Esophageal Granular Cell Tumor Research Articles

Gastrointestinal Tract Granular Cell Tumor in the Pediatric Population: A Multicenter Experience.

Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date. Multicenter retrospective study of pediatric GIT GCT. A total of 10 cases were included in the study with a median age of 13.5 years (range: 7-18 years) and were predominantly female patients (60%). In half of the patients no significant medical history was present with the remaining 5 having Crohn disease (10%), eosinophilic esophagitis (EoE) (10%), Crohn disease and EoE (10%), growth hormone deficiency (10%), and aplasia cutis congenita (10%). The GCT median size was 1.3 cm (range: 1-1.6 cm) and were more commonly located in the esophagus (70%) followed by the stomach (20%) and rectum (10%). Most of the cases showed round/polygonal tumor cells with abundant granular cytoplasm, and none of the cases had nuclear atypia, increased mitotic activity, or tumor cell necrosis. None of our cases received specific therapy for GCT other than clinical follow-up, and none of the patients had evidence of local recurrence or metastatic disease. We present our multicenter experience with GIT GCT, all cases had a benign course. Interestingly, 4 of the esophageal GCT cases (including 2 patients with EoE) showed an eosinophil-rich esophagitis in the underlying mucosa.

A Case of a Granular Cell Tumor Arising in a Patient with Long-segment Barrett's Esophagus.

Esophageal cell tumors are rare. Esophagogastroduodenoscopy performed on a 48-year-old woman revealed an elevated esophageal lesion and the presence of long-segment Barrett's esophagus. Endoscopic ultrasonography showed a 15 mm homogeneous hypoechoic tumor extending from the lamina propria mucosa to the submucosa. Pathological examination of the biopsy tissue revealed a sheet-like cluster of histiocytoid cells with an abundant eosinophilic granular cytoplasm. Immunohistochemical examination revealed S-100 (+) and CD68 (+), thus suggesting the diagnosis of a granular cell tumor. The tumor was resected by endoscopic submucosal dissection. Pathologically, the background mucosa was Barrett's mucosa. This is the first reported case of an esophageal granular cell tumor in long-segment Barrett's esophagus.

Clinical outcomes of esophageal granular cell tumors with different endoscopic resection methods

Esophageal granular cell tumors (GCTs), the second most common subepithelial tumors (SETs) of the esophagus, are potentially malignant with no definite management guidelines available. We retrospectively enrolled 35 patients with endoscopically resected esophageal GCTs between December 2008 and October 2021 and evaluated the clinical outcomes from the various methods performed. Several modified endoscopic mucosal resections (EMRs) were performed for treating esophageal GCTs. Clinical and endoscopic outcomes were evaluated. Mean age of patients was 55.8 ± 8.2, with majority being men (57.1%). Mean tumor size was 7.2 ± 2.6 mm, most (80.0%) were asymptomatic and present in the distal third of the esophagus (77.1%). Endoscopic characteristics predominantly included broad-based (85.7%) and whitish-to-yellowish color changes (97.1%). Endoscopic ultrasound (EUS) of 82.9% of the tumors revealed homogeneous hypoechoic SETs originating from the submucosa. The five endoscopic treatment methods used were: ligation-assisted (77.1%), conventional (8.7%), cap-assisted (5.7%), and underwater (5.7%) EMRs and ESD (2.9%). Mean procedure time was 6.6 ± 2.1 min, and no procedure-associated complications were noted. The en-bloc and complete histologic resection rates were 100% and 94.3%, respectively. No recurrences were noted during follow-up, and no significant differences in the clinical outcomes of the different methods of endoscopic resection were found. Based on tumor characteristics and therapeutic outcomes, modified EMR methods can be effective and safe. However, there were no significant differences in the clinical outcomes of the different methods of endoscopic resection.

Combination conventional endoscopy and endoscopic ultrasound can differentiate between esophageal granular cell tumors and leiomyomas.

Esophageal leiomyomas and granular cell tumors (GCTs) are the 2 most common subepithelial tumors found in the esophagus. We attempted to differentiate the 2 tumors using endoscopic findings and endoscopic ultrasound (EUS) features. Between December 2008 and June 2021, a total of 38 esophageal GCTs and 11 esophageal leiomyomas originating from the muscularis mucosa were selected. Clinical characteristics and endoscopic features were retrospectively reviewed. Although esophageal GCTs are mainly located in the lower third of the esophagus (81.6%), esophageal leiomyomas are mainly located in the upper third of the esophagus (45.5%). Broad-based (84.2%, P = .002) and whitish-to-yellowish color changes (97.4%, P < .001) are significant endoscopic features of esophageal GCTs. The echogenicity of esophageal leiomyoma was similar to that of proper muscle echogenicity. However, the echogenicity of esophageal GCTs was hyperechoic compared to that of the proper muscle layer (90.0% vs 9.1%, respectively, P < .001). EUS revealed a clearer hyperechoic epithelial lining in the esophageal leiomyoma than in esophageal GCTs (100% vs 26.7%, respectively, P < .001). The 5 endoscopic factors (location of the lower third, broad base, whitish-to-yellowish color, hyper-echogenic, and unclear demarcated hyperechoic epithelial line) were counted to differentiate esophageal GCTs from esophageal leiomyomas. Tumors with 3 or more endoscopic factors were all esophageal GCTs. The characteristic endoscopic and EUS features of esophageal GCTs were broad-based, whitish-to-yellowish colored subepithelial tumors located in the lower third of the esophagus and hyperechoic tumor with an unclear demarcated hyperechoic epithelial line. A combination of these features can predict esophageal GCTs before endoscopic resection.

Underwater endoscopic mucosal resection of upper gastrointestinal subepithelial tumors: A case series pilot study (with video).

Introduction:Underwater endoscopic mucosal resection (UW-EMR) has been recently introduced as an effective technique for rectal third layer subepithelial tumors. Therefore, we aimed to assess the safety, efficacy, and procedure time of UW-EMR for upper gastrointestinal subepithelial tumors (SETs) originating from the deep mucosal and/or submucosal layers.Methods:Between August 2018 to July 2022, a total of 17 SETs (7 duodenal SETs, 6 gastric SETs, and 4 esophageal SETs) were included in this study. On endoscopic ultrasound examinations, the tumors were found to be embedded in the submucosa without muscularis propria invasion. All SETs were resected successfully using UW-EMR. The characteristics of the tumors and their R0 resection rate, adverse event rate, and recurrence rate were evaluated retrospectively.Results:The mean tumor size was 0.9 cm (range, 0.3-1.5 cm). En bloc resection and complete resection rates were 100%, respectively. The patients showed no complications such as perforation or bleeding. Histologic assessments of the resected tumors revealed 9 neuroendocrine tumors (7 on the duodenum, 2 on the stomach), 2 gastric cystica profunda, 1 gastric follicular lymphoma, 1 gastric fibromyxoma, 3 esophageal granular cell tumors, and 1 esophageal adenoid cystic carcinoma. The mean procedural time was 3.2 min (range, 1.3-8.7 minutes). The overall en bloc and complete resection rates were 100%, respectively. No recurrence was observed during the follow-up period.Conclusion:UW-EMR is a safe and effective treatment for upper gastrointestinal SETs embedded in the submucosal layer. Further studies are needed to compare other endoscopic resection techniques.

Role of endoscopic ultrasound and endoscopic resection in the diagnosis and treatment of esophageal granular cell tumors

Background and Aim Diagnosis and complete resection of esophageal granular cell tumors (GCTs) is an area of concern. However, articles on endoscopic ultrasound (EUS) and endoscopic resection of esophageal granular cell tumors are few. To evaluate the role of endoscopic ultrasound and endoscopic resection in the diagnosis and treatment of esophageal granular cell tumors. Methods A retrospective analysis of 15 patients with esophageal granular cell tumors who underwent endoscopic ultrasound examination and endoscopic resection in our hospital was conducted. The clinical data, endoscopic ultrasound images, endoscopic treatment, pathological characteristics, postoperative complications and follow-up status of all patients were evaluated. Ten board-certified endoscopists independently evaluated the white light endoscopic images of the 15 patients (Test 1) and the endoscopic ultrasound images together with white light endoscopic images of the same patient set (Test 2). Result Female patients accounted for 53.4% of the participants. The average age at the time of diagnosis was 49.13 ± 9.31 years old. Ten lesions (66.67%) showed hypoechoic signal, four lesions (26.67%) showed hyperechoic signal and one lesion showed medium signal. The diagnostic accuracy was significantly higher with Test 2(65.3% vs. 92.0%, p < .001). Complete endoscopic resection was performed in all the patients. No complications occurred in any of the patients. No esophageal stenosis, recurrence, or metastases was found in all patients during the follow-up period. Conclusion The endoscopic ultrasound images of esophageal granular cell tumors have certain characteristics that help diagnose esophageal granular cell tumors. Endoscopic resection of esophageal granular cell tumors is an effective, safe and feasible treatment method.

S2400 Granular Cell Tumor: A Rare Disease of Esophagus

Introduction: Granular cell tumors (GCTs) are rare, often benign tumors of neurogenic origin. GCTs are usually found incidentally but can present with dysphagia in symptomatic patients. They are diagnosed via biopsy and histology following an esophagogastroduodenoscopy( EGD). We present a case of a 46-year-old female who presented with dysphagia, GERD, and weight loss diagnosed with esophageal granular cell tumor following an EGD. Case Description/Methods: A 46 years old female with a past medical history of H. pylori gastritis presents with complaints of dysphagia for 3 months and GERD associated with weight loss. EGD reviewed a single 4 mm nodule found in the lower third of the esophagus with the appearance of a lipoma (Figure). Biopsy of esophagus nodule resulted in esophageal squamous epithelium with focal granular cell tumor; Immunohistochemistry tumor cells are positive for S100, CD56, CD68, negative for calretinin and AE1/3 supporting rare esophageal granular cell tumor. EUS demonstrated a 4.7 mm x 2.6 mm intramural (subepithelial) lesion appeared to originate from within the superficial mucosa (Layer 1), Patient underwent repeat EGD with en-bloc cap and band-assisted endoscopic mucosal resection (EMR) followed by 3 hemoclips placed for hemostasis. The patient was discharged home on a proton pump inhibitor. The patient reports clinical improvement on follow-up after 4 weeks. Discussion: GCTs are thought to originate from Schwann cells; the prevalence of GCTs is challenging to determine as there have been roughly 300 documented cases of GCTs discussed in the literature. Conversely, patients with larger diameter lesions typically present with indolent dysphagia and other less common symptoms such as gastro-esophageal reflux disease, dyspepsia, chest pain, cough, or nausea. GCTs on EGD appear sessile, yellow-white in color, and firm, with a negative pillow sign. After performing EGD, an endoscopic ultrasound (EUS) is diagnostically utilized to ascertain tumor invasion and provide a biopsy through fine needle aspiration (FNA). Histopathological diagnosis, including immunohistological staining, is essential for confirmation of GCT. Immunohistological staining is positive for S-100, PAS (Periodic Acid Schiff), neuron-specific enolase, nestin, and vimentin. Patients whose neoplasms are less than 1 cm in diameter are treated conservatively with endoscopic follow-up, with tumors greater than 1 cm in diameter, or having clinical symptoms that need endoscopic or surgical resection.Figure 1.: A: EUS showing 4.7 mm x 2.6 mm hypoechoic intramural (subepithelial) lesion was found in the lower third of the esophagus. It appeared to originate from within the superficial mucosa (Layer 1). A tissue diagnosis was obtained prior to this exam. B: EGD significant for sessile, yellow white in color, and firm mass followed by EMR. C: Histopathology with H&E showing clusters of polygonal cells with granular eosinophilic cytoplasm (top) and S100 stain (below).

A case of esophageal granular cell tumor diagnosed by mucosal incision-assisted biopsy

For an esophageal submucosal mass suspicious of granular cell tumor (GCT) based on gross appearance and endoscopic ultrasound findings, a sufficient number of biopsy specimens is required for a definite diagnosis using immunohistochemical examination. When the specimen obtained by forceps biopsy is insufficient, endoscopic ultrasound-fine needle aspiration (EUS-FNA) is believed to be an useful alternative. However, it may be difficult to obtain an adequate amount of tumor material using EUS-FNA. Mucosal incision-assisted biopsy (MIAB) is a simple method that can collect larger amounts of specimens. This procedure is helpful for physicians who encounter the problem of obtaining an adequate amount of biopsy material from esophageal tumors suspicious for GCT. We present a case of esophageal GCT that was successfully diagnosed through MIAB.

S1867 Rectal and Esophageal Granular Cell Tumors; A Report of Two Cases

Introduction: Granular cell tumors (GCT) are a predominantly benign tumor arising from mesenchymal origin - specifically Schwann cells. They typically appear as a solitary mass; in the gastrointestinal tract (GIT), they can present as a painless yellow-gray polyp or nodule. It is more commonly found in women ages 10-50 years old. They can arise in any location, most commonly from the skin, and only 10% found in the GIT. Within the GIT, the esophagus is the most common location while the rectum is the rarest location. Case description/methods: Case #1: Patient is a 51-year-old female with no significant past medical history coming in with right upper quadrant (RUQ) abdominal pain. Pain has been present for 1 month prior to seeking medical attention. Pain is not consistently associated with eating food. There is no associated nausea, vomiting, diarrhea, or constipation. EGD was performed at that time and revealed a white localized plaque (image 1A). Histopathology on H&E stain revealed esophageal mucosa with underlying sheets of epithelioid cells with abundant granular eosinophilic cytoplasm (image 1B). S100 stain was positive, but MART1 and SOX10 were negative. Case #2: Patient is a 64-year-old male with a past medical history significant for aortic stenosis who presented for his first screening colonoscopy. At that time, colonoscopy revealed mild erythematous mucosa in the terminal ileum and sigmoid colon as well as a small mass in the rectum (image 1C). Biopsy results revealed focal active colitis in the sigmoid colon and a rectal granular cell tumor in the rectum. Histopathology of the tumor revealed colorectal mucosa with packets of uniform epithelioid cells with abundant eosinophilic granular cytoplasm interdigitating with the colonic crypts on H&E stains (Image 1D). S100 immunohistochemistry stain demonstrated diffuse expression within the granular cell tumor with absent staining in crypt epithelium (Image 1E-F). MART1 and SOX10 were negative as well. Discussion: GCTs are rare tumors ad account for 0.5% of all soft tissue tumors. Only 10% occur in the GIT and rarely in the rectum. Most are benign with only 1-2% being malignant. If ulceration is found, malignancy should be ruled out. It is also important to differentiate them from other tumor occurring in the GIT such as neurogenic and gastrointestinal stromal tumors. It is also important to differentiate them from melanomas as they stain positive for S100; melanoma specific stains, such as MART1 and SOX10, are used to differentiate them.Figure 1.: (A) White localized plaque in the esophagus. (B) Esophageal mucosa with underlying sheets of epithelioid cells with abundant granular eosinophilic cytoplasm. (C) Small yellowish mass in the rectum. (D) H&E stain revealing colorectal mucosa with packets of uniform epithelioid cells with abundant eosinophilic granular cytoplasm interdigitating with the colonic crypts. (E) S100 immunohistochemistry stain demonstrating diffuse expression within the granular cell tumor with absent staining in crypt epithelium. (F) Rectal granular cell tumor negative for MART1 and SOX10 at 20x magnification.

S2114 IgG4-Related Disease: An Uncommon Location

Introduction: Granular cell tumors (GCTs) of the esophagus are rare and benign neoplasms of neural cell origin. The lesions are often incidentally identified during upper endoscopic evaluation for other indications. We report a case of esophageal GCT in a young patient who presented with heartburn. Case Description/Methods: A 29-year-old white woman with a history of bulimia nervosa, gastroesophageal reflux disease, and chronic diarrhea following a recent COVID-19 infection presented to the clinic with heartburn and regurgitation that had been ongoing for years. She recently switched her omeprazole to famotidine to prepare for conception, which made her symptoms worse. Her physical examination and laboratory investigations were unremarkable. Esophagogastroduodenoscopy showed a single 6 mm yellowish, firm, submucosal nodule in the distal esophagus (Fig. 1A) and chronic gastritis. The nodule was biopsied with cold forceps and proved to be a granular cell tumor (Fig. 1B). The granular cell tumor cells were negative for pan-cytokeratin and positive for S100 (Fig 1C). They were negative for CD45 and Synaptophysin by immunohistochemistry staining. Biopsy of the distal esophagus also confirmed a short segment of Barrett's esophagus without dysplasia. Gastric body and antrum biopsy showed mild chronic gastritis negative for Helicobacter pylori. She was scheduled for an endoscopic ultrasound followed by possible endoscopic mucosal resection. Discussion: Even though esophageal GCTs are benign incidental findings, they have malignant potential (1-4%). Mortality is related to the size, growth, and invasiveness of the tumor. Indications for endoscopic or surgical resection are large size ≥ 1 cm, rapid growth, high suspicion of malignancy, transmural infiltration, and persistent symptoms. Endoscopic resection techniques include endoscopic mucosal resection and submucosal tunnel endoscopic resection. The later is more preferred in lesions < 3 cm. One study reported no recurrence of esophageal GCTs after resection in 14 cases at a median of 4-year follow-up.

Esophageal granular cell tumor and eosinophils: a multicenter experience

BackgroundEsophageal granular cell tumor (eGCT) is rare, and the recent literature suggests a link between eosinophilic esophagitis (EoE) and eGCT. The aim of our study was to determine if EoE or other disorders associated with eosinophilia are consistently associated with eGCT.MethodsWe retrospectively searched pathology databases of three academic institutions from 1999 to 2018 for eGCTs. The archived slides and medical records were reviewed.ResultsFrom 294,855 esophagogastroduodenoscopy procedures, 45 patients (17 males and 28 females) with eGCTs were identified. The patients (30–73 years in age, median 50) had eGCT 0.2–2.0 cm in size (average 0.71). Thirteen had a history of gastroesophageal reflux disease, 5 had Barrett esophagus/goblet cell metaplasia and 1 had EoE. Thirty-four eGCTs had intralesional eosinophils (14 with peak > 10 eosinophils/400x hpf); of these, 21 also had eosinophils in lamina propria (9 with peak > 10 eosinophils/hpf). eGCT with atypical features (including nuclear enlargement and prominent nucleoli) were more likely to have increased eosinophils in non-epithelial compartments than those without atypia. Pleomorphism and spindled cells were seen in 3 eGCT cases (mean peak intralesional eosinophils: 43 per hpf); 2 of these had goblet cell metaplasia. We found no association between EoE and eGCT, p = 0.5966, (95% C.I. 0.0276, 6.5389, Fisher’s exact test). Instead, most patients had gastroesophageal reflux disease or Barrett esophagus.ConclusionEosinophilia, common in eGCT and adjacent stroma, likely drives atypical/reactive histologic features, but a pathogenic relationship between eosinophil rich inflammatory conditions and eGCT has not yet been established.

Cervical esophaguectomy plus tracheal resection and free jeyuno graft as treatment of esophageal granular cell tumor

Granular cell tumor is a very rare entity (0.03%) with controversial management, due to its low incidence and its usually benign behavior (98%). Its location in the digestive tract is unusual (3–11%), being the esophagus the most frequently affected organ, with about 400 published cases. However, the incidence in the cervical esophagus is anecdotal (20 published cases).We present a patient with an esophageal granular cell tumor that invaded trachea, thyroid and soft tissues, performing a block resection of the cervical esophagus, two tracheal rings, thyroid and prethyroid musculature. For digestive reconstruction, a free jejunum graft with microvascular anastomosis was made and tracheal reconstruction was performed using term-terminal anastomosis.The importance of the case is twofold: to contribute more casuistry, and to describe a highly complex surgical technique never indicated in this type of tumors, due to its rarely aggressive character.

Esofaguectomía cervical más resección traqueal e injerto de yeyuno libre como tratamiento de un tumor de células granulares esofágico

Granular cell tumor is a very rare entity (0.03%) with controversial management, due to its low incidence and its usually benign behavior (98%). Its location in the digestive tract is unusual (3-11%), being the esophagus the most frequently affected organ, with about 400 published cases. However, the incidence in the cervical esophagus is anecdotal (20 published cases).We present a patient with an esophageal granular cell tumor that invaded trachea, thyroid and soft tissues, performing a block resection of the cervical esophagus, two tracheal rings, thyroid and prethyroid musculature. For digestive reconstruction, a free jejunum graft with microvascular anastomosis was made and tracheal reconstruction was performed using term-terminal anastomosis.The importance of the case is twofold: to contribute more casuistry, and to describe a highly complex surgical technique never indicated in this type of tumors, due to its rarely aggressive character.

S2028 A Rare Case of Esophageal Granular Cell Tumor

INTRODUCTION: Granular cell tumors (GCTs) are rare soft tissue neoplasms thought to originate from Schwann cells. Usually they are found in skin and subcutaneous tissue of the head and neck, particularly inside the mouth (70%). Gastrointestinal (GI) tract GCTs are very uncommon, occurring in only 6-10% of all cases. Esophageal GCTs account for only 30-60% of this subset. Given their rarity, esophageal GCTs are not well reported in the literature. We describe a patient who underwent an esophagogastroduodenoscopy (EGD) for abdominal pain and nausea and was found to have a 1 cm GCT in her proximal/mid esophagus. CASE DESCRIPTION/METHODS: A 48 year old African American female with history of gastro-esophageal reflux disease (GERD) presented to GI clinic for months of abdominal pain associated with nausea despite taking 40mg daily Omeprazole and as needed Calcium Carbonate. She was afebrile and hemodynamically stable. Her abdomen was non-tender to palpation. An EGD was scheduled to rule out endoscopic causes of her symptoms. Her EGD revealed a 1 cm firm, mobile nodule in her upper/mid esophagus (Figure 1). The rest of the esophagus, stomach, and duodenum were otherwise normal. Biopsy of the nodule revealed esophageal granular cell tumor, confirmed by S100 immunohistochemical staining. The tumor was excised by endoscopic mucosal resection (EMR) (Figure 2). Repeat histology revealed possible incomplete resection of the tumor, as it extended to deep and lateral specimen margins. An endoscopic ultrasound (EUS) expert is currently reviewing the case to determine whether the patient should have an EUS versus a repeat EGD next. DISCUSSION: Esophageal GCTs were first described in 1926 by Abrikossoff. They are most commonly reported in African American women around 40-60 years of age. Endoscopically they appear as firm, smooth intramural lesions with normal overlying mucosa. EUS determines their malignancy potential by assessing tumor size, location, depth of invasion, and lymph node involvement. Esophageal GCTs often stain positive for periodic acid-Schiff, nestin, S100, and CD68 given their neurologic origin. Although the vast majority of esophageal GCTs are benign, with fewer than 40 (< 2%) malignant cases noted in the literature, malignant cases tend to be aggressive. Esophageal GCTs that cause symptoms, have malignant features, or are greater than 1-2cm in diameter should be removed by EMR or endoscopic submucosal dissection. High suspicion and detailed workup are needed so that providers do not miss this rare entity.Figure 1.: Endoscopic view of a single 1 cm localized, firm, mobile nodule with normal overlying mucosa in the upper/mid esophagus at 25 cm from incisors.Figure 2.: Removal of the nodule by single-piece polypectomy.

S1953 Esophageal Granular Cell Tumor: A Rare Entity

INTRODUCTION: Esophageal Granular Cell Tumor (EGCT) is a rare condition; up to date, only 300 cases reported since 1931, it majorly affects the population between the ages of 25 to 53 years old, female-African American predominance compared to male caucasian patients. Histologically The Tumor is made of Schwann cells with granular cytoplasm, large polygonal cells, and positive for Periodic Acid Shiff (PAS) stain; In its majority, granular cell tumors appear in the skin and various other tissues, including the respiratory tract, biliary tract, and breast. Up to 8% of the cases can occur in the Gastrointestinal tract, and the esophagus is the most common location. At the moment there is no consensus regarding appropriate screening, diagnosis, and management. CASE DESCRIPTION/METHODS: The patient is a 56 year old male with a past medical history of iron deficiency anemia secondary to heavy menses, coronary artery disease, hypertension, diabetes, hyperlipidemia who was referred to the gastroenterology clinic for dyspepsia. An initial Esophagealduodenoscopy (EGD) was performed which showed an esophageal nodule noted at 35 cm (Figure 1). Endoscopic ultrasound (EUS) was then performed revealing a sub-centimeter mass on the second layer of the submucosa of benign appearance, with no adenopathy. The management plan at the time was to follow up in 1 year. Initially differential diagnosis included lipoma versus leiomyoma. A repeat EGD was then done where a biopsy was taken showing granular cell tumor (diagnosis supported by positive staining with CD68 and S100 immunostain), benign squamous mucosa, negative for intestinal metaplasia, negative for increased intraepithelial eosinophils (Figure 2). DISCUSSION: Management of EGCT is conservative for those masses < 10mm in diameter, endoscopic removal is advised for those lesions with malignant features or causing symptoms secondary to obstruction of the digestive or airway tract. Although EGCT is mainly benign, malignant transformation occurs in 1-2 % of the cases; EGD and EUS are essential to evaluate for lymph node invasion with the purpose of directing management, endoscopic resection can be performed and endoscopic surveillance is advised, although there is no consensus, the rapid development of endoscopic techniques remain the cornerstone of its management.Figure 1.: Esophagogastroduodenoscopy showing an esophageal nodule at 35cm.Figure 2.: Biopsy specimen of esophageal nodule staining positively to CD68.

S1910 A Case Series of Esophageal Granular Cell Tumor in Eosinophilic Esophagitis

INTRODUCTION: Granular cell tumors (GCTs) are rare, soft tissue neoplasms arising from Schwann cells and can be found anywhere in the body. Gastrointestinal GCTs represent 6–10% of all cases with the majority arising in the esophagus. On endoscopy, GCTs appear as white-grey or a yellow subepithelial lesion. Most GCTs are benign, but approximately 0.5–2% of cases progress to malignancy and carry a poor prognosis. Eosinophilic esophagitis (EoE) is a chronic, immune-mediated condition characterized by eosinophil-predominant inflammation of the esophagus. We present three cases of esophageal GCTs diagnosed endoscopically in the setting of EoE. CASE DESCRIPTION/METHODS: Case 1: A 25 year-old female with asthma and environmental allergies presented with chronic dysphagia and intermittent solid food impaction never requiring endoscopy. She underwent EGD which showed mild inflammation. There was also a 5 mm small nodular area with adherent exudate (Figure 1). Biopsies of the proximal and distal esophagus revealed squamous mucosa containing innumerable intraepithelial eosinophils, consistent with EoE. Additionally, the nodule was found to be a GCT. Case 2 :A 38 year-old male with a history of biopsy-proven EoE presented for a follow up EGD after receiving PPI therapy for twelve weeks. EGD revealed a normal esophagus (Figure 2); however, random biopsies revealed incidental GCT in the mid-esophagus. Case 3: A 33 year-old male with a history of childhood environmental allergies presented with chronic dysphagia and intermittent solid food impaction. On EGD, the esophagus appeared normal. However, random biopsies from the mid esophagus showed eosinophilia consistent with EoE as well as a GCT. Therefore, repeat EGD was performed with white light along with endoscopic ultrasound to further evaluate for GCT and revealed a 2 mm submucosal lesion (Figure 3). DISCUSSION: The relationship between GCT and EoE remains unclear. Previously, case reports noted the simultaneous finding of GCT in patients with EoE on EGD. Here, we present three different endoscopic findings of esophageal GCT. Most notably, in two cases GCT was not diagnosed visually, but rather histologically. These cases add to the growing body of literature associating EoE and GCT, though a pathophysiologic relationship has not yet been drawn. Whether the presence of GCT in these cases is completely incidental or immune-mediated is unknown. Thus, as the prevalence of EoE continues to increase, clinicians should be aware of the association between GCT and EoE.Figure 1.: EGD of the esophagus revealing a 5 mm granulosa cell tumor (GCT).Figure 2.: Normal endoscopic appearance of the esophagus without endoscopic appearance of a GCT.Figure 3.: Submucosal GCT seen on white light endoscopy.

Mo1287 LIGATION-ASSISTED ENDOSCOPIC MUCOSAL RESECTION FOR ESOPHAGEAL GRANULAR CELL TUMORS IS SAFE AND EFFECTIVE

Esophageal granular cell tumors (GCTs) are usually asymptomatic and incidentally found, but have malignant potential. Given this, resection is recommended, but optimal technique remains unknown. We present the largest US series of dedicated endoscopic resection using band ligation (EMR-B) of esophageal GCTs. We performed a retrospective analysis, identifying patients diagnosed with esophageal GCTs between 2002-2019 using a prospectively-collected pathology database at our tertiary care referral center. We included those patients who underwent EMR-B of esophageal GCT using the Duette Multi-Band Mucosectomy system. Medical records were queried for patient demographics, endoscopic and pathologic findings, resection technique, adverse events, and follow up. Among 21 patients, median age was 39 years, 16 (76%) were female. Eight (38%) had preceding symptoms attributable to esophageal GCT. Upon endoscopic evaluation, 12 (57%) GCTs were found in the distal esophagus and 4 (19%) in the mid esophagus. Median lesion size was 7mm (IQR 4-8mm). The largest lesion was 12mm. Endoscopic images of esophageal GCTs are presented in Figure 1A-C. Endoscopic ultrasound was used in 15 (71%) cases. Endosonographic appearance of a granular cell tumor is presented in Figure 2. Resection was performed in all 21 patients. Submucosal lift with normal saline was used in 9 (43%). A total of 20/21 (95%) had en bloc resection upon pathologic examination. Post-resection endoscopic appearance is depicted in Figure 1D. The case without complete resection upon review was a 7mm distal esophageal GCT, resected using saline lift followed by EMR. EUS was not used. Upon pathologic evaluation, tumor was focally present at margins. Repeat endoscopy with biopsies in 6 months showed no residual tumor. No patients experienced bleeding, perforation, or stricturing. No patients have had known recurrence of esophageal GCT. EMR-B of esophageal GCTs achieves complete histopathologic resection with minimal adverse events. EMR-B is safe and effective and seems prudent compared with observation for what could be an aggressive and malignant tumor. EMR-B should be considered first-line therapy for esophageal GCT up to 12 mm in diameter.Figure 2Classic EUS appearance of a granular cell tumor (indicated by red arrow), with surrounding submucosal layer (blue dashed line) and muscularis layer (yellow arrow)View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Experience with Esophageal Granular Cell Tumors: Clinical and Endoscopic Analysis of 22 Cases.

Esophageal granular cell tumors (GCTs) are rare tumors. Differences in reports on the clinical features of GCTs in the esophagus and some controversies about the diagnostic strategy for esophageal GCTs exist. We aimed to investigate the clinical features and diagnosis of esophageal GCTs. Additionally, we sought to determine the prevalence of gastroesophageal reflux disease and reflux esophagitis in patients with esophageal GCTs. We retrospectively studied the clinical features, endoscopic features, and management of 22 patients with esophageal GCTs. Esophageal GCTs were more common in men than in women with a ratio of 1.2:1 and were predominantly found in the distal esophagus. Ten patients with esophageal GCTs had regurgitation and/or heartburn symptoms, and eight patients were confirmed to have reflux esophagitis by endoscopy. All esophageal GCTs were protuberant lesions covered by normal esophageal epithelium. The endoscopic morphology of esophageal GCTs was diverse. On endoscopic ultrasonography, these tumors appeared as homogeneous or inhomogeneous hypoechoic lesions with clear borders originating from the submucosal or mucosal layer. Eleven patients underwent endoscopic forceps biopsy at the first endoscopy, and only six patients were correctly diagnosed by pathology. Nevertheless, the 18 lesions treated with endoscopic resection were all correctly diagnosed without complications, and no patients developed recurrence during the follow-up period. The occurrence of esophageal GCTs may be related to esophageal inflammation. As a method for obtaining an accurate pathological diagnosis and for treatment, endoscopic resection should be offered as the primary option for patients with esophageal GCTs.

Ligation-assisted endoscopic mucosal resection for esophageal granular cell tumors is safe and effective.

Given their malignant potential, resection of esophageal granular cell tumors (GCTs) is often undertaken, yet the optimal technique is unknown. We present a large series of dedicated endoscopic resection using band ligation (EMR-B) of esophageal GCTs. Patients diagnosed with esophageal GCTs between 2002 and 2019 were identified using a prospectively collected pathology database. Endoscopic reports were reviewed, and patients who underwent dedicated EMR-B of esophageal GCTs were included. Medical records were queried for demographics, findings, adverse events, and follow-up. We identified 21 patients who underwent dedicated EMR-B for previously identified esophageal GCT. Median age was 39years; 16 (76%) were female. Eight (38%) had preceding signs or symptoms, potentially attributable to the GCT. Upon endoscopic evaluation, 12 (57%) were found in the distal esophagus. Endoscopic ultrasound was used in 15 cases (71%). Median lesion size was 7mm, interquartile range 4mm-8mm. The largest lesion was 12mm. A total of 20 (95%) had en bloc resection confirmed with pathologic examination. The only patient with tumor extending to the resection margin underwent surveillance endoscopy that showed no residual tumor. No patients experienced bleeding, perforation, or stricturing in our series. No patients have had known recurrence of their esophageal GCT. EMR-B of esophageal GCT achieves complete histopathologic resection with minimal adverse events. EMR-B is safe and effective and seems prudent compared with observation for what could be an aggressive and malignant tumor. EMR-B should be considered first-line therapy when resecting esophageal GCT up to 12mm in diameter.

Endoscopic Resection of a Granular Cell Tumor: A Case Report

Background: Granular cell tumors (GCTs) are benign soft tissue tumors that are thought to originate from Schwann cells. They are relatively rare, and presentation in the gastrointestinal tract is even more uncommon. Case report: In this paper, we present a 26-year-old female patient who was incidentally diagnosed with an esophageal GCT. The tumor was initially detected by endoscopy, evaluated by endoscopic ultrasonography (EUS), then resected using an endoscopic mucosal resection technique