• Title/Summary/Keyword: GISTs

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Clinical Evaluation of Gastrointestinal Stromal Tumor of Stomach (위에서 기원한 위장관 간질성 종양의 임상적 고찰)

  • Min, Byung-Wook;Ryu, Keun-Won;Kim, Seung-Joo;Mok, Young-Jae;Kim, Chong-Suk
    • Journal of Gastric Cancer
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    • v.1 no.1
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    • pp.50-54
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    • 2001
  • Purpose: The aim of this study was to analyze the outcomes of patients with gastrointestinal stromal tumors(GISTs) of the stomach who were treated in our hospital. Materials and Methods: We retrospectively studied 31 patients who were treated for primary gastrointestinal stromal tumors of the stomach from 1990 to 1999 at Korea University Guro Hospital. Clinical characteristics, including age, sex and tumor size were analyzed. In addition, the relation between the 5-year survival rate and tumor size, operative procedure, and malignancy were analyzed to identify the factors that predict survival. Results: The malignant GISTs were 11 cases, borderline GISTs were 2 cases, and benign GISTs were 18 cases. The overall 5-year cumulative survival rate of the patients was $84.6\%$, and the 5-year survival rates according to malignancy were $100\%$ for benign and borderline GISTs and $78.1\%$ for malignant GISTs, p=0.1119. The 5-year survival rates according to tumor size were $100\%$ for tumor sizes smaller than 5 cm and $78.4\%$ for tumor sizes larger than 5 cm, p=0.0453. The 5-year survival rate according to lymph node dissection during operative procedure of malignant GISTs was not significant statistically. Conclusions: GISTs of the stomach are infrequently encountered tumors. Tumor size was the most important factor for predicting survival in a clinical situation, and performing a complete resection of the tumor, especially tumors larger than 5 cm, will improve the outcome of treatment.

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Gastrointestinal Stromal Tumors: Case Report, Aeromedical Assessment of Therapy (위장관기질종양)

  • Jeon, Jong Deuk
    • Korean journal of aerospace and environmental medicine
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    • v.30 no.2
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    • pp.80-82
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    • 2020
  • Gastrointestinal Stromal Tumors (GISTs) are relatively uncommon soft tissue sarcomas that can be located in any part of the digestive system. GISTs originate in specialized nerve cells located in the walls of the digestive system. This case report is about a 53-year-old airman who was recently diagnosed as peritoneal GISTs. He got a surgical removal of the tumor and chemotherapy, including imatinib (Gleevec®). Although his GISTs have shown excellent clinical progress, he still needs ongoing treatment. This case involves an airline pilot applicant for Class-I medical certification who has had GISTs under chemotherapy.

Malignant Gastrointestinal Stromal Tumor of the Esophagus - One case report - (식도에 발생한 악성 위장관 간질종양 -1예 보고-)

  • 김경화;김민호;구자홍
    • Journal of Chest Surgery
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    • v.36 no.8
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    • pp.619-622
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    • 2003
  • Gastrointestinal stromal tumors (GISTS) are rare, but potentially aggressive tumors. GISTS are generally found in the stomach or small intestine and less commonly in the colon, rectum, or an intra-abdominal sites but have rarely been documented in the esophagus. GISTS were definded as the most common mesenchymal tumors of the gastrointestinal tract for which there is incomplete understanding of their lineage, while their relationship with differenciated. We reported a very rare case of GISTS of lower esophagus in a 60-year-old woman with relevant literature review.

C-Kit-Negative Gastrointestinal Stromal Tumor in the Stomach

  • Seo, Ho Seok;Hyeon, Ji Yeon;Shin, Ok-Ran;Lee, Han Hong
    • Journal of Gastric Cancer
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    • v.15 no.4
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    • pp.290-294
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    • 2015
  • C-kit-negative gastrointestinal stromal tumors (GISTs) are uncommon, and there have been few reports about the diagnosis and treatment of c-kit-negative GISTs in the stomach. We report the case of a patient who was diagnosed with a huge and atypical GIST in the stomach. The GIST was completely resected and finally diagnosed as c-kit-negative GIST based on immunohistochemical staining of tumor cells, which were negative for CD117 and CD34 and positive for Discovered on GIST-1 (DOG1). C-kit-negative GISTs could be treated by complete resection and/or imatinib, which is the same treatment for c-kit-positive GISTs.

Expression of DOG1, CD117 and PDGFRA in Gastrointestinal Stromal Tumors and Correlations with Clinicopathology

  • Sun, Xiu-Wei;Feng, Zhan-Jun;Huang, Peng;Hao, Wang;Sui, Xing-Ling
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.4
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    • pp.1389-1393
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    • 2012
  • Objective: To discuss the significance of DOG1, CD117 and PDGFRA in the diagnosis of gastrointestinal stromal tumors (GISTs), and analyze their correlations with clinicopathological features and risk ranking. Method: DOG1, CD117 and PDGFRA were detected with IHC Envision ldpe-g-nvp in 63 GISTs and 43 cases of non-GISTs, and analyzed for relations with clinicopathological factors (gender, age, location, tumor size, mitotic phase, histology) and risk degree. Results: The positive expression rate of DOG1, CD117 and PDGFRA in GISTs was 84.1% (53/63), 90.5% (57/63), 53.2% (33/63), respectively. Among the 6 CD117 negative cases, all were DOG1 positive and 5 were PDGFRA positive. Rates in patients with non-GISTs was 11.6%, 16.3%, 6.98%, respectively. Expression of DOG1 and PDGFRA demonstrated no significant variation with gender, age, position, tumor size, mitotic phase, histology, and risk rank. However, CD117 was related with position and histology (P=0.008 and P=0.045), those in the mesentery having a higher positive rate than those derived from stomach, small intestine, colon and rectum (50.0% vs 94.7%, P=0.008). Furthermore CD117 was also highly expressed in spindle and epithele types. Conclusions: DOG1 had a good sensitivity and specificity as a kind of newly discovered marker, especially for KIT negative GISTs. However, DOG1, CD117 and PDGFRA cannot be used for assessing the rish of patients.

Endoscopic resection of gastric gastrointestinal stromal tumor using clip-and-cut endoscopic full-thickness resection: a single-center, retrospective cohort in Korea

  • Yuri Kim;Ji Yong Ahn;Hwoon-Yong Jung;Seokin Kang;Ho June Song;Kee Don Choi;Do Hoon Kim;Jeong Hoon Lee;Hee Kyong Na;Young Soo Park
    • Clinical Endoscopy
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    • v.57 no.3
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    • pp.350-363
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    • 2024
  • Background/Aims: To overcome the technical limitations of classic endoscopic resection for gastric gastrointestinal stromal tumors (GISTs), various methods have been developed. In this study, we examined the role and feasibility of clip-and-cut procedures (clip-and-cut endoscopic full-thickness resection [cc-EFTR]) for gastric GISTs. Methods: Medical records of 83 patients diagnosed with GISTs after endoscopic resection between 2005 and 2021 were retrospectively reviewed. Moreover, clinical characteristics and outcomes were analyzed. Results: Endoscopic submucosal dissection (ESD) and cc-EFTR were performed in 51 and 32 patients, respectively. The GISTs were detected in the upper third of the stomach for ESD (52.9%) and cc-EFTR (90.6%). Within the cc-EFTR group, a majority of GISTs were located in the deep muscularis propria or serosal layer, accounting for 96.9%, as opposed to those in the ESD group (45.1%). The R0 resection rates were 51.0% and 84.4% in the ESD and cc-EFTR groups, respectively. Seven (8.4%) patients required surgical treatment (six patients underwent ESD and one underwent cc-EFTR,) due to residual tumor (n=5) and post-procedure adverse events (n=2). Patients undergoing R0 or R1 resection did not experience recurrence during a median 14-month follow-up period, except for one patient in the ESD group. Conclusions: cc-EFTR displayed a high R0 resection rate; therefore, it is a safe and effective therapeutic option for small gastric GISTs.

The Role of $^{18}F-fluorodeoxyglucose$ Positron Emission Tomography in Gastrointestinal Stromal Tumors (위장관 간질 종양(Gastrointestinal stromal tumor)에서 $^{18}F-fluorodeoxyglucose$ positron emission tomography의 역할)

  • Yoo, Ie-Ryung
    • Nuclear Medicine and Molecular Imaging
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    • v.42 no.sup1
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    • pp.46-51
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    • 2008
  • Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasm of the gastrointestinal tract, and can be distinguished from the smooth muscle or neural tumors in approximately 95% of patients by expression of the KIT receptor tyrosine kinase (CD117). GISTs are known to have high malignant potential and none can be labeled definitely as benign. However, GISTs are unresponsive to standard sarcoma chemotherapy, and only complete surgical resection provides chance for cure. Although the imaging modality of choice is enhanced CT scan in patients with GIST, FDG PET can reflect the malignant potential of GIST. Clinical management of patients with GISTs has dramatically changed with the introduction of novel therapeutics, such as imatinib mesylate (Glivec). This has created a need to re-evaluate the existing criteria used to assess treatment response. FDG PET as functional imaging modality proved to be significantly more accurate than CT alone when assessing GIST response to imatinib. And, FDG PET and PET ICT have been found to be highly sensitive in detecting early response, and to be useful in predicting long-term response to imatinib in patients with recurrent or metastatic GISTs.

Mutational Analysis of the Epidermal Growth Factor Receptor Gene in Gastrointestinal Stromal Tumors (위장관 간질성 종양의 Epidermal Growth Factor Receptor 유전자 돌연변이 연구)

  • Yoo Nam Jin;Lee Jong Woo;Soung Young Hwa;Jeon Hae Myung;Nam Suk Woo;Kim Su Young;Park Won Sang;Lee Jung Young;Lee Sug Hyung
    • Journal of Gastric Cancer
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    • v.4 no.4
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    • pp.268-271
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    • 2004
  • Purpose: Most gastrointestinal stromal tumors (GISTs) have gain-of-function mutations of the KIT or the platelet-derived growth factor receptor alpha (PDGFRA) genes, but approximately $10\%$ of the GISTs are wild types for both the KIT and the PDGFRA genes. The purpose of this study was to investigate the possibility that epidermal growth factor receptor (EGFR) gene mutation might be responsible for the pathogenesis of GIST. Materials and Methods: We analyzed the EGFR gene in 60 GISTs for the detection of somatic mutations by using the polymerase chain reaction (PCR), the single strand conformation polymorphism (SSCP), and DNA sequencing in exon 18, 19, and 21 encoding the kinase domain. Results: The SSCP analysis revealed no evidence of EGFR mutations in exon 18, 19, and 21 in GISTs. Conclusion: The data indicate that the EGFR gene may not be mutated in human GIST and suggest that therapies targeting the mutated EGFR gene products might not be useful in the treatment of GISTs.

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Role of $^{18}F$-fluoro-2-deoxyglucose Positron Emission Tomography in Gastric GIST: Predicting Malignant Potential Pre-operatively

  • Park, Jeon-Woo;Cho, Chang-Ho;Jeong, Duck-Su;Chae, Hyun-Dong
    • Journal of Gastric Cancer
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    • v.11 no.3
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    • pp.173-179
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    • 2011
  • Purpose: It is difficult to obtain biopsies from gastrointestinal stromal tumors (GISTs) prior to surgery because GISTs are submucoal tumors, despite being the most common nonepithelial neoplasms of the gastrointestinal tract. Unlike anatomic imaging techniques, PET-CT, which is a molecular imaging tool, can be a useful technique for assessing tumor activity and predicting the malignant potential of certain tumors. Thus, we aimed to evaluate the usefulness of PET-CT as a pre-operative prognostic factor for GISTs by analyzing the correlation between the existing post-operative prognostic factors and the maximum SUV uptake (SUVmax) of pre-operative 18F-fluoro-2-deoxyglucose (FDG) PET-CT. Materials and Methods: The study was conducted on 26 patients who were diagnosed with gastric GISTs and underwent surgery after being examined with pre-operative FDG PET-CT. An analysis of the correlation bewteen (i) NIH risk classification and the Ki-67 proliferation index, which are post-operative prognostic factors, and (ii) the SUVmax of PET-CT, which is a pre-operative prognostic factor, was performed. Results: There were significant correlations between (i) SUVmax and (ii) Ki-67 index, tumor size, mitotic count, and NIH risk group (r=0.854, 0.888, 0.791, and 0.756, respectively). The optimal cut-off value for SUVmax was 3.94 between "low-risk malignancy" and "high-risk malignancy" groups. The sensitivity and specificity of SUVmax for predicting the risk of malignancy were 85.7% and 94.7%, respectively. Conclusions: The SUVmax of PET-CT is associated with Ki-67 index, tumor size, mitotic count, and NIH classification. Therefore, it is believed that PET-CT is a relatively safe, non-invasive diagnostic tool for assessing malignant potential pre-operatively.

Genetic Characterization of Molecular Targets in Korean Patients with Gastrointestinal Stromal Tumors

  • Park, Joonhong;Yoo, Han Mo;Sul, Hae Jung;Shin, Soyoung;Lee, Seung Woo;Kim, Jeong Goo
    • Journal of Gastric Cancer
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    • v.20 no.1
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    • pp.29-40
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    • 2020
  • Purpose: Gastrointestinal stromal tumors (GISTs) frequently harbor activating gene mutations in either KIT or platelet-derived growth factor receptor A (PDGFRA) and are highly responsive to several selective tyrosine kinase inhibitors. In this study, a targeted next-generation sequencing (NGS) assay with an Oncomine Focus Assay (OFA) panel was used for the genetic characterization of molecular targets in 30 Korean patients with GIST. Materials and Methods: Using the OFA that enables rapid and simultaneous detection of hotspots, single nucleotide variants (SNVs), insertion and deletions (Indels), copy number variants (CNVs), and gene fusions across 52 genes relevant to solid tumors, targeted NGS was performed using genomic DNA extracted from formalin-fixed and paraffin-embedded samples of 30 GISTs. Results: Forty-three hotspot/other likely pathogenic variants (33 SNVs, 8 Indels, and 2 amplifications) in 16 genes were identified in 26 of the 30 GISTs. KIT variants were most frequent (44%, 19/43), followed by 6 variants in PIK3CA, 3 in PDGFRA, 2 each in JAK1 and EGFR, and 1 each in AKT1, ALK, CCND1, CTNNB1, FGFR3, FGFR4, GNA11, GNAQ, JAK3, MET, and SMO. Based on the mutation types, majority of the variants carried missense mutations (60%, 26/43), followed by 8 frameshifts, 6 nonsense, 1 stop-loss, and 2 amplifications. Conclusions: Our study confirmed the advantage of using targeted NGS with a cancer gene panel to efficiently identify mutations associated with GISTs. These findings may provide a molecular genetic basis for developing new drugs targeting these gene mutations for GIST therapy.