Management of Gastrointestinal Stromal Tumors

2015 ◽  
Author(s):  
César Serrano ◽  
Suzanne George
Keyword(s):  
Differential Diagnosis ◽  
Gastrointestinal Stromal Tumors ◽  
Abdominal Mass ◽  
Growth Factor Receptor ◽  
Driver Mutations ◽  
Gastric Gist ◽  
Genetic Syndromes ◽  
Stromal Tumors ◽  
Successful Model ◽  
Computed Tomographic

Gastrointestinal stromal tumors (GISTs) are the most common tumors of mesenchymal origin in the gastrointestinal tract. These tumors are believed to arise from the interstitial cells of Cajal. The oncogenic activation of KIT or platelet-derived growth factor receptor–α is common to these tumors, and GISTS are considered to be a successful model for rational development of personalized treatments against oncogenic driver mutations in cancer. This review covers the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of GISTs. Figures show KIT primary and secondary mutations, the clinical and molecular progression of GISTs, a contrast-enhanced computed tomographic scan showing a gastric GIST presenting as a huge abdominal mass, a magnetic resonance image showing a rectal GIST at baseline and responding to neoadjuvant imatinib after 8 months of treatment, and hematoxylin-eosin stain of a fusocellular and an epithelioid GIST. Tables list demographics and clinical characteristics, associated genetic syndromes, relevant differential diagnosis, risk stratification systems used in GIST, and the comparative activity of approved regimens for GIST. This review contains 5 highly rendered figures, 5 tables, and 74 references.

Gastrointestinal Stromal Tumors: Actin Expression, a New Prognostic Factor?

2010 ◽  
Vol 76 (11) ◽  
pp. 1244-1250 ◽  
Author(s):  
Jaime Ruiz-Tovar ◽  
María Diez-Tabernilla ◽  
Gada Housari ◽  
Enrique Martinez-Molina ◽  
Alfonso Sanjuanbenito
Keyword(s):  
Prognostic Factors ◽  
Small Bowel ◽  
Gastrointestinal Stromal Tumors ◽  
Abdominal Mass ◽  
Disease Free Survival ◽  
Gastric Gist ◽  
Free Survival ◽  
Stromal Tumors ◽  
Actin Expression ◽  
Disease Free

The aim of this study is to analyze the clinical outcome of gastrointestinal stromal tumors (GISTs) and to determine new prognostic factors. We perform a retrospective study of all the patients diagnosed with GIST in any location and operated on between 2000 and 2008 at our institution. We analyzed 35 patients, 16 males (45.7%) and 19 females (54.3%), with a mean age of 64 ± 13.8 years. The tumors were located in the stomach in 22 patients (62.9%), in the small bowel in 10 (28.6%), and the retroperitoneum in three (8.6%). Referring to gastric GIST, endoscopy revealed an ulceration in the mucosa in five cases, suggesting an epithelial neoplasm. In all these cases, pathology of the biopsy specimen was nonconclusive. Survival rate at 1 and 5 years was 94.3 and 88.6 per cent, respectively. Disease-free survival at 1 and 2 years was 91.4 and 88.6 per cent, respectively. Analyzing prognostic factors, a lower disease-free survival was observed among patients with constitutional syndrome at diagnosis ( P = 0.000), small bowel GIST ( P = 0.037), and tumors not expressing actin ( P = 0.015). A lower global survival was observed among men ( P = 0,036), patients with an abdominal mass ( P = 0.033) or with constitutional syndrome ( P = 0.007) at diagnosis and tumors at a retroperitoneal location ( P = 0.0002). Gastric GIST may be confused with epithelial neoplasms, modifying the surgery. In our patients, masculine gender, constitutional syndrome and abdominal mass at diagnosis, small bowel and retroperitoneal location, and actin negative tumors are bad prognostic factors.

scholarly journals Gastrointestinal Stromal Tumors—A Mini Review

2021 ◽  
Vol 11 (8) ◽  
pp. 694
Author(s):  
Gina Gheorghe ◽  
Nicolae Bacalbasa ◽  
Gabriela Ceobanu ◽  
Madalina Ilie ◽  
Valentin Enache ◽  
...  
Keyword(s):  
Digestive Tract ◽  
Gastrointestinal Stromal Tumors ◽  
Histopathological Examination ◽  
Correct Diagnosis ◽  
Growth Factor Receptor ◽  
Risk Category ◽  
Genetic Syndromes ◽  
Stromal Tumors ◽  
Factor Receptor Alpha ◽  
Potentially Malignant

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They are potentially malignant, and have an unpredictable evolution. The origin of these tumors is in the interstitial cells of Cajal, which are cells that are interposed between the intramural neurons and the smooth muscle cells of the digestive tract. GISTs are characterized by mutations in the gene c-Kit, but also other mutations, such as those of the platelet-derived growth factor receptor alpha. The most common locations of these tumors are the stomach and small intestine, although they can occur at any level of the digestive tract and occasionally in the omentum, mesentery and peritoneum. Most cases of GISTs are sporadic, and about 5% of cases are part of family genetic syndromes. The correct diagnosis of GIST is determined by histopathological examination and immunohistochemistry. According to histopathology, there are three main types of GISTs: spindle cell type, epithelioid type and mixed type. The therapeutic management of GIST includes surgery, endoscopic treatment and chemotherapy. The prognosis of patients with GIST varies depending on a number of factors, such as risk category, GIST stage, treatment applied and recurrence after treatment.

scholarly journals SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib

2020 ◽  
Vol 22 (2) ◽  
pp. 133-136
Author(s):  
Daria A. Filonenko ◽  
Andrey A. Meshcheryakov ◽  
Petr P. Arkhiri ◽  
Maxim P. Nikulin ◽  
Evgeniia S. Kolobanova
Keyword(s):  
Receptor Tyrosine Kinase ◽  
Gastrointestinal Stromal Tumors ◽  
Left Lung ◽  
Growth Factor Receptor ◽  
Gastric Gist ◽  
Stromal Tumors ◽  
Carney Triad ◽  
Duration Of Response ◽  
Radiological Response ◽  
Node Metastases

Succinate dehydrogenase deficient gastrointestinal stromal tumors (dSDH GIST) is a unique group of GISTs with an energy metabolism defect as the key oncogenic mechanism without mutations in the proto-oncogene receptor tyrosine kinase (KIT) and platelet-derived growth factor receptor a (PDGFRA). SDH-deficiency is a result of mutations in SDHA, SDHB, SDHC, SDHD. There are three variants of dSDH GIST: sporadic dSDH GIST, Carney triad or Carney-Stratakis syndrome. dSDH GISTs are characterized by young age, female prevalence, gastric location, multiple tumors, lymph node metastases, indolent behavior and poorly response to imatinib. Despite the literature data, we report the response to imatinib in patient with dSDH GIST. 21 year old female patient presented with incomplete Carney triad (multiply gastric GIST with liver and peritoneal metastases, left lung chondroma). The patient received imatinib with clinical response in a month and radiological response in three months-cystic transformation of primary gastric tumor and liver metastases. The duration of response was 8 months.

scholarly journals Gastric schwannoma: a benign tumor often misdiagnosed as gastrointestinal stromal tumor

2015 ◽  
Vol 5 (3) ◽  
Author(s):  
Apurva S. Shah ◽  
Pravin M. Rathi ◽  
Vaibhav S. Somani ◽  
Astha M. Mulani
Keyword(s):  
Differential Diagnosis ◽  
Gastrointestinal Stromal Tumor ◽  
Gastrointestinal Stromal Tumors ◽  
Rare Case ◽  
Benign Tumor ◽  
Stromal Tumor ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Gastric Schwannoma ◽  
Gastric Mass

Gastric schwannomas are rare mesenchymal tumors that arise from the nerve plexus of gut wall. They present with nonspecific symptoms and are often detected incidentally. Preoperative investigation is not pathognomic and many are therefore misdiagnosed as gastrointestinal stromal tumors. We report a rare case of a 37-year old woman who underwent laparotomy for complex bilateral ovarian cyst with resection of gastric-gastrointestinal stromal tumor preoperatively, but confirmed to have a gastric schwannomas postoperatively. This case underscores the differential diagnosis of submucosal, exophytic gastric mass as schwannoma.

scholarly journals The importance of molecular biology in development, prognosis, treatment and resistance to targeted therapy in gastrointestinal stromal tumors

2011 ◽  
pp. 69-79
Author(s):  
Alessandro Comandone ◽  
Elisa Berno ◽  
Simona Chiadò Cutin ◽  
Antonella Boglione
Keyword(s):  
Tyrosine Kinase ◽  
Gastrointestinal Stromal Tumors ◽  
Kinase Inhibitors ◽  
Growth Factor Receptor ◽  
Neoplastic Transformation ◽  
Response To Therapy ◽  
Mesenchymal Tumors ◽  
Kit Mutation ◽  
Stromal Tumors ◽  
Receive Treatment

Gastrointestinal stromal tumors (GISTs) are the commonest mesenchymal tumors of the gastroenteric tract, and are generally believed to originate from the neoplastic transformation of the interstitial cells of Cajal, the pacemaker structures of the stomach and intestine. Exon and genetic mutations (point/deletions) are fundamental for the development of GISTs: the constitutional characteristic of this neoplasm is the presence of the cell surface Kit receptor. Kit is the product of the proto-oncogene cKit, situated in chromosome 4. Ninety-eight percent of GISTs express mutated isoforms of Kit or of PDGFRA (Platelet growth factor receptor a). Kit mutation is the basic condition for autophosphorylation of tyrosine kinase residues in proteins. Autophosphorylation initiates pathogenetic processes in Cajal cells, toward a neoplastic transformation. Imatinib mesilate and, more recently, sunitinib are tyrosine kinase inhibitors, specific antagonists for Kit and PDGFRA, with good activity against GISTs. Most molecular and clinical data currently available concern imatinib. Exon mutations are strategic as prognostic and as predictive factors. In recent years, much evidence suggests that survival, response to therapy and resistance to imatinib are related to different mutations. In the near future, GIST patients will receive treatment differentiated by expressed Kit and PDGFRA mutations, thus truly individualized therapy.

scholarly journals Mutation status and immunohistochemical correlation of EGFR mutations in gastrointestinal stromal tumors

2021 ◽  
Vol 24 (1) ◽  
pp. 67-72
Author(s):  
H Ozkayalar ◽  
MC Ergoren ◽  
G Tuncel ◽  
S Kurt ◽  
E Cevik ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Kinase Inhibitors ◽  
Treatment Options ◽  
Growth Factor Receptor ◽  
Egfr Mutations ◽  
Stromal Tumors ◽  
Molecular Alterations ◽  
Treatment Regimes ◽  
Research Areas ◽  
Turkish Patients

Abstract Being one of the leading causes of cancer deaths worldwide and their resistance to conventional treatment methods, made gastrointestinal stromal tumors (GISTs) one of the hot topics in medical research areas in the past decade. To investigate molecular alterations underlying the tumor is of great importance to be able to develop new, targeted treatment options. In this study, GIST samples obtained from 40 Turkish patients were analyzed for actionable epidermal growth factor receptor (EGFR) mutations that are related to treatment regimes in non small cell lung cancer (NSCLC) to understand whether EGFR expression is altered in GISTs. Established alterations in EGFR can make the use of tyrosine kinase inhibitors possible, which are currently used in cancer therapy, especially in NSCLC. Our results indicated that EGFR mutations are rare in GISTs. Further research is needed to sequence whole coding regions of the gene to investigate new actionable mutations in EGFR in an increased sample size.

Gain-of-function mutations of platelet-derived growth factor receptor α gene in gastrointestinal stromal tumors

2003 ◽  
Vol 125 (3) ◽  
pp. 660-667 ◽  
Author(s):  
Seiichi Hirota ◽  
Akiko Ohashi ◽  
Toshirou Nishida ◽  
Koji Isozaki ◽  
Kazuo Kinoshita ◽  
...  
Keyword(s):  
Growth Factor ◽  
Gastrointestinal Stromal Tumors ◽  
Growth Factor Receptor ◽  
Platelet Derived Growth Factor ◽  
Gain Of Function ◽  
Stromal Tumors

Comparison of characteristic computed tomographic findings of gastrointestinal and non-gastrointestinal stromal tumors in the small intestine

2019 ◽  
Vol 44 (4) ◽  
pp. 1237-1245 ◽  
Author(s):  
Akitoshi Inoue ◽  
Shinichi Ota ◽  
Shigetaka Sato ◽  
Norihisa Nitta ◽  
Tomoharu Shimizu ◽  
...  
Keyword(s):  
Small Intestine ◽  
Gastrointestinal Stromal Tumors ◽  
Stromal Tumors ◽  
Computed Tomographic

scholarly journals Gastrointestinal Stromal Tumors (GIST): A Prospective Analysis and an Update on Biomarkers and Current Treatment Concepts

2015 ◽  
Vol 7s1 ◽  
pp. BIC.S25045 ◽  
Author(s):  
Anna Koumarianou ◽  
Panagiota Economopoulou ◽  
Panagiotis Katsaounis ◽  
Konstantinos Laschos ◽  
Petroula Arapantoni-Dadioti ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Kinase Inhibitor ◽  
Treatment Guidelines ◽  
Growth Factor Receptor ◽  
Current Treatment ◽  
University Hospital ◽  
Stromal Tumors ◽  
Outpatient Unit ◽  
Factor Receptor Alpha ◽  
Metastatic Setting

Gastrointestinal Stromal tumors (GIST) are the most common sarcomas of the gastrointestinal tract, with transformation typically driven by activating mutations of cKIT and less commonly platelet-derived growth factor receptor alpha (PDGFRA). Successful targeting of tyrosine-protein kinase Kit with imatinib, a tyrosine kinase inhibitor, has had a major impact in the survival of patients with GIST in both the adjuvant and metastatic setting. A recent modification of treatment guidelines for patients with localized, high-risk GIST extended the adjuvant treatment duration from 1 year to 3 years. In this paper, we review the clinical data of patients with GIST treated in the Oncology Outpatient Unit of “Attikon” University Hospital and aim to assess which patients are eligible for prolongation of adjuvant imatinib therapy as currently suggested by treatment recommendations.

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