant due to his age/comorbidities, so he was started on guideline-based CITR of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) followed by rituximab maintenance therapy. PET/EGD done 1 year later showed improvement of the LAD and GEJ mass, with negative biopsies for lymphoma. To the best of our knowledge, there have been no reported cases of GEJ involvement of blastic MCL appearing as a large friable lesion, illustrating the unique aspect of this case.
1822 Esophageal Neuroendocrine Tumor: A Rare Case of High-Grade Carcinoma of the Esophagus
Authors: Parsa Nour , Mansoor Muhammad Sohail , Tageldin Omar , Zelman Sara , Batool Asra 
Published: October 2019

INTRODUCTION:
Neuroendocrine tumors (NET) of the esophagus are extremely uncommon. The rarity of esophageal NETs is thought to be due to an underdeveloped neuroendocrine system in the esophagus. When diagnosed, esophageal NETs are usually found in the distal esophagus where neuroendocrine cells are primarily distributed in associated mucosal glands.

CASE DESCRIPTION/METHODS:
We describe a unique case of a 62-year-old man diagnosed with an esophageal NET. His PMHx is notable for psoriasis, chronic tobacco use (45 pack years), cirrhosis from heavy alcohol use and illicit drug use including cocaine. He first presented to the hospital with complaints of intermittent episodes of chest pain. He underwent full cardiac workup which was unremarkable. Several months later, he returned to clinic with symptoms of progressive dysphagia to solids. Esophagram revealed a 7cm narrowed segment along the distal esophagus with mucosal irregularity concerning for esophageal neoplasm. A follow up CT abdomen/pelvis showed circumferential wall thickening of the distal esophagus, a 5.2cm necrotic lymph node within the gastrohepatic ligament, as well as a smaller necrotic lymph node at the level of the gastroesophageal junction concerning for malignancy. Follow up EGD revealed a fungating mass in the distal esophagus with biopsies confirming tumor cells positive for MNF116, CD56 and synaptophysin; the overall findings were consistent with high grade neuroendocrine carcinoma of the esophagus. The patient started chemotherapy with Carboplatin and Etoposide; he was subsequently treated with single agent Paclitaxel. The metastatic burden continued to worsen with increased involvement of the liver, lymph nodes and most recently the brain. At his most recent clinic visit, he continues to lose weight and still experiences dysphagia to solids though he is tolerating liquids. He will be starting radiation treatments to the brain and also palliative radiation to the GE junction to help alleviate his dysphagia.

DISCUSSION:
Prompt diagnosis of esophageal NET is critical as these tumors are known to be rapidly progressive and often have already metastasized by the time of diagnosis. Favorable prognostic factors include no lymphatic metastasis, tumor size of <1.0 cm and histology that reveals well-differentiated tumor cells. For unclear reasons, men are disproportionately more likely to develop NET of the esophagus. The most frequent clinical complaint is dysphagia followed by unintentional weight loss, melena and then typical carcinoid symptoms.