• Journal of Chest Surgery
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• 제36권8호
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• pp.619-622
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• 2003

위장관 간질종양(Gastrointestinal stromal turners, GIST)은 매우 드문 질환이다. 대개 위나 소장에서 발생하는 것으로 되어 있으나, 식도에서의 생기는 경우는 흔하지 않다. 위장관 간질종양은 위장관에서 발생하며 다양한 형태학적 소견을 보일 수 있으며, 기원이 분명치 않은 종양으로 알려져 왔다 저자들은 66세 여자의 하부 식도에서 발생한 악성 위장관 간질종양 1예를 경험하였기에 문헌 고찰과 함께 보고하고자 한다.

• 대한임상검사과학회지
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• 제43권2호
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• pp.48-56
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• 2011

Gastrointestinal stromal tumor (GIST) is a mesenchymal tumor and is associated with a specific immunophenotype index. It is very important to identify the specific immunophenotype and the diagnosis for the treatment GIST patients. Ninety two cases of GIST analyzed in this study were immuno-stained for c-kit, DOG1, CD34, PKC-${\theta}$, PDGFR-${\alpha}$. The rate of positive staining and statistical significance were then compared. In addition, the GISTs were analyzed as followings: very low risk, low risk, intermediate risk and high risk according to tumor size and nuclear division, and later correlated with clinical parameters. The results of the GIST positive stainings were: DOG1 (95.7%), PKC-${\theta}$ (90.2%), PDGFR-${\alpha}$ (88.0%), c-kit (87.0%) and CD34 (71.7%). Only DOG1 staining showed a statistical significance of p<0.05. It was identified in the classification system of histologic risk that staining expression of DOG1, PKC-${\theta}$, PDGFR-${\alpha}$ were significantly increased as histologic risk increases (p<0.05). However, clinical parameters such as age and sex of patients have no correlations with the classification system of histologic risk (p>0.05). Therefore, in this study, the expression of DOG1 showed statistical significance and DOG1, PKC-${\theta}$, PDGFR-${\alpha}$ staining increased significantly as the histologic risk increases in histologic classification system. Taken together, the DOG1 staining should be very effective for the diagnosis of GIST patients.

• Journal of Gastric Cancer
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• 제8권2호
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• pp.104-109
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• 2008

목적: 국내 위 점막하 종양의 임상병리학적 양상과 치료방법에 대해 파악하고자 전국조사를 실시하였다. 대상 및 방법: 국내 47개 병원에서 2005년부터 2006년까지 위 점막하 종양으로 수술 받은 환자 878명의 자료를 수집하여, 진단명, 증상, 위치, 수술법, 사망률, 종양의 크기, 악성도 등을 조사하였다. 결과: 위 점막하 종양의 수술 후 진단으로는 위장관 간질 종양(Gastrointestinal stromal tumor, GIST)이 586명(66.8%)으로 가장 많았고, 평활근종(Leiomyoma) 97명(11.1%), 신경초종(schwannoma) 70명(8.0%), 이소성 췌장(Ectopic pancreas) 68명(7.8%), 유암종(carcinoid) 16명(1.8%) 순이었다. 환자의 평균 나이는 56.0세, 남녀 비는 1:1.18이었으며, 50.9%의 환자가 증상 없이 발견되었다. 위 상부 1/3에 위치한 위 점막하 종양이 가장 흔했으며(n=449, 52.0%), GIST의 55.4% (n=319), 평활근종의 84.5% (n=82)가 위 상부 1/3에 위치하였다. 복강경적 접근이 전체 환자의 44.2% (n=388)에서 시행되었으며, 수술방법으로는 쐐기 절제술(n=726, 82.8%)이 가장 빈번히 시행되었다. 전체 환자 중 1명(0.1%)만이 수술 후 30일 이내에 사망하였다. GIST 환자 중 115명(21.1%, 115/544)이 고위험도의 악성도를 가졌고, 41명(8.3%, 45/495)에서 수술 후 imatinib mesylate 항암 치료를 하였다. 결론: 조사된 국내 점막하 종양의 약 2/3가 GIST였으며, 약 1/2에서 복강경하 절제술이 이루어졌다. 향후 이 보고가 위점막하 종양의 진료 및 연구의 참고자료로 활용되기를 기대한다.

• Journal of Yeungnam Medical Science
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• 제30권2호
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• pp.105-108
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• 2013

Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous-disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gastrointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeung-nam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomography (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

• 대한기관식도과학회지
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• 제15권1호
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• pp.24-27
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• 2009

Background: Benign tumors of the esophagus are rare. They include leiomyomas, gastrointestinal stromal tumors, neurofibromas and lipomas. In this study we present our experience with enucleation of these 13 tumors for 10 years. Material and Method: A retrospective review of patients who underwent enucleation of benign esophageal tumors between 1995 and 2005 was conducted. Symptom, tumor location and size, operative approach and outcomes after surgery were recorded. Result Thirteen patients were identified(leiomyoma: n=12; GIST n=l). Eight patients were men, five patients were women. Most of them were 4rd and 5th decade. The tumors arose in the lower(7 patients) and middle(6 patients) thirds of the esophagus. Eleven patients underwent a thoracotomy; the remainder were resected using VATS. All of patients underwent simple enucleation. There were no complications and recurrences after surgery. Conclusion: We present our experience with enucleation of these 12 leiomyomas and one GIST for 10 years.

• Journal of Digestive Cancer Research
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• 제1권2호
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• pp.100-103
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• 2013

A 79-year-old man was diagnosed with gastrointestinal stromal tumor (GIST) of the gastric cardia. We performed proximal subtotal gastrectomy and started imatinib therapy as adjuvant treatment after surgery. Whole body skin rash with urticaria was onset on 10 days after imatinib treatment, and the patient decided to stop imatinib because of side effect. After 3 months, PET CT revealed GIST was recurred at spleen and abdominal lymph nodes, abdominal wall. The patient was then restarted on imatinib therapy. On follow-up imaging studies, the tumor almost disappeared, but both pleural effusion and pericardial effusion were found. In this paper, we describe a case of clinical course and side effects in recurred GIST after adjuvant imatinib mesylate treatment.

• Asian Pacific Journal of Cancer Prevention
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• 제14권8호
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• pp.4751-4758
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• 2013

Background: To investigate the predictive and prognostic effects of clinicopathologic and immunohistochemical (IHC) features in patients with gastrointestinal stromal tumours (GISTs). Materials and Methods: Fifty-six patients who were diagnosed with GIST between 2002 and 2012 were retrospectively evaluated. Relationships between clinicopathologic/immunohistochemical factors and prognosis were investigated. Results: Median overall survival (OS) of the whole study group was 74.9 months (42.8-107.1 months), while it was 95.2 months in resectable and 44.7 months in metastatic patients respectively (p=0.007). Epitheliolid tumor morphology was significantly associated with shortened OS as compared to other histologies (p=0.001). SMA(+) tumours were significantly correlated with low (<10/50HPF) mitotic activity (p=0.034). Moreover, SMA(+) patients tended to survive longer and had significantly longer disease-free survival (DFS) times than SMA (-) patients (37.7 months vs 15.9 months; p=0.002). High Ki-67 level (${\geq}30%$) was significantly associated with shorter OS (34 vs 95.2 months; 95%CI; p=0.001). CD34 (-) tumours were significantly associated with low proliferative tumours (Ki-67<%10) (p=0.026). Median PFS (progression-free survival) of the patients who received imatinib was 36 months (27.7-44.2 months). CD34 (-) patients had significantly longer PFS times than that of negative tumours; (50.8 vs 29.8 months; p=0.045). S100 and desmin expression did not play any role in predicting the prognosis of GISTs. Multivariate analysis demonstrated that ${\geq}10/50HPF$ mitotic activity/HPF was the only independent factor for risk of death in GIST patients. Conclusions: Despite the negative prognostic and predictive effect of high Ki-67 and CD34 expression, mitotic activity remains the strongest prognostic factor in GIST patients. SMA positivity seems to affect GIST prognosis positively. However, large-scale, multicenter studies are required to provide supportive data for these findings.

• Journal of Gastric Cancer
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• 제4권4호
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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.

• 대한위암학회:학술대회논문집
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• 대한위암학회 2004년도 제18회 추계학술대회
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• pp.41-41
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• 2004

• Journal of Gastric Cancer
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• 제18권1호
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• pp.99-107
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• 2018

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration. She underwent a completion total gastrectomy with splenectomy for the gastric SET and ITP. The pathology showed storiform fibrosis, and IgG4 was positive in immunohistochemistry (IHC) stain. IgG4-RD is known as a medical disease that could be treated with oral steroids. The difficulty in preoperative diagnosis of the disease occasionally causes unnecessary gastric resection. Thus, preoperative diagnostic methods for IgG4-RD such as deep biopsy with IHC stain or magnetic resonance imaging are needed.