scholarly journals Gastrointestinal stromal tumors (gists): Definition, clinical, histological, immunohistochemical and molecular genetic features and predictors of malignant potential and differential diagnosis

2002 ◽  
Vol 10 (4) ◽  
pp. 267-271 ◽  
Author(s):  
Vesna Zivkovic ◽  
Vuka Katic ◽  
Aleksandar Nagorni ◽  
Ljubinka Velickovic ◽  
Maja Milentijevic ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Molecular Genetic ◽  
Malignant Potential ◽  
Gi Tract ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cellular Origin ◽  
Genetic Features ◽  
Exon 11 ◽  
Malignant Gists

Gastrointestinal stromal tumors (GISTs) represent a distinct and the most important subset of mesenchymal tumors of the gastrointestinal (GI) tract GISTs occur throughout the GI tract but are usually located in the stomach and small intestine. The cellular origin, differentiation, nomenclature and prognosis of GISTs are controversial. Because GISTs, like the interstitial cells of Cajal, the GI pacemaker cells, express CD117 (c-kit protein), the origin of GISTs from the Cajal cells has recently been suggested. GISTs are also known for their wide variability in clinical behavior and for the difficulty to determine their malignant condition The most reproducible predictors of malignancy are mitotic count >1-5 per10 high-powered fields (HPF), size >5 cm, tumor necrosis, infiltration and metastasis to other sites. However, some tumors with mitotic activity <1/10 HPF may metastasize indicating some uncertainty in malignant potential of GISTs, especially those larger than 5 cm. Recently, mutations in c-kit gene (exon 11) preferentially occur in malignant GISTs and may be a clinically useful adjunct marker in evaluation of GISTs. In conclusion, the strong CD117 expression mostly defines primary GI mesenchymal tumors as GIST. Specific identification of GIST may become clinically important if therapies targeting the c-kit tyrosine kinase activation become available.

Imatinib in the Management of Multiple Gastrointestinal Stromal Tumors Associated With a Germline KIT K642E Mutation

2007 ◽  
Vol 131 (9) ◽  
pp. 1393-1396
Author(s):  
Janet Graham ◽  
Maria Debiec-Rychter ◽  
Christopher L. Corless ◽  
Robin Reid ◽  
Rosemarie Davidson ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Germline Mutations ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Pdgfra Gene ◽  
Oncogenic Mutations ◽  
Esophageal Tumors ◽  
Exon 11 ◽  
Cells Of Cajal

Abstract Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gut and are distinguished by expression of CD117 (c-Kit). Oncogenic mutations in the KIT or PDGFRA gene are detected in approximately 85% of sporadic GISTs. In recent years, examples of familial GIST have been reported in which germline mutations of KIT or PDGFRA result in multiple GISTs, skin disorders, and other abnormalities. The most common germline mutations are in KIT exon 11, mutations in exons 8 and 17 have also been described, and there are 2 families with germline PDGFRA mutations. We present a case in which a germline KIT exon 13 mutation (K642E) was discovered in a patient with multiple GISTs of rectum, small intestine, and esophagus, as well as diffuse hyperplasia of the interstitial cells of Cajal. To our knowledge, this is only the second germline example of this particular mutation. The patient's esophageal tumors were stabilized with imatinib.

Cellular Origin of Gastrointestinal Stromal Tumors

2000 ◽  
Vol 124 (10) ◽  
pp. 1471-1475 ◽  
Author(s):  
Lina Wang ◽  
Hernan Vargas ◽  
Samuel W. French
Keyword(s):  
Stem Cells ◽  
Smooth Muscle ◽  
Gastrointestinal Tract ◽  
Gastrointestinal Stromal Tumors ◽  
Benign Tumors ◽  
Cell Phenotype ◽  
Stromal Tumors ◽  
Cellular Origin ◽  
Benign Leiomyoma ◽  
Malignant Gists

Abstract Context.—Interstitial cells of Cajal (ICCs), also known as pacemaker cells, are cells in the gastrointestinal tract that play a role in the control of gut motility. The ICCs express the c-kit proto-oncogene encoding a type III tyrosine kinase (KIT) receptor, a ligand that is known as stem cell factor (SCF). The maturation of ICCs is dependent on SCF-KIT interaction. The cellular origin, differentiation, nomenclature, and prognosis of gastrointestinal stromal tumors (GISTs) are controversial. Objective.—To test the hypothesis that GISTs originate from CD34-positive stem cells and differentiate toward an ICC phenotype. Materials and Methods.—We studied 27 cases of smooth muscle differentiated GISTs collected for 14 years (1985–1999), including 8 benign (leiomyoma), 15 malignant primary (leiomyosarcoma), and 4 metastatic to the liver. Immunohistochemical studies of selected lineage-directed monoclonal antibodies of c-kit (CD117), CD34, vimentin, desmin, α-actin, S100, and MIB-1 were performed on both normal and tumor tissues. Results.—Immunoperoxidase stains of normal gastrointestinal tract showed both c-kit and CD34-positive cells surrounding the Auerbach ganglia plexus in the gastrointestinal tract. Twenty-seven of 27 tumors strongly expressed c-kit. Fourteen of 27 tumors were positive for CD34. Of the malignant GISTs, 14 of 19 were positive for CD34; of the benign tumors, 0 of 8 were positive for CD34. Thus, CD34 was the best indicator of malignant phenotype. Conclusion.—This is the first description of benign smooth muscle GISTs negative for CD34. The results of this study suggest that GISTs originate from CD34-positive stem cells and differentiate toward pacemaker cell phenotype. The lack of expression of CD34 in the benign GIST may indicate that benign GISTs are composed of more mature ICCs, whereas malignant GISTs are composed of dedifferentiated ICCs that express CD34-positive stem cells.

scholarly journals Clinical Diagnosis of Gastrointestinal Stromal Tumor (GIST): From the Molecular Genetic Point of View

Cancers ◽  
2019 ◽  
Vol 11 (5) ◽  
pp. 679 ◽  
Author(s):  
Chiao-En Wu ◽  
Chin-Yuan Tzen ◽  
Shang-Yu Wang ◽  
Chun-Nan Yeh
Keyword(s):  
Gold Standard ◽  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Molecular Genetic ◽  
Therapeutic Strategies ◽  
Point Of View ◽  
Protein Markers ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal

Gastrointestinal stromal tumors (GISTs) originating from the interstitial cells of Cajal are mesenchymal tumors of the gastrointestinal tract and have been found to harbor c-KIT mutations and KIT (CD117) expression since 1998. Later, PDGFRA mutations, SDH alterations, and other drive mutations were identified in GISTs. In addition, more and more protein markers such as DOG1, PKCθ were found to be expressed in GISTs which might help clinicians diagnose CD117-negative GISTs. Therefore, we plan to comprehensively review the molecular markers and genetics of GISTs and provide clinicians useful information in diagnostic and therapeutic strategies of GISTs. Twenty years after the discovery of KIT in GISTs, the diagnosis of GISTs became much more accurate by using immunohistochemical (IHC) panel (CD117/DOG1) and molecular analysis (KIT/PDGFRA), both of which constitute the gold standard of diagnosis in GISTs. The accurately molecular diagnosis of GISTs guides clinicians to precision medicine and provides optimal treatment for the patients with GISTs. Successful treatment in GISTs prolongs the survival of GIST patients and causes GISTs to become a chronic disease. In the future, the development of effective treatment for GISTs resistant to imatinib/sunitinib/regorafenib and KIT/PDGFRA-WT GISTs will be the challenge for GISTs.

scholarly journals An audit of gastrointestinal stromal tumors from a tertiary medical college hospital in Eastern India

2018 ◽  
Vol 04 (01) ◽  
pp. 006-010 ◽  
Author(s):  
Samrat Dutta ◽  
Bidisha Ghosh ◽  
Rupsha Pal ◽  
Prashant Pandey ◽  
Madhumita Mukhopadhyay ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Gastrointestinal Stromal Tumors ◽  
Progression Free Survival ◽  
Eastern India ◽  
Gi Tract ◽  
Mesenchymal Tumors ◽  
College Hospital ◽  
Stromal Tumors ◽  
Medical College ◽  
Immunohistochemistry Stain

Abstract Introduction: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of GI tract. They are recently recognized as a distinct pathological entity although previously they were grouped with sarcomas of smooth muscle origin, i.e., leiomyomas, leiomyoblastomas, or leiomyosarcomas. However, apart from GI tract, GIST can occur in any smooth muscle-like in the urinary bladder. Proper diagnosis by immunohistochemistry stain CD133 and risk stratification by morphological parameters has been the cornerstone of treatment. Materials and Methods: This study was conducted as a retrospective analysis to audit the number of cases presenting as GIST in the tertiary medical colleges of eastern India and find out the patterns of care with the available modalities of therapy. Results: Out of total 15 cases, the median age of presentation was 45 years; the Male: Female (M: F) ratio was 2:3 and persisting dragging prolonged chronic abdominal pain was present in the majority. Intestinal complications were few (20%). All were treated with imatinib mesylate 400 mg once daily. However, two patients progressed for whom the dose of imatinib was escalated and one patient was metastatic at onset who was later switched over to sorafenib even after disease progression with dose escalation with imatinib. The median follow-up was 17.5 months and the median time to response to imatinib was 3.2 months. The 2-year actuarial overall survival was 79.12%, and progression-free survival was 86.67%. Conclusions: The future directions are to determine appropriate duration of imatinib therapy in adjuvant/neoadjuvant and therapeutic setting.

scholarly journals Laparoscopic intermediate gastrectomy for treatment of non metastatic gastric gastrointestinal stromal tumors located at the posterior wall and lesser curvature

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Tommaso Marcucci ◽  
◽  
Elena Falsetti ◽  
Chiara Genzan ◽  
Lorenzo Pandolfini ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Interstitial Cells Of Cajal ◽  
Posterior Wall ◽  
Gi Tract ◽  
Pacemaker Cells ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Lesser Curvature ◽  
Laparoscopic Techniques ◽  
Cells Of Cajal

Gastrointestinal Stromal Tumors (GISTs) are rare neoplasms but they represent the most common type of mesenchymal tumors found in the Gastrointestinal (GI) tract [1-5]. GISTs arise from the interstitial cells of Cajal, “Pacemaker Cells”, that play a neuromotor role in normal gut motility [3,6]. They account for < 1% of gastrointestinal tumors with an estimated annual incidence of 10 to 20 cases for one million of the general population [1,2]. Treatment has not yet been standardized, but surgical resection remains the mainstay for non-metastatic GISTs [5]. The use of laparoscopic techniques has been widely debated.

scholarly journals Gastric Schwannoma mimicking gastrointestinal stromal tumors: a rare case with review of literature

2020 ◽  
Vol 7 (5) ◽  
pp. 1691
Author(s):  
Nagari Bheerappa ◽  
Digvijoy Sharma ◽  
Gangadhar Rao Gondu ◽  
Nirjhar Raj ◽  
Kamal Kishore Bishnoi ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Gastrointestinal Stromal Tumors ◽  
Malignant Potential ◽  
Review Of Literature ◽  
Mesenchymal Tumors ◽  
Submucosal Tumors ◽  
Stromal Tumors ◽  
Gastric Schwannoma ◽  
Gastric Mass ◽  
Slow Growing

Gastric schwannoma (GS) is a rare neoplasm of the stomach. It accounts for 0.2% of all gastric tumors and is mostly benign, slow-growing, and asymptomatic. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors and up to 60-70% of GIST occur in the stomach. Schwannoma and GIST have similar radiological and endoscopic features making it extremely difficult to differentiate them preoperatively. Differential diagnosis of these two submucosal tumors is important because of the malignant potential of GIST and the relatively benign course of gastric schwannomas. This case stresses on the importance of including gastric schwannomas in the differential diagnosis of a submucosal gastric mass as it has the ability to mimic a gastrointestinal stromal tumor, which is a leading differential diagnosis because of its common occurrence at this site.

scholarly journals KIT exon 11 codon 557/558 deletion/insertion mutations define a subset of gastrointestinal stromal tumors with malignant potential

2008 ◽  
Vol 14 (12) ◽  
pp. 1891 ◽  
Author(s):  
Katerina Kontogianni-Katsarou ◽  
Euthimios Dimitriadis ◽  
Constantina Lariou ◽  
Evi Kairi-Vassilatou ◽  
Nikolaos Pandis ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Malignant Potential ◽  
Stromal Tumors ◽  
Exon 11 ◽  
Insertion Mutations

Imaging Gastrointestinal Stromal Tumors

2005 ◽  
Vol 12 (2) ◽  
pp. 111-115 ◽  
Author(s):  
Marla R. Hersh ◽  
Junsung Choi ◽  
Chris Garrett ◽  
Robert Clark
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Malignant Tumors ◽  
Mesenchymal Tumors ◽  
Small Bowel Tumors ◽  
Stromal Tumors ◽  
Imaging Characteristics ◽  
Positron Emission ◽  
Pet Ct ◽  
Malignant Gists ◽  
Ct And Mri

Background: Because of the recent reclassification of mesenchymal tumors, which was based on a better understanding of the genetics and immunophenotype of gastrointestinal stromal tumors (GISTs), only a limited number of studies have described the radiologic appearance of GISTs. Methods: This study reviews the imaging characteristics of GISTs, with an emphasis on differentiating benign and malignant tumors using positron emission tomography (PET), computed tomography (CT), and magnetic resonance imaging (MRI). We reviewed the data from 53 cases of GISTs treated at our institute. The imaging studies from these cases, which were recorded at our institute from January 1998 through June 2003, included PET, CT, and MRI. Results: Of the 53 GIST cases, stomach and small bowel tumors accounted for 80% of the tumors. Malignant lesions were larger and more heterogeneous, had ulcerations, and were PET positive. Peritoneal and liver metastases were most common. Conclusions: PET, CT, and MRI appear to be useful in differentiating nonmetastasizing from malignant GISTs.

scholarly journals Histomorphological spectrum of gastrointestinal stromal tumors – An institutional experience in a series of seven cases

2020 ◽  
Vol 2 ◽  
pp. 112-116
Author(s):  
Anshul Gupta ◽  
Vijay Suri ◽  
Nikita Goyal ◽  
Sahil Chhabra ◽  
Anamika Garg ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Muscularis Propria ◽  
Gi Tract ◽  
Pacemaker Cells ◽  
Mesenchymal Tumors ◽  
Diagnostic Modality ◽  
Stromal Tumors ◽  
Spindle Cell Lesion ◽  
Institutional Experience ◽  
Risk Categorization

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the abdominal area. They can involve any portion of GI tract, omentum, mesentery, retroperitoneum, and other sites. The tumor is seen to be arising from interstitial cell of Cajal, the pacemaker cells of the GI muscularis propria. About 60% of GISTs occur in stomach. More than 95% of GISTs are positive for immunohistochemical marker Crohn’s disease (CD) CD117. Still in 5% of cases, CD117 expression is not found. Although, in these cases, staining with discovered on GIST-1 (DOG1, also known as ANO1) can be helpful in confirming the diagnosis of GIST histomorphological diagnosis remains the gold standard diagnostic modality with CD 117 positivity leading to more confirmation. Tumor size and mitotic count remain the main factors in the risk categorization of tumors. Furthermore, every spindle cell lesion should undergo CD117 immunohistochemistry markers as an initial workup.

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