Large Gastrointestinal Stromal Tumors Research Articles

Unusual presentation of a large GIST: a challenging diagnostic dilemma

Introduction: Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal tract associated withhigh rates of malignant transformation. Most GISTs present asymptomatically. They are best identified by computedtomography (CT) scan and most stain positive for CD 117 (C-Kit), CD34, and/ or DOG-1. There have been many riskstratification classifications systems which are calculated based on tumor size, mitotic rate, location and perforation. Case presentation: A 42 years old man presented with dysphagia and weight loss for 2.5 months. On examination, hewas severely anaemic having a huge intra-abdominal lump. He underwent laparotomy followed by removal of tumormass with partial left lobectomy of the Liver. Conclusion:We present a case of GIST of unusual location and presentation pattern. In general, only completeresection of tumor can lead to cure, although recurrence is common after surgery. Bangladesh Crit Care J March 2023; 11 (1): 54-60

No-touch endoscopic full-thickness resection technique for gastric gastrointestinal stromal tumors.

BACKGROUND : There remain concerns regarding the technical feasibility of endoscopic resection for large gastrointestinal stromal tumors (GISTs), mainly relating to the risk of tumor rupture and the adequacy of the resection margins. This study aimed to evaluate the feasibility and therapeutic outcomes of the newly developed no-touch endoscopic full-thickness resection (NT-EFTR) technique for GISTs. METHODS : In this retrospective study, 92 patients with gastric GISTs undergoing NT-EFTR were included. Clinicopathological, endoscopic, and follow-up data were collected and analyzed. RESULTS : The median tumor size was 2.5 cm and en bloc resection was achieved in all patients with negative surgical margins. The median time of the NT-EFTR procedure was 59.5 minutes. Large tumors (> 3.0 cm), extraluminal tumor growth pattern, and large gastric defects were significant contributors to long operative times. Patients were discharged within 4 days postoperatively. During follow-up, all patients were free from local recurrence and distant metastasis. CONCLUSIONS : NT-EFTR was a feasible method for the resection of gastric GISTs and can be expected to achieve complete radical resection. Large tumors with extraluminal growth and large gastric defects impact procedural difficulty.

Relationship between computed tomography and histological features of gastrointestinal stromal tumors

Background and Aim: Histomorphological and immunohistochemical (IHC) properties of gastrointestinal stromal tumors (GISTs) allow for accurate diagnosis and determine the prognosis. We aimed to evaluate the relationship between the computed tomography (CT) features, histomorphological properties, and IHC markers. Materials and Methods: This retrospective study comprised patients with pathologically confirmed GISTs between 2016 and 2021. The predefined CT characteristics comprised tumor size, hemorrhage and calcification, CT-growth pattern (exophytic/endophytic), and contrast enhancement pattern of the solid component (homogeneous/heterogeneous). The GISTs were divided into groups according to the National Institutes of Health risk category, cell type, presence of necrosis, CD117 and α-SMA positivity, and Ki-67 index. The frequencies of CT phenotypes were compared between groups. Results: Overall 24 (14 [58.3%] males) patients with a median age of 64 (59.5–75.5) having 25 GISTs were included. Of 25 GISTs, 16 (64%) were gastric and 9 (36%) were intestinal in origin. Among CT features, the maximum diameter was higher in epithelioid, infiltrative, a mitotic count ≥5/50, necrotic, high-risk GISTs (P < 0.05 for all). The median tumor size was higher in Ki-67 >8 than Ki-67 <6 subjects (112.5 [39.25–153.75] vs. 22.5 [16.75–57.5] mm, P = 0.014). A heterogeneous enhancement was also more frequent in Ki-67 >8 tumors (P = 0.04). The enhancement pattern did not differ according to CD-117 or SMA positivity. Logistic regression analysis revealed that the only independent predictor of a Ki-67 >8 status was the tumor size (odds ratio: 1.02, 95% confidence interval: 1.001–1.046, P = 0.04). Conclusions: Heterogeneously enhanced large GISTs at CT imaging strongly suggest the presence of poor prognostic factors including a high Ki-67 index and/or high-risk category.

Oncological outcomes of large gastrointestinal stromal tumors treated by laparoscopic resection.

The resection of large gastric gastrointestinal stromal tumors (GISTs) by laparoscopic has been controversial. Extending from our prior study, the long-term oncological outcome of laparoscopic resection of large (5-8cm) gastric GISTs was reported. From 2002 to 2018, a consecutive 66 patients with gastric GISTs of 5-8cm were treated at National Taiwan University Hospital. Among them, 30 patients received open surgery, and 36 received laparoscopic surgery. The clinicopathological data, peri-operative and oncological outcomes were compared between groups. The clinical demographics including sex, age, BMI, tumor locations and ratio of wedge resection were similar between groups. The mean tumor size was 6.0 ± 0.83cm versus 6.3 ± 1.07cm (Open vs. Laparoscopic, p = 0.3). The operation time, blood loss, and post-operative complications, were also similar. The mean hospital stay was shorter in the laparoscopic group (8.8 ± 2.5days) than in the open group (12.0 ± 8.9days), though not significantly different. The median follow-up time was 108 ± 58months (97 ± 50 in laparoscopic group; 122 ± 64 in open group). All except three patients remain disease-free. One in the open group and two in the laparoscopic group had recurrence of tumor, and they were stable of disease under Imatinib treatment. Eight patients died in non-GIST causes during follow-up. The 5-year recurrence-free survival were 100% for the open and 94.2% for the laparoscopic group (p = 0.2). Our data showed that laparoscopic surgery for gastric GIST between 5 and 8cm was safe and oncologically feasible.

Proactive over reactive: the snare-based pretraction method improving the full-thickness resection of a large gastrointestinal stromal tumor.

Proactive over reactive: the snare-based pretraction method improving the full-thickness resection of a large gastrointestinal stromal tumor.

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.

Large sized gastrointestinal stromal tumors

Background: large gastrointestinal stromal tumors (GISTs) represent a surgical challenge for complete en block resection, which is the mainstay strategy for management. This study was done to determine long term oncological outcomes following resection of GISTs >10 cm. Material and methods: This retrospective study included 52 patients who underwent surgical resection for GIST >10 cm. Our main outcome is to determine the overall survival along with the predictors of post-operative recurrence after large GISTs resection. Results: The stomach was the most commonly affected organ (55.8%). Mass size was larger than 20 cm in 36.5% of our patients and the rest of them ranged between 10 – 20 cm. Anatomical/formal resection was performed in 63.5%of patients, and the remaining ones received a limited resection. After follow-up (median 57 months), disease-free and overall survival had mean values of 80.97 and 100.56 months, respectively. Recurrence was encountered in 36.5%, while the total mortality rate was 26.9%. Multivariate analysis revealed that tumor rupture and CD 34 positive tumors were strong predictors of recurrence. Conclusion: surgical resection of GISTs is the mainstay therapy, but it is associated with relatively high rate of recurrence even with adjuvant tyrosine kinase inhibitor therapy (TKIs).

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.

A Giant Posterior Mediastinal Metastatic Mass of GIST Gastric Origin

Gastrointestinal stromal tumors (GISTs) are uncommon visceral sarcomas that predominantly arise from the gastrointestinal tract, particularly from interstitial cells of Cajal. Although the liver is a common site for metastasis, rarely GISTs show extraintestinal manifestation in the bone and lungs. We report a case patient with an uncommon location and unusually large GIST metastasis as a mediastinal mass 3 years after undergoing total gastrectomy and chemotherapy with imatinib, a tyrosine kinase inhibitor.

Intraoperative Finding of a Patient with Uterine Fibroid

INTRODUCTION: Most neoplasms in the pelvic cavity of women arise from organs of the reproductive system. However, diseases of the gastrointestinal and urinary tracts and neoplasms of a neurogenic and primary extraperitoneal nature can be also encountered in the pelvis where they may be mistaken as gynecological pathology. Thus, in an intraoperative discovery of previously undiagnosed foci of tumor lesions the surgical approach has to be changed, which can negatively affect the results of surgical treatment. Gastrointestinal stromal tumors (GISTs) refer to the most common tumors of the gastrointestinal tract originating from the mesenchymal anlage. Up to 70% of GISTs are located in the stomach, 20%40% in the duodenum and small intestine, 5%15% in the colon, and 2%5% in the esophagus, with single tumors occurring in the appendix.&#x0D; CONCLUSION: The article presents a clinical case of a patient who underwent hysterectomy for multiple uterine fibroids and in whom a large GIST of the sigmoid colon was found intraoperatively that was previously mistaken as one of the subserous nodes of uterine fibroids.

60 Neoadjuvant Imatinib Mesylate – an Ace of Spaces for Surgical Management of an Oesophago-Gastric Junction Gastro-Intestinal Stromal Tumour

Abstract Introduction Oesophageal gastro-intestinal stromal tumours (GISTs) are rare, accounting for less than five percent of all GIST, with the majority of these occurring at the oesophago-gastric junction (OGJ). Due to the anatomical location and size of these tumours at presentation, surgical resection is often a major undertaking. Neoadjuvant treatment with tyrosine kinase inhibitors, such as Imatinib mesylate, have the potential to downstage such tumours, thereby limiting the extent of subsequent surgical resection. This may therefore reduce any potentially associated morbidity and mortality. Case Description We report the case of a 75-year-old gentleman who presented with a large OGJ GIST, which presented with dysphagia. The tumour was deemed too large for local excision due to the risk of compromising the lumen at the OGJ in attempting to obtain a clear resection margin. Given the patient’s co-morbidity, oesophagectomy or total gastrectomy was deemed too major an undertaking. Therefore, endoscopic biopsies were obtained with a view to possible neoadjuvant therapy. These confirmed DOG-1 and Exon-11 mutations, suggesting sensitivity to Imatinib mesylate. A standard dose regime of 400mg daily for a planned 36-month course was commenced. Subsequent imaging showed a substantial decrease in tumour bulk and the decision was made to perform a local excision. A local enucleation procedure was performed, and the patient made a sound recovery. Post-operative histology of resected tumour showed almost complete tumour regression. Discussion This significant response to neoadjuvant Imatinib mesylate therapy demonstrates clear potential for its use in similar cases, where radical resection would cause significant morbidity and risk mortality.

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Gastrointestinal stromal tumors (GIST) are defined as mesenchymal tumors of the gastrointestinal tract expressing proto-oncogene protein CD 117. GIST is a rare tumor of the small bowel, which can be difficult to diagnose and has a varied clinical outcome. Prognosis is dependent on tumor size and mitotic count. Radical surgical excision (R0) is the treatment of choice for resectable and non-metastatic disease. Imatinib is a first-line therapy for metastatic, unresectable or recurrent disease. In this case report, we describe large GIST of the small bowel with high malignant potential. Eight years after the operation, the patient is in good general condition with no signs of recurrence of the disease.

Single Surgical Approach for Gastric Gastrointestinal Stromal Tumor and Cholecystolitiasis by Laparoscopic Procedure

Introduction: Gastrointestinal stromal tumor (GIST) is a rare neoplasm representing 80% of gastrointestinal neoplasms. Laparoscopic resection is only recommended for GIST less than 2 cm and 5 cm for laparoscopic wedge resection. Thus, a large GIST is recommended to complete resection through laparotomy. A large GIST of the gastric fundus can be found in patients, which coincides with an upper right abdominal quadrant pain caused by cholecystolithiasis. A suggested laparotomy for tumor resection and cholecystectomy may result in poor recovery in the postoperative period. Therefore, one approach surgery with minimally invasive laparoscopic partial gastrectomy using Endoflex stapler and cholecystectomy was performed to achieve the optimal outcome.Case Presentation: A 43-year-old male had a major complaint of hematemesis and melena for one month and colicky pain in the right upper quadrant of the abdomen with increased intensity in the last two months. Contrast-enhanced abdominal MSCT revealed a solid 4.6 x 4 x 5.6 cm exophytic mass on the gastric fundus, added with 0.5 cm gall bladder stone. Laparoscopic partial gastrectomy and cholecystectomy were performed in a single minimal invasive surgical procedure. The post-operative period was uneventful. The patient experienced minimum pain, early mobilization, and better diet tolerance. He was discharged on day four after the surgery. Besides, the histopathologic result revealed a malignant mesenchymal tumor according to GIST with a tumor-free margin of resection.Conclusions: Single approach surgery with minimal laparoscopic partial gastrectomy and cholecystectomy has provided optimal outcomes for patients with large GIST and cholecystolithiasis.

Complete laparoscopic wedge resection of a giant locally advanced gastric GIST with near pathological complete response after preoperative treatment with imatinib mesylate: A case report.

Introduction and importanceSurgical resection is the only curative treatment for gastrointestinal stromal tumor (GIST). Laparoscopic approach for large (>5 cm) and giant (>10 cm) gastric GIST remains under controversy. What's more, whether laparoscopic surgery could be performed after preoperative imatinib treatment of giant gastric GIST is still unknown.Case presentationWe report a 68-year-old man with a giant (almost 30 cm) locally advanced gastric GIST which required resection of contiguous organs initially. After received 12 months imatinib therapy, the tumor became resectable and he finally achieved a complete laparoscopic wedge resection. Pathological evaluation of the resected specimen revealed a near pathological complete response was obtained. The imatinib treatment was ongoing after surgical resection and there was no radiological or clinical evidence of disease recurrence until to October 2021.Clinical discussionLaparoscopic approach is safe and effective for gastric GIST. Even for lesions greater for 5 cm. However, there are few reports for the application of laparoscopic wedge resection for gastric GIST larger than 10 cm. Preoperative use of imatinib can decrease the tumor size, so that may increase the chance of laparoscopic approach.ConclusionPreoperative imatinib therapy was effective for reducing the gastric GIST, which may increase the chance of minimally invasive approach and organ preservation. Patients with locally advanced GIST could benefit from the multidisciplinary approach.

A rare case of extraluminally pedunculated gastrointestinal stromal tumor with postoperative metastasis to pancreas

The gastrointestinal stromal tumor (GIST) is the most common type of sarcomatous tumor of the gastrointestinal tract. Many GISTs appear as submucosal tumors with intraluminal protrusion. GISTs with malignant features have a high risk of postoperative recurrence or metastasis, usually to the liver or peritoneum. We present a case of gastric GIST with double rarities: arising completely extraluminally with a pedicle and postoperative metastasis to the pancreas. A woman in her seventies diagnosed with a large extraluminal gastric GIST underwent complete removal of the tumor. Nine months later, a solitary metastatic tumor in the pancreas was detected. Imatinib controlled metastasis well for four years before the tumor became resistant. The patient then had a partial pancreatectomy with splenectomy. She is currently free from recurrence. We genetically analyzed the primary and metastatic GISTs and found known mutations related to poor prognosis and imatinib resistance.

An incidental finding of duodenal GIST in a patient with penetrating abdominal trauma: A case report.

Introduction and importanceDuodenal Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the digestive tract. The tumors are derived from interstitial cells of Cajal and usually they present as gastrointestinal bleeding or non-specific abdominal pain, but they can also be asymptomatic even when they have reached considerable size.Case presentationWe report a case of a 40-year-old male presented to our emergency department after sustaining a stab wound on the abdomen. Abdominal imaging tests weren't done; instead an emergency laparotomy was undertaken upfront in an attempt to catch up with a “golden hour”. Intra-operatively, an incidental solid mass measuring 4 × 5 cm was noted on the third/fourth portion of the interior duodenal flexure. Histopathologically, the lesion demonstrated spindled shaped cells which were immunopositive for positive CD117. The patient fared well postoperatively and during subsequent follow up visits.Clinical discussionHigh-risk GISTs have malignant potential. In some cases, GIST is diagnosed as incidentally finding, mostly during surgical procedure, upper endoscopy or radiological studies related to GI tract. Surgical resection is recommended curative option and its extension depends on different factors. Tyrosine kinase inhibitors are of the utmost importance in high-risk and metastatic disease.ConclusionEven considerably large duodenal GISTs can be asymptomatic, thus incidentally found during an abdomino-pelvic imaging tests. Histopathological evaluation of the operative specimen plays a key role in assessing the need of adjuvant therapy, with a significant impact on the patients' survival.

On the Track of New Endoscopic Alternatives for the Treatment of Selected Gastric GISTs-A Pilot Study.

Background and Objectives: GISTs (Gastrointestinal stromal tumors) are the most common mesenchymal gastrointestinal tract tumours and are mainly located in the stomach. Their malignant potential depends on size, location, and type. Endoscopic techniques are a less invasive modality for patients not eligible for surgery. ESD (endoscopic submucosal dissection) is mainly used for the removal of smaller GISTs, with intraluminal growth and a more superficial location. Thus, R0 resection capability in some cases may be not sufficient, limited by tumour size, location in the gastric wall, and its connection level with the muscularis propria. In such cases, an endoscopic full-thickness resection can become a new alternative. In this retrospective pilot study, we evaluated ESD and hybrid resection techniques in terms of safety, efficacy, and disease recurrence for selected types of gastric GISTs. Materials and Methods: A retrospective comparison was conducted in a group of patients who underwent ESD or a hybrid technique combining endoscopic resection with endoscopic suturing using the OverStitch system (HT) for type II or III gastric GISTs. A total of 21 patients aged 70 ± 8 years underwent endoscopic resection. Seventeen lesions were treated with ESD and four with the HT. Results: R0 resection was achieved in all patients treated using HT (type III lesions) and in 53% of those treated with ESD (p = 0.08). None of the type III lesions treated with ESD were excised with R0. Lesions treated with R0 ESD resections were significantly smaller (1.76 ± 0.35 cm) than those with R1 ESD resections (2.39 ± 0.40 cm) (p < 0.01). The mean lesion size treated with the HT was 2.88 ± 0.85 cm. Conclusions: HT may be a new resection modality for large gastric GISTs with high muscularis propria connection grades. Further studies are required to evaluate its safety and efficacy and to form precise inclusion criteria for endoscopic resection techniques.

Jejunal GIST masquerading as an ovarian mass: A case report.

Introduction and importanceGastrointestinal stromal tumors (GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal tract. Jejunal GIST is the rarest subtype. Large GIST can present with an abdominopelvic mass which can be preoperatively misdiagnosed as a gynecological tumor.Case historyA 44-year regularly menstruating woman presented with lower abdominal pain which was diagnosed as a malignant ovarian tumor preoperatively with an MRI. However, intraoperatively, a lobulated mass was present in the abdominal cavity arising from a jejunal portion of the small intestine. With an intraoperative diagnosis of jejunal GIST, the mass was excised and jejunum anastomosed. Histopathology examination report showed GIST which was further confirmed by immunohistochemistry.DiscussionGIST presenting as a large abdominopelvic mass can mimic a gynecological tumor. Contrast-enhanced CT scan is the preferred imaging modality for the evaluation of patients with suspected GIST to determine the extent of the tumor, the presence or absence of metastatic disease alongside evaluation of the possibility of complete resection. Adjuvant imatinib therapy following complete excision can decrease the disease recurrence.ConclusionGynecologists should keep in mind that primary gastrointestinal tumors can present as a pelvic mass. A proper histopathological examination helps to confirm the diagnosis. Complete surgical removal of the tumor should be obtained as it determines the prognosis of the disease.

Feasibility of laparoscopic surgery for large gastric gastrointestinal stromal tumors: systematic review and meta-analysis

Feasibility of laparoscopic surgery for large gastric gastrointestinal stromal tumors: systematic review and meta-analysis

Extensive Degenerative Change Masquerading Histomorphology in a Giant Cystic Gastrointestinal Stromal Tumor With Rare PDGFRA Mutation

Gastrointestinal stromal tumors (GISTs) are the most frequent type of mesenchymal tumors of the gastrointestinal (GI) tract, and most of the time they acquire the mutation of special kinds of genes. GISTs may be familial or inherited and affect several family members of the family or can be sporadic. The risk of GIST is increased in people with mutations in the receptor tyrosine kinase (KIT) and platelet-derived growth factor receptor alpha (PDGFRA) genes. In this report, we present a case of a large GIST with extensive cystic and degenerative change in a 76-year-old female patient with a rare Asp842-His845 deletion mutation detected in PDGFRA exon 18, that required subtotal gastrectomy with en bloc resection.