Esophageal Gastrointestinal Stromal Tumors Research Articles

Optimal management options for esophageal gastrointestinal stromal tumors (E-GIST)

ObjectiveTo explore the different treatment modalities for esophageal gastrointestinal stromal tumors (E-GIST) and their respective applicability and clinical outcomes. MethodsThis is a retrospective study in which consecutive patients diagnosed with E-GIST at our hospital from January 2017 to August 2023 were included. The clinical characteristics of all the patients as well as long-term quality of life were recorded and analyzed. ResultsA total of 23 (12 males, 11 females) E-GIST patients with a mean age of 56.7 ± 12.0 years were included in this study. Common symptoms, including upper abdominal pain, acid reflux, and heartburn, accounted for over 60 % of cases. Fifteen patients underwent endoscopic resection, five patients underwent surgical resection, two patients underwent surgical resection after receiving preoperative imatinib therapy, and one patient received conservative management. ConclusionDifferent treatment strategies may be applied to the patients with E-GIST depending on the their clinical features. Our study provides insights into precise treatment for different patients. However, due to the rarity of the disease, it is challenging to collect a large sample size from a single center, necessitating more multicenter prospective large-scale studies.

Endoscopic resection for esophageal gastrointestinal stromal tumors: a multi-center feasibility study

Endoscopic resection for esophageal gastrointestinal stromal tumors: a multi-center feasibility study

Clinical outcomes of endoscopic resection for the treatment of esophageal gastrointestinal stromal tumors: a ten-year experience from a large tertiary center in China.

Esophageal gastrointestinal stromal tumors (E-GISTs) are extremely rare and surgical resection is the recommended approach. However, surgical resection usually causes severe trauma that may result in significant postoperative morbidity. Endoscopic resection (ER) has developed rapidly in recent years and has been widely used in gastrointestinal lesions. Nevertheless, the feasibility and efficacy of ER in the management of E-GISTs are unknown. Retrospective data were collected from January 2011 to December 2020 in a large tertiary center of China. Twenty-eight patients with E-GISTs treated by ER were included in the study. Of the 28 patients, there were 21 males and 7 females, with a median age of 55years (40-70years). The median tumor size was 15mm (5-80mm). The technical success rate was 100% (28/28), while the en bloc resection rate was 96.4% (27/28). The median operation time was 35min (10-410min). Sixteen (57.2%) tumors were categorized into very low risk group, six (21.4%) into low risk group, and six (21.4%) into high risk group. Pathologists carefully examined margins of each lesion. There were 11 lesions (39.3%) determined as R0 resection and 17 lesions (60.7%) as R1 resection with positive margins. The median hospital stay was 2days (range, 1-8days). One patient suffered from hydrothorax and required drainage, leading to a major adverse event rate of 3.6% (1/28). There was no conversion to surgery, and no death occurred within 30days after the procedure. Imatinib was given to two patients after ER under multidisciplinary team surveillance. During follow-up (median of 54months, 9-122months), no recurrences or metastasis were observed. ER is safe and effective for E-GISTs and might become an optional choice in the future. Multicenter, prospective, large samples with long-term follow-up studies are still needed.

Rectal and esophageal gastrointestinal stromal tumors—how to deal with it?—technical approach and role of neoadjuvant treatment

Rectal and esophageal gastrointestinal stromal tumors—how to deal with it?—technical approach and role of neoadjuvant treatment

Effect of tumor location on the efficacy of chemotherapy for gastrointestinal stromal tumors.

790 Background: Gastrointestinal stromal tumors (GISTs) arise from multiple sites within the gastrointestinal tract. Since the introduction of imatinib in 2002, patients have seen improved overall survival. However, a recent National Cancer Database (NCDB) study suggested no survival benefit to adjuvant imatinib for colorectal GIST patients. Another recent study showed that small bowel GISTs have larger median tumor size and higher incidence of tumors with at least 5 mitoses compared to gastric GISTs. These findings suggest a differential tumor biology for GISTs arising from different primary tumor sites, potentially conferring differential response to imatinib. In this NCDB study, we compare overall survival outcomes with and without chemotherapy, stratifying by primary tumor site. Methods: We queried the NCDB from 2004-2018 for patients with GIST histology. We excluded patients with age <40, age>80, high comorbidity score, and patients who received additional therapy other than surgery or chemotherapy. Of the 22,846 patients meeting criteria for our study, 27 patients had tumors originating from the anus, 454 from the colon, 173 from the esophagus, 15,296 from the stomach, 556 from the rectum, 32 from the rectosigmoid junction, and 6,308 from the small intestine. 8,983 (39%) received chemotherapy. Treatment with chemotherapy was used to extrapolate response to imatinib, the mainstay of treatment. Results: Cox proportional hazards analysis demonstrated increased hazard of death for chemotherapy, increasing age, and comorbidity score of 1. Positive predictive factors for survival included female sex and later year of diagnosis. For anal, esophageal, gastric, and rectosigmoid GIST patients, there was no statistically significant overall survival benefit for any treatment modality. For colonic GISTs, neoadjuvant chemotherapy combined with surgery improved overall survival. For rectal GISTs, adjuvant chemotherapy with surgery also provided survival benefit. For small intestinal GISTs, surgery alone or surgery with neoadjuvant, adjuvant, or both neoadjuvant and adjuvant chemotherapy improved overall survival. Conclusions: While our study is retrospective and limited as a NCDB study, our Kaplan-Meier survival analysis demonstrated survival benefit for chemotherapy only in patients with colon, rectal, or small intestinal GIST tumors. Our Cox proportional analysis demonstrated an overall negative survival impact for chemotherapy. These findings lend further support to the idea that differential tumor biology at each primary site may lead to differential response to imatinib. Overall, our study provides rationale for further investigating the heterogeneity of GIST tumor microenvironments, as these differences could contribute to the differential treatment outcomes.

Large esophageal gastrointestinal stromal tumors resected thoracoscopically after oral imatinib therapy: a report of two cases

Esophageal gastrointestinal stromal tumors (GISTs) are very rare, accounting for 2–5% of all GISTs. As with other GISTs, the principle of surgical treatment is complete resection with negative margins. In addition to biological grades of GISTs itselves, local recurrence due to capsular damage is a known risk. We describe two cases of massive esophageal GISTs that were successfully resected thoracoscopically after 2 months administration of 400 mg imatinib, with some discussion of the literature. Case 1, the patient was a 51-years-old man. After treated with 400 mg of imatinib as preoperative chemotherapy for 2 months, we performed surgery that included right thoracoscopic subtotal esophagectomy, gastric tube reconstruction, and jejunostomy. The resection specimen and histopathology were esophageal GIST-LtMtAeG, 110 × 95 mm. The postoperative course was uneventful, and was discharged on postoperative day 14. The patient has been recurrence free for 11 months postoperatively. Case 2, the patient was a 70-years-old man. After treated with 400 mg of imatinib as preoperative chemotherapy for 2 months, we performed surgery that included right thoracoscopic subtotal esophagectomy, gastric tube reconstruction, and jejunostomy. The resection specimen and histopathology were esophageal GIST-LtAeG, 90 × 52 mm. The postoperative course was uneventful, and was discharged on postoperative day 14. The patient has been recurrence free for 9 months postoperatively.

S2436 A Rare Case of a Large Esophageal Gastrointestinal Stromal Tumor

Introduction: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms, accounting for approximately 1 to 2 percent of primary gastrointestinal (GI) cancers. Presenting symptoms include abdominal pain, nausea, vomiting, early satiety, bleeding, and dysphagia, but vary depending on primary tumor location. Most tumors are discovered incidentally with the majority found in the stomach (40 to 60 percent) followed by small bowel (25-30 percent), colon and rectum (5-15 percent). We present a case of an elderly patient diagnosed with esophageal GIST after he was found to have an incidental esophageal mass on imaging. Case Description/Methods: 80-year-old male with PMH of HTN, HLD, DM2, CKD Stage III, COPD, former tobacco user was referred for an upper endoscopy for evaluation of an incidental finding on CT scan. CT demonstrated a mass arising from the distal esophagus, measuring 6.5 x 7.5 cm. Patient denied any dysphagia, abdominal pain, nausea, vomiting, weight loss, hematemesis, or melena. Endoscopy showed submucosal mass in the distal esophagus (Figure). Biopsy was inconclusive and subsequent EUS-guided fine needle biopsy was performed. Upper endoscopic ultrasound (EUS) showed an intramural lesion in the distal esophagus arising from the muscularis propria. Pathology results confirmed GIST, positive for CD117 and DOG-1. Further testing revealed a mutation in the KIT gene exon 11. Patient is currently undergoing neoadjuvant therapy with imatinib mesylate with anticipated surgical resection. Discussion: This is a rare case of a large esophageal GIST. Most mesenchymal esophageal tumors are leiomyomas, and GIST only accounts for 0.7% of them. EUS with fine need aspiration biopsy is the most accurate method for diagnosis and definite diagnosis is made histologically. Majority of GISTs, expresses the CD117 antigen, a KIT receptor tyrosine kinase. Another commonly found mutation is the platelet derived growth factor (PDGFRA) tyrosine kinase gene. Tumor size and mitotic rate are used for prognostication, with increased malignancy risk seen in tumor size > 3 cm with irregular margins. Tumor location is also a key factor in risk stratification; non gastric primary location has higher rates of metastases. Liver and peritoneum are the most common sites of metastatic GISTs. Treatment focuses on surgical resection and neoadjuvant therapy with imatinib. Given significant implication for its management, esophageal GIST should be considered in differential diagnosis and distinguished from more common esophageal leiomyomas.Figure 1.: Submucosal mass in distal esophagus.

389. BENEFITS OF BILATERAL THORACOSCOPIC SURGERY IN THE PRONE POSITION FOR A LARGE ESOPHAGEAL TUMOR

Abstract Esophageal gastrointestinal stromal tumors (GISTs) are relatively less symptomatic and may have a significantly large tumor diameter at diagnosis. Large esophageal tumors are difficult to resect because it is tough to maintain the surgical field in the narrow mediastinal region. In addition, because GIST has a risk of dissemination if the tumor collapses during surgery, it must be resected with as little force as possible. We herein report a middle and lower thoracic esophageal GIST with a maximum lateral diameter of 12 cm that could be safely resected by a bilateral thoracoscopic approach in the prone position. The case was that of a 70-year-old man. Since his tumor diameter was large and the surrounding organs were severely compressed, it was deemed to be borderline resectable, and preoperative chemotherapy with imatinib was decided. However, administration of imatinib did not result in tumor shrinkage, and interstitial pneumonia occurred. Thus, the final decision was to resect it. It was difficult to remove the left side of the tumor by normal thoracoscopic operation from the right side as the tumor was large. There was also the risk of the tumor collapsing due to unreasonable traction. Therefore, we selected a surgical procedure in which the left and right sides of the tumor were detached by bilateral thoracoscopic operation in the prone position and then the tumor was removed from the abdominal cavity by a transesophageal hiatal operation under laparotomy. Followed by gastric tube reconstruction by the posterior sternal route. The postoperative course was uneventful. In this case, taking advantage of the thoracoscopic approach from both sides, the tumor could be safely resected without unreasonable traction. Furthermore, the prone position has the advantage that bilateral thoracoscopic operation is possible without changing the position. This technique could also be applied for the excision of esophageal cancer involving the left side.

Hemothorax due to a ruptured esophageal gastrointestinal stromal tumor: case report

BackgroundEsophageal gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal interstitium, and comprise less than 0.7% of all GISTs. The presentation of esophageal GIST is relatively benign, commonly characterized by symptoms of dysphagia and gastrointestinal bleed. On the contrary, it is highly unusual for these tumors to present as surgical emergencies.Case presentationHere, we describe a case of hemothorax secondary to the rupture of a massive (19 cm) esophageal GIST in a 79-year-old male. The patient presented with mild back pain, vomiting, and hypotension. A CT scan revealed significant mediastinal enlargement and left hemothorax. We conducted an emergency thoracotomy which revealed a 19 × 15 × 7 cm ruptured esophageal tumor that was bleeding profusely into the left thoracic cavity. Piecemeal resection without esophagectomy was performed to achieve hemostasis. Pathological evaluation of resected tissue confirmed the diagnosis of GIST. The patient was provided adjuvant imatinib therapy and remains progression-free at the 10-month follow-up.ConclusionsTo the best of our knowledge, this is the first reported case of life-threatening hemothorax caused by a ruptured esophageal GIST. Findings from this case may aid in the diagnosis and management of these rare tumors.

Gastrointestinal stromal tumors (GISTs) arising in uncommon locations: clinicopathologic features and risk assessment of esophageal, colonic, and appendiceal GISTs

Risk stratification of gastrointestinal stromal tumors (GISTs) is based on experience with tumors of the stomach, small bowel, and rectum, which are far more common than GISTs of other sites. In this study from 47 institutions, we analyzed GISTs of the esophagus (n = 102), colon (n = 136), and appendix (n = 27) for their size, mitotic rate, morphology, and outcome to determine which criteria predict their behavior. Esophageal GISTs were small (median: 2.5 cm) with spindle cell morphology and a low mitotic rate (mean: 3.6/5 mm2). Twelve (12%) tumors progressed, including 11 with a mitotic rate >5/5 mm2 and one large (6.8 cm) GIST with a mitotic rate of 2/5 mm2. Colonic GISTs were smaller (median: 1.4 cm) and presented with abdominal pain or bleeding in 29% of cases. Most (92%) were composed of spindle cells with a mean mitotic rate of 4.6/5 mm2. Sixteen (12%) tumors progressed: 14 had mitotic rates >5/5 mm2, and two were >5.0 cm with a mitotic rate <5/5 mm2. All but one appendiceal GIST measured <2.0 cm. These tumors were composed of spindle cells with low mitotic rates (<5/5 mm2), and none progressed. Our results suggest that progression risk among esophageal and colonic GISTs is associated with increased mitotic activity (>5/5 mm2) and size >5.0 cm. These findings support the use of size and mitotic rate for prognostication of GISTs in these locations, similar to tumors of the stomach, small bowel, and rectum.

Endoscopic treatment can be a viable therapeutic option for esophageal gastrointestinal stromal tumors

Background Esophageal gastrointestinal stromal tumors(GISTs) are extremely rare. We sought to determine whether endoscopic treatment can be a viable therapeutic option for esophageal GISTs. Methods A total of 20 cases with histological diagnosis of esophageal GISTs were obtained from our center between 2008 and 2020. Data on the clinicopathological features and treatment were recorded. Results There were 9 males (45%) and 11 females (55%) in this study, with a median age of 56 years. The tumors preferentially occurred in the middle and lower parts of the thoracic esophagus (45 and 40%, respectively). The mean size of the tumors was 2.27 cm and mitotic index was no more than 5/50 high power field (HPF) in all patients. In this study, 11 patients received endoscopic treatment and nine patients underwent surgical resection. Tumors ranged from 0.6 to 4 cm in the endoscopic treatment patients and 0.5 to 7 cm in the surgical patients. There were no significant differences in gender, age, symptoms, tumor location, tumor size, mitotic index, and adjuvant imatinib therapy between the endoscopic treatment group and the surgery group (all p > .05). The Kaplan-Meier curve suggested that there was also no significant difference in disease-free survival between the two groups (p = .264). Conclusions Endoscopic treatment may be an option for the treatment of esophageal GISTs smaller than 5 cm with a mitotic index no more than 5/50 HPF.

Review of Management of Gastrointestinal Stromal Tumor in Low-Resource Centers

Gastrointestinal stromal tumors (GIST) account for 1% to 3% of gastrointestinal tract tumors and are the most common of the mesenchymal tumors. Carcinogenesis of GIST arises in the interstitial cells of Cajal (ICC) and in the myenteric plexus of the gastrointestinal tract due to a mutation of the kinase receptor (KIT, also known as CD117) and the platelet-derived growth factor A (PDGFA) gene leading to activation of the tyrosine kinase receptor. The exact incidence and prevalence of GIST is not known. Symptoms of GIST are non-specific; they present with GI bleeding due to ulceration (50%), abdominal pain (20% to 50%), dysphagia (esophageal GIST) and GI obstruction (10% to 30%) (7,10). Signs include abdominal mass and fullness. A computerized tomographic (CT) scan is the preferred imaging to evaluate GIST. Diagnosis is confirmed by immunohistochemical (IHC) staining a of biopsy sample for medical treatment tyrosine kinase inhibitors (TKI). Surgical resection with negative microscopic margins is the gold standard treatment of GIST. TKI are required for tumor reduction to increase chances of respectability (neoadjuvant therapy) or to prevent recurrence and reduce the progression of advanced, resectable GIST.

A nationwide survey on esophageal gastrointestinal stromal tumors at accredited institutions by the Japan Esophageal Society.

As esophageal gastrointestinal stromal tumors (GISTs) are very rare, their clinicopathological features remain obscure. We conducted a nationwide survey to clarify the characteristics of these tumors and to establish a consensus on their diagnosis and treatment. The clinicopathological information of patients with esophageal GISTs who underwent treatment between January 2010 and June 2016 at the accredited institutions by the Japan Esophageal Society was collected via a questionnaire method and analyzed statistically. Fifty-one patients (29 men and 22 women; median age, 68years) were recruited from 31 institutions. Diagnosis was triggered most frequently during screening and other disease scrutiny. Symptoms were seen only in 17 patients: highest in 11 patients with dysphagia. Thirty-five patients underwent surgery alone; 15 patients, surgery with imatinib therapy; and one patient, endoscopic resection. The tumors preferentially occurred in the lower and middle parts of the thoracic esophagus, with a median size of 36.5mm. Neoadjuvant and adjuvant imatinib therapies were performed in seven and eight patients, respectively. Administration of imatinib 400mg/day was the standard regimen. Postoperative follow-up observations were conducted mostly via computed tomography (CT) scans every 3 or 6months until 5years after surgery. The tumors recurred in ten patients within 5years postoperatively (high risk, 38.5%; intermediate risk, 20%; low risk, 0%; very low risk, 0%; three cases of relapse with an unknown risk assessment). A patient with a high-risk GIST died from the tumor 54months after surgery. This nationwide survey revealed the current status of esophageal GISTs in Japan and provided important information for making a consensus on the treatment and follow-up method.

Esophageal Gastrointestinal Stromal Tumor with Rare Intracranial Metastasis

Introduction. Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors and constitute the largest group of nonepithelial digestive neoplasms. However, they do not represent more than 1% of primary digestive tumors. They commonly metastasize to the liver and peritoneum, but brain metastases are extremely rare. Clinical Case. A 76-year-old woman with a diagnosis of esophageal GIST with liver and lung metastases for 13 years, medicated with imatinib, is presented. She was brought to the emergency department after falling and due to changes in behavior and vertigo with 24 hours of evolution. On physical examination, she presented changes in behavior, dysarthria, dysmetria on the right, gait imbalance, and no motor or sensory deficits. On brain computed tomography and posteriorly on magnetic resonance, 2 lesions were observed, left frontal and right cerebellar, compatible with metastatic lesions. After contribution of neurosurgery, histology was obtained that confirmed the lesions were GIST metastases. Imatinib was maintained, and whole brain radiotherapy was performed. After 6 months, she died. Discussion. The rarity of GIST brain metastases is noteworthy, and because of that, there is not enough experience to be certain of the best treatment. Our patient lived for 13 years with excellent disease control with imatinib, but the fact that it does not cross the blood-brain barrier makes it not useful in preventing or treating brain lesions. New tyrosine kinase inhibitors that may cross the blood-brain barrier could be the answer to these cases.

Successful Cryoablation Eradication of an Esophageal Gastrointestinal Stromal Tumor

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. They are most commonly found in the stomach but have also been described in other segments of the gastrointestinal tract, including the small intestine and more rarely, the esophagus. For gastric and intestinal GISTs, the current standard of care involves surgery and tyrosine kinase inhibitors. However, the appropriate treatment strategy for the management of esophageal GISTs are less well-established. In this case report, we present a case of an esophageal GIST successfully treated by cryoablation therapy. A 69-year-old male with a prior history of Barrett's esophagus and esophageal adenocarcinoma (T1a disease), previously successfully treated with resection, was found to have a ulcerated 13x5 mm mid esophageal GIST on surveillance esophagogastroduodenoscopy (EGD) and endoscopic ultrasound (EUS). The patient was deemed a poor surgical candidate due to his multiple medical comorbidities and a prior history of a complicated post-operative course following his prior esophageal resection, and so cryoablation therapy was offered. The TruFreeze spray cryotherapy system (CSA Medical Inc, Lexington, MA, USA) was used for this procedure. The tumor was treated for 3 freeze-thaw cycles lasting 30 seconds each. On first followup EUS, the GIST was noted to have decreased in size to 10x2 mm on EUS. At 3-month follow up EGD, the GIST had shrunken considerably, with only a slight bump and subtle vessel dilation noted at the site. Cryotherapy was again performed over this area with three 30-second cycles. The patient did report some post-procedure discomfort this time, which self-resolved over a period of 2 weeks. On subsequent EGD/EUS, the previous site of the GIST was replaced by a small scar. No residual lesion was identified on EUS. Biopsy from the scar had no evidence of GIST, dysplasia or malignancy. Case Highlights: Our case demonstrates the potential for cryoablation therapy to resolve esophageal GISTs without invasive surgical intervention. Cryoablation therapy was successful and well-tolerated by the patient, and no significant adverse events were noted during the procedure or on follow up aside from mild discomfort that self-resolved. Cryotherapy is generally a safe procedure and is associated with low rates of serious adverse events and has the advantage of allowing for repeat sessions or other treatments if necessary.1824_A Figure 1. Endoscopic view of esophageal gastrointestinal stromal tumor1824_B Figure 2. Cryotherapy being applied to GIST1824_C Figure 3. Appearance of GIST at 3-month EGD follow up. Mild bump with dilated vessels noted at site of esophageal GIST

Is There a Place for Thoracoscopic Enucleation of Esophageal Gastrointestinal Stromal Tumors?

Esophageal gastrointestinal stromal tumors (E-GISTs) represent less than 1% of all GISTs. The rarity of this lesion precludes the realization of randomized studies, and its treatment remains a matter of debate. We aimed to evaluate the feasibility of enucleation by video-assisted thoracic surgery (VATS) for low- to intermediate-risk E-GIST. We performed a retrospective review of patients treated by enucleation through VATS between January 2004 and January 2014 and reviewed the literature. We included five patients (four men and one woman). Mean age was 53 years (range: 49-79). Three patients were diagnosed because of dysphagia and two others incidentally. The diagnosis was made by immunostaining demonstrating CD117 expression on tumor cells. The mitotic index of all E-GISTs was low (≤ 5 per 50 high-power field). Median postoperative follow-up was 5.5 years, and there was no recurrence. Thoracoscopic enucleation of E-GIST seems to represent a valuable option as the postoperative morbidity/mortality is low and the oncological outcome is good for low-to-intermediate grade of malignity tumors.This is a retrospective study focused on minimally invasive treatment of E-GIST. We evaluated the feasibility of VATS enucleation of low-to-medium grade of malignity E-GIST.

Clinicopathologic features and outcomes of gastrointestinal stromal tumors arising from the esophagus and gastroesophageal junction.

Our aim was to characterize the clinicopathological features and outcomes of gastrointestinal stromal tumors (GISTs) arising from the esophagus and gastroesophageal junction (GEJ) and describe the survival of patients treated at our institution as well as from a national hospital-based registry. Twenty-eight cases were identified using the Mayo Clinic Cancer Registry from 1997 to 2016, and 1,010 cases from the National Cancer Database (NCDB) between 2004 and 2014, with analysis of TNM staging, histopathological features, mitotic index, immunohistochemical studies, and KIT mutational analysis. At Mayo Clinic, the tumors ranged in size from 0.3-13 cm (mean 5.40 cm). IHC results were: CD117 (KIT) in 100% (23/23 cases) and DOG1 in 100% (6/6), followed by CD34 (85.7%, 12/14), smooth muscle actin (27.8%, 5/18), desmin (18.2%, 2/11), and S-100 protein (13.3%, 2/15). Mutational analysis (performed in 10 cases) showed KIT exon 11 mutations in 8 cases; KIT mutation was not identified in 2 cases (presumed wild-type). Two-thirds of patients underwent surgery, of which 70% had an esophagectomy. Fourteen patients received adjuvant imatinib mesylate. Five patients had liver metastases at the time of diagnosis; none had lymph node metastases. A total of 38.9% of cases had recurrent or metastatic disease. Complete clinical follow-up was available for 10 patients (median follow-up duration 31.5 months; range, 10-145 months): one (male) had a local recurrence at the anastomotic site and one (female) suffered a liver metastasis; the others were either disease-free or had stable disease at the time of last follow-up. There was a significant association seen among metastatic disease and mitotic count >5/50 high-powered field (HPF) (P=0.016), with median mitotic rate 90/50 HPF (range, 7-500) for metastatic tumors versus 6/50 HPF (range, 0-100) for non-metastatic tumors. For metastatic disease, median tumor size was 7.3 cm (range, 1-66 cm) compared to 4.8 cm (range, 0.02-71 cm) for non-metastatic disease, which was also statistically significant (P≤0.0001). Two hundred and fifty-eight NCDB cases were risk stratified using the Joensuu criteria. Among 89 low risk category tumors, only 2 (2.2%) were ultimately metastatic. A total of 10.9% (15/138) of high risk category tumors were metastatic. The median overall survival (OS) from the time of diagnosis for the Mayo Clinic cohort was 129.5 months (95% CI, 55.7-not reached), with 5-year OS 85.7%. Median OS for the NCDB cohort was 135.95 months (95% CI, 104.08-not reached) with 5-year OS 68.2%. Superior OS was seen in females (HR 0.67, 95% CI, 0.49-0.89, P=0.006). Among esophageal and GEJ GISTs, metastatic disease was associated with increased mitotic count and increased tumor size. Men were found to have inferior OS. The Joensuu risk criteria were validated for risk stratification of esophageal and GEJ GISTs.

Gastrointestinal stromal tumor of the esophagus: current issues of diagnosis, surgery and drug therapy.

Gastrointestinal stromal tumors (GISTs) often arise in the stomach and small intestine, while esophageal GISTs are rare. Due to their rarity, clinicopathological data on esophageal GISTs are extremely limited, and this results in a lack of clear recommendations concerning optimal surgical management for esophageal GISTs. It is difficult to distinguish esophageal GIST from leiomyoma, the most frequent esophageal mesenchymal tumor, prior to resection, because the two types of tumors appear similar on computed tomography (CT), endoscopic ultrasound (EUS), and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). Fine-needle aspiration biopsy (FNAB) under EUS enables definitive diagnosis, but it is often avoided because scarring could make enucleation more difficult and increase the risk of tumor dissemination by capsule destruction. Esophageal segmental and wedge resections are not usually performed due to the anatomical peculiarity of the esophagus, and the surgical options are limited to the highly invasive esophagectomy or the much less invasive surgical tumor enucleation. The decision as to which surgical procedure should be performed for esophageal GISTs is still under debate. Tumor enucleation may be permitted for smaller tumors, and esophagectomy may be recommended for larger GISTs or high-risk tumors with a high mitotic rate. The purpose of neoadjuvant imatinib administration is downsizing of the GIST to reduce the extent of resection and to reduce the risk of intraoperative complications, including tumor rupture. The efficacy of neoadjuvant/adjuvant imatinib therapy for esophageal GISTs is poorly understood, because the reports are limited to case reports or case series with small numbers. More clinicopathological data and clinical trials for esophageal GIST are expected.

A Case of Esophageal Gastrointestinal Stromal Tumor Managed With Endoscopic Ultrasound Surveillance

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. They are classified based on their immunohistochemical studies with the expression of c-kit and typically present between 40-50-year-olds. GIST predominantly occur in the stomach (50%) and intestine (25%), followed by the colon and rectum (10%) and rarely in the esophagus (< 5%). Due to the rarity of esophageal GIST (ESOGIST), they are poorly understood and there is a paucity of research evaluating tumor characteristics and management. Our patient is an 83-year-old Hispanic male with a history of prior esophageal impactions due to known Schatzki's rings, presenting with postprandial vomiting. He denies any constitutional symptoms, dysphagia or odynophagia. His initial physical exam and labs are unremarkable. Endoscopy on admission shows a Schatzki's ring at the gastroesophageal junction and new 5mm nodule in the distal esophagus with three ulcers in the esophagus, stomach and duodenum respectively. Pathology of the nodule demonstrates squamous cell mucosa with smooth muscle bundles positive for desmin and negative for S100. Stomach biopsy is positive for H. pylori, which is treated with triple therapy. Endoscopic ultrasound (EUS) guided fine needle aspiration shows a spindle cell neoplasm that is C-kit, DOG-1, and SMA positive and desmin negative. Management of lesions < 2cm are controversial. Thus, given the tumor size and no high risk features on EUS, shared-decision-making was utilized to pursue management with EUS surveillance and planned resection if progressive. ESOGIST is uncommon and comprises less than 1% of all GIST. For lesions < 2cm with suspicious features on EUS, resection is indicated. Surgical resection is also directed for localized ESOGIST ≥2cm or which are symptomatic, with increased overall survival and disease-free survival with adjuvant Imatinib therapy. Enucleation is recommended for management of small tumors (2-5cm) with esophagectomy recommended for tumors >90mm. More research on the management of tumors between 60-90mm needs to be conducted. For larger tumors preoperative Imatinib therapy should be considered. This can improve resectability and is also the standard of therapy when surgery is contraindicated. This case is unique given the rarity of EOSGIST, and highlights the importance of further research on the management of these tumors to establish continued guidelines for care.

Current research status and progress of primary esophageal gastrointestinal stromal tumors in China

Primary esophageal gastrointestinal stromal tumors (GIST) is a rare independent disease with clinicopathological and molecular features different from other mesenchymal tumors. Authors searched and reviewed associated reports and summarized the morbidity trends, characteristics, diagnosis and treatment of esophageal GIST in China. Data show that the incidence and detection rate of esophageal GIST presents the rising trend. Imaging has some characteristics. Ultrasonography and CT are main methods and effective examinations to detect and prompt diagnosis of esophageal GIST. Pathology and immunohistochemistry are the evidence for a definite diagnosis. Risk classification is the important basis for selecting surgical methods and predicting prognosis. Surgery is the mainstay treatment. Very low and low risk patients with tumors less than 3 cm can choose endoscopic resection. For tumors of 3 to 5 cm, tumor resection must be considered. Thoracoscopy appears to be the first choice for surgery. Medium and high risk patients with tumors >5 cm should be treated with partial resection of esophagus. Surgery combined with targeted therapy and neoadjuvant therapy is the main treatment pattern and research direction.