A case of gastrointestinal stromal tumor with liver and bone metastases effectively treated with radiofrequency ablation and imatinib mesylate]

A 58-year-old man was admitted because of perforation of the small intestine by a gastrointestinal stromal tumor (GIST). First, the small intestine including a GIST was resected; and then 2 month later, a part of the liver (S5) conforming to metastatic lesion was surgically removed. Twelve months later, another liver metastases was found, and surgical treatment was recommended; but the patient requested non-surgical therapy, so a radiofrequency ablation (RFA) was successfully performed. After that, recurrence of liver metastasis was not observed, but another metastasis was observed on the fifth lumbar vertebra; so administration of imatinib mesylate was started. 28 months after the initial administration the metastatic liver lesion was still invisible, and the bone metastatic lesion had not grown. The patient is alive with good performance status. This report shows that multi-modality therapy by surgery, RFA and imatinib mesylate was effective for liver and bone metastases of GIST.     

Gastrointestinal stromal tumor of stomach with inguinal ymph nodes metastasis: A case report

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the alimentary tract. To the best of our knowledge, few cases have been reported in the literature about the peripheral lymph node metastasis of GIST. Here we report an unusual case of gastric GIST with inguinal lymph nodes metastasis. After the metastatic lymph nodes were resected, the. Patient started to take imatinib 400 mg/d for 12 mo. There were no signs of tumor recurrence at follow-up after 29 mo.This case suggests that the inguinal lymph nodes can be a potential metastatic site of GIST.     

Gastrointestinal stromal tumor of stomach with inguinal lymph nodes metastasis: A case report

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the alimentary tract. To the best of our knowledge, few cases have been reported in the literature about the peripheral lymph node metastasis of GIST. Here we report an unusual case of gastric GIST with inguinal lymph nodes metastasis. After the metastatic lymph nodes were resected, the. patient started to take imatinib 400 mg/d for 12 mo. There were no signs of tumor recurrence at follow-up after 29 mo. This case suggests that th...     

Urgent surgery for intraperitoneal bleeding from GIST during imatinib therapy

The rupture or bleeding of a gastrointestinal stromal tumor (GIST) is a life-threatening adverse event that can happen during imatinib therapy, but few such cases have been reported in the medical literature. Here, we report a case of intraperitoneal bleeding from GIST during imatinib therapy. A 75-year-old man was diagnosed with a large GIST with liver metastasis and admitted to our hospital for abdominal pain on the 13th day of imatinib therapy. The pain disappeared after 7 days of hospitalization; however, the patient complained of diffuse abdominal pain 5 days after discharge. He presented with muscular guarding, and abdominal-pelvic CT demonstrated dense ascites. The tentative diagnosis was peritoneal hemorrhage from GIST, and urgent laparotomy was performed. During the laparotomy, we noted hemoperitoneum of approximately 500 ml; we resected a bulky metastatic tumor on the greater omentum and a primary tumor on the jejunum. The patient took imatinib (400 mg daily) from the ninth postoperative day and underwent monthly checkups for 9 months after the surgery. When GIST patients complain of sudden and severe abdominal pain during imatinib therapy, bleeding from GIST should be considered as a possible adverse effect of imatinib.     

Surgical treatment for gastric GIST with special reference to liver metastases

BACKGROUND/AIMS: Gastrointestinal stromal tumor (GIST) is recently defined as a tumor expressing c-kit and sometimes showing liver metastasis. We investigated the outcomes of gastric GIST with special attention to the effect of repeated treatment for the patients with liver metastases. METHODOLOGY: Fourteen gastric GISTs were retrospectively reviewed and the significance of the NIH stratification system was examined. RESULTS: Liver metastasis was found in 4 of the 14 patients with gastric GISTs. Three of the 4 patients had metachronous liver metastases and underwent repeated hepatectomies after the initial operation for gastric GIST. The other patient had a synchronous liver metastasis and underwent total gastrectomy and partial resection of the liver as the initial treatment. Two of the 4 patients have received treatments 6 times, including hepatic resection, microwave coagulation therapy (MCT), radiofrequency ablation (RFA), or by imatinib. All 4 patients with hepatic metastases are alive after the first hepatectomy with a mean survival time of 64.8 months with or without imatinib treatment. None classified to very low risk and low risk groups by the NIH system showed liver metastasis, while 43% of the patients classified to the high risk showed liver metastasis. CONCLUSIONS: Repeated surgical and medical treatments for liver metastases from gastric GIST improve survival. Special attention should be paid to be the high risk group categorized by the NIH classification system.     

SLITRK3 expression correlation to gastrointestinal stromal tumor risk rating and prognosis

AIM: To assess the influence of SLIT and NTRK-like family member 3 (SLITRK3) on the prognosis of gastrointestinal stromal tumor (GIST) and determine whether SLITRK3 can help improve current risk stratification systems.METHODS: We hypothesized that SLITRK3 could be used as a prognostic molecular biomarker for GIST. 35 fresh tumor samples and 417 paraffin-embedded specimens from GIST patients were utilized. SLITRK3 mRNA expression in GIST tumor tissue was detected by real-time polymerase chain reaction, and SLITRK3 protein levels were estimated by immunohistochemistry. The correlation of SLITRK3 expression with various tumor clinicopathological characteristics and follow-up data were analyzed.RESULTS: GIST tumors had high expression of SLITRK3 compared with adjacent normal tissues and the expression level gradually increased with risk grade. SLITRK3 protein expression was closely associated with gastrointestinal bleeding, tumor site, tumor size, mitotic index, and National Institutes of Health (NIH) classification. Survival analysis showed that SLITRK3 expression was closely correlated with overall survival and disease-free survival of GIST patients. Multivariate analysis also identified SLITRK3 expression, mitotic index, and NIH stage as significant risk factors of GIST recurrence.CONCLUSION: SLITRK3 expression is a highly significant predictor of GIST recurrence and metastasis. Combinations of SLITRK3 and NIH stage have strong predictive and prognostic value, and are feasible markers for clinical practice in gastrointestinal stromal tumor.     

Effect of sunitinib on metastatic gastrointestinal stromal tumor in patients with neurofibromatosis type 1： A case report

Gastrointestinal stromal tumor (GIST) represents the most common mesenchymal malignancy of the gastrointestinal (GI) tract. In neurofibromatosis (NF), the increased incidence of tumor needs to be considered even in non-symptomatic individuals. Patients with neurofibromatosis NF type 1 have an increased risk of developing GI tumors including rare types such as GIST. We report a case of GIST in a 53-year-old male patient with neurofibromatosis. The patient was diagnosed with NF four years ago and his medical history revealed that he was hospitalized 5 times with a provisional diagnosis of massive lower gastrointestinal bleeding. GIST was diagnosed at explorative laparotomy and the tumor was 21 cm × 13 cm × 7 cm in size. Immunohistochemical examination showed that vimentin, actin and CD117 were positive. Computerized tomography showed peritoneal implants three months later. Imatinib mesylate (600 mg/d) was initiated. However, control computerized tomography revealed liver and omental metastasis. The dosage was elevated to 800 mg/d. Despite high dosage, the progression of the metastatic lesions continued in the liver and omentum. The patient started oral sunitinib malate (Sutent? Pfizer Inc, New York, NY, USA) 50 mg per day for 4 consecutive weeks, followed by 2 wk off per treatment cycle. The metastatic lesions in the liver and omentum were decreased in size after four courses, suggesting that sunitinib is also an effective treatment modality for metastatic GIST in NF patients.     

Gastrointestinal stromal tumor of the rectum with liver metastasis: report of a case

Gastrointestinal stromal tumors (GISTs) have unique immunohistochemical and molecular genetic features that set them apart from leiomyomas, leiomyosarcomas, and schwannomas. Although recurrence of GIST usually tends to develop locally or in the liver, rectal GIST reoccur predominantly at the original site of the tumor. We describe a rare case of rectal GIST with multiple liver metastases. We carried out immunohistochemical staining for p53 protein, proliferating cell nuclear antigen (PCNA), integrins, and interleukin-1 receptor type I (IL-1RI) in order to investigate the degree of malignancy of this neoplasm in addition to the immunohistochemical analyses that were necessary for diagnosing GIST. Histologically, the rectal tumor was classified as an uncommitted type of rectal GIST with multiple liver metastases. Positive immunostaining for PCNA, alpha6 integrin subunit, and IL-1RI was found in both the rectal and hepatic tumors. The patients with a rectal GIST may have an increased risk of liver metastasis and a poor prognosis independent of the size of the tumor.     

Gastrointestinal stromal tumor of the ampulla of Vater:A case report

Gastrointestinal stromal tumors(GISTs)usually develop in the stomach and small intestine and only rarely occur at the ampulla of Vater,with only 11 cases reported in the literature.We report a case of a GIST of the ampulla of Vater.A 36-year-old,previously healthy man presented with a loss of consciousness lasting a few minutes.A gastroduodenal endoscopy revealed a submucosal tumor with central ulceration at the ampulla of Vater.The enhanced computed tomography scan revealed a smooth-outlined hypervascular solid mass(24 mm×30 mm)in the second part of the duodenum.Neither lymphadenopathy nor metastasis was observed.Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed normal bile and pancreatic ducts.Biopsies were collected from the ulcerative lesion,and the tumor was diagnosed as a GIST.A submucosal tumor with central ulceration may be a characteristic form of GISTs of the ampulla of Vater,and biopsy studies are useful for the diagnosing such tumors.The patient underwent pancreatoduodenectomy,and the operative specimen revealed a 2.2-cm GIST with 1 mitosis per 50 highpower fields.The gold standard for treatment of GISTs is surgical resection without rupture of a capsule.If technically possible,local resection may be considered.However,when the location of the lesion presents challenges,a pancreatoduodenectomy should be performed for GIST of the ampulla of Vater.     

A treatment using STI571 for jejunal gastrointestinal stromal tumor (GIST) accompanied with liver metastasis and peritoneal dissemination

A 64-year-old man with a chief complaint of melena visited our emergency outpatient clinic. After several examinations, he was diagnosed as a gastrointestinal stromal tumor (GIST) with liver metastasis. Surgical resection of the jejunal lesion and postoperative adjuvant therapy with STI571 for one year was performed. Due to recent immunohistological studies and introduction of STI571, the diagnosis, treatment, and prognosis of GIST are about to change profoundly. Further accumulation of cases is necessary to investigate the diagnosis, treatment, and prognosis of GIST.     

胃肠间质瘤研究新进展

胃肠间质瘤（GIST）是胃肠问质组织中最常见的恶性肿瘤,可发生于食管到肛门的任何部位,以胃和小肠最多见。e—kit原癌基因和血小板起源生长因子受体基因突变是GIST发生的关键因素。初步诊断主要依靠影像学,可帮助了解肿瘤发生的解剖部位、大小、与周围脏器的关系及有无转移等。确诊依靠病理学和免疫组织化学CD117蛋白过表达。GIST传统的治疗方法是外科切除,但目前主张手术联合分子靶向药物的综合治疗,对一些失去手术时机或复发转移的患者使用靶向药物可获得相对好的预后。近年来,随着分子生物学的发展,GIST的诊断和治疗迅速进步,本文针对其临床特征、免疫组织化学、鉴别诊断和治疗等几方面的最新研究进展作一综述。     

Secondary resistance to imatinib mesylate 70 months after initiation of therapy in a patient with a metastatic gastric gastrointestinal stromal tumor

It is unknown how long the risk of developing secondary resistance to imatinib persists in patients with gastrointestinal stromal tumors (GISTs). Here we report a case of a patient with a metastatic gastric GIST who developed secondary resistance to imatinib 70 months after initiation of imatinib therapy. A 62-year-old woman with a gastric GIST underwent total gastrectomy with pancreaticosplenectomy. Immunohistochemistry revealed a KIT-positive GIST. The mitotic index of the tumor was 13/50 high-power fields, indicating a high-risk malignancy. After surgery, the patient developed a solitary liver metastasis and underwent right hepatic lobectomy. Four months later, a metastatic tumor was found at the left adrenal gland, and imatinib therapy was initiated in December 2004. Imatinib therapy led to marked tumor shrinkage and complete clinical remission in the patient. However, in October 2010, computed tomography scans revealed a peritoneal metastasis in the ileocecal area. The tumor progression was clinically determined to be due to the development of secondary resistance to imatinib, and the patient’s treatment was switched to sunitinib. This case illustrates secondary resistance to imatinib can develop even after a sustained and marked treatment response. Long-term therapy and close monitoring are recommended for the management of patients with metastatic GISTs.     

Concomitant gastric carcinoid and gastrointestinal stromal tumors： A case report

A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor （GIST） is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type 111 gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.     

胃肠道间质瘤110例临床诊治分析

目的 研究胃肠道间质瘤(gastrointestinal stromaltumor,GIST)的临床特点、治疗方法和预后.方法 对2002年2月至2008年7月110例经手术后病理确诊为GIST患者的临床资料进行回顾性分析.结果 所有患者的GIST主要起源于胃(50.9%)和小肠(31.8%),103例(93.6%)有临床症状.最常见的临床表现为消化道出血、腹痛和腹部肿块.手术病理结果发现良性14例(1 2.7%),交界性37例(33.6%),恶性59例(53.6%).免疫组化检测CD117、CD34的阳性率分别为98.2%(108/110)和77.3%(85/110).81.8%的患者进行了根治性的手术切除.15例手术时已转移的GIST和6例复发的GIST行伊马替尼治疗,15例可评估,临床获益率80.0%.结论 GIST临床表现缺乏特异性,确诊须依赖大体标本或免疫组化病理结果,CD117对诊断有重要价值.肿瘤的完整切除是主要治疗方法,分子靶向药物伊马替尼能延长晚期GIST患者的生存期.     

Current clinical management of gastrointestinal stromal tumor

Gastrointestinal stromal tumors(GISTs) are the most common malignant subepithelial lesions(SELs) of the gastrointestinal tract. They originate from the interstitial cells of Cajal located within the muscle layer and are characterized by over-expression of the tyrosine kinase receptor KIT. Pathologically, diagnosis of a GIST relies on morphology and immunohistochemistry [KIT and/or discovered on gastrointestinal stromal tumor 1(DOG1) is generally positive]. The prognosis of this disease is associated with the tumor size and mitotic index. The standard treatment of a GIST without metastasis is surgical resection. A GIST with metastasis is usually only treated by tyrosine kinase inhibitors without radical cure; thus, early diagnosis is the only way to improve its prognosis. However, a GIST is usually detected as a SEL during endoscopy, and many benign and malignant conditions may manifest as SELs. Conventional endoscopic biopsy is difficult for tumors without ulceration. Most SELs have therefore been managed without a histological diagnosis. However, a favorable prognosis of a GIST is associated with early histological diagnosis and R0 resection. Endoscopic ultrasonography(EUS) and EUS-guided fine needle aspiration(EUSFNA) are critical for an accurate diagnosis of SELs. EUSFNA is safe and effective in enabling an early histological diagnosis and adequate treatment. This review outlines the current evidence for the diagnosis and management of GISTs, with an emphasis on early management of small SELs.     

Gastric gastrointestinal stromal tumor with synchronous metastasis to the esophagus: case report and review of the literature

We report a patient with synchronous gastrointestinal stromal tumors (GISTs) of the stomach and esophagus. The gastric GIST was larger than that in the esophagus, and no direct connection between the two lesions was seen microscopically. Based on molecular analysis, both lesions had the same c-kit mutation on exon 11, with deletion of codon 557–558. Analysis of normal gastric tissue showed no mutations. The gastric GIST was the primary lesion, and the esophageal tumor was thought to suggest a hematogenous metastasis. A liver metastasis in S6 appeared 6 months after surgery. Imatinib mesylate has been an effective treatment for liver metastasis at follow-up of 2 years.     

Two cases of gastrointestinal stromal tumor of the stomach with lymph node metastasis

Lymph node metastasis from gastrointestinal stromal tumor (GIST) is quite rare. We report two cases of gastric GIST with nodal metastases and results of their mutation analyses. In the first case (78-year-old male), a mass 4.0 cm in size was located at the gastric cardia. Proximal gastrectomy was performed. In the other case (40-year-old female), the gastric tumor was 2.5 cm in size. Computed tomography scan revealed a hepatic metastasis. Imatinib mesylate was administered as primary treatment, at the patient's preference, but the tumors exhibited no response. Wedge resection of the stomach and partial hepatectomy were performed. In both cases, histological examination revealed that the tumors consisted of spindle cells. In the former case, there was an isolated lymph node metastasis at the right cardia. In the latter, three of 5 sampled nodes adjacent to the tumor were positive. In both cases, immunohistochemical analyses showed that primary and metastatic tumors were diffusely positive for CD117 and CD34 and negative for desmin and S100-protein. In the former case, there was a deletion mutation in CD117 exon 11, the most common genotype in GIST. In the latter, there were no detectable mutations in CD117 or platelet-derived growth factor receptor alpha.     

The clinicopathologic observation, c-KIT gene mutation and clonal status of gastrointestinal stromal tumor in the sacrum

Background  

It is very rare that gastrointestinal stromal tumor (GIST) occurs in the sacrum. Only one case of GIST occuring in the sacral region, with intracranial metastasis, has been reported in the literature. Moreover, only few cases have been published in literature about its clonal origin.     

Prognosis of gastrointestinal stromal tumors

BACKGROUND/AIMS: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors (GIMT) of the gut. The aim of this retrospective study is to correlate the histological risk factors with the survival of our patients operated for GIST. METHODOLOGY: In our department, from 1980 to October 2003, 15 patients were operated for GIST. Their mean age was 58 years old and 8 of them were males; 10 (67%) were localized in the stomach and 5 (33%) in the small bowel. In 7 cases liver metastases were present at laparotomy and 4 of them also had peritoneal diffusion. We performed immunohistochemistry for c-Kit, SMA and S100p. Mitotic index (MI) and size neoplasm were the main pathological criteria for malignity. The patients with c-kit (CD117) positive neoplasms were divided according to NIH Consensus Conference risk class, MI, tumor size, localization, SMA or S100p presence, liver metastasis and peritoneal metastasis to compare the different 5-year survival rates. Survival analysis was performed using Kaplan-Meier method and log-rank test and a p < 0.05 was considered as significant. RESULTS: Global survival rate after 5 years was 40% and the mortality was, in all cases, due to GIST. In our experience gender, age, tumor size, localization and S100p positivity did not play any role in predicting the prognosis of GIST. On the contrary high MI and SMA positivity are significantly associated to a lower survival rate (33% vs. 86% and 39% vs. 100% at 5 years, respectively). Finally patients with metastases at laparotomy have a significantly lower 5-year survival rate (hepatic 29% vs. 100%, hepatic and peritoneal 25% us. 78%). CONCLUSIONS: In our experience high MI and in some cases SMA expression can be considered assessed risk factors. On the other hand, criteria of benign behavior did not completely predict the long-term clinical outcome.     

Clinical,Radiologic and Pathologic Characteristics of the Carney Triad: A Case Report and Literature Review

This report describes a patient with a gastrointestinal stromal tumor (GIST) and upper gastrointestinal hemorrhage as initial presentations of the Carney triad, a synchronous or metachronous condition involving gastric leiomyosarcoma, extra-adrenal paraganglioma and pulmonary chondroma. A 26-year-old woman presented with dizziness and several episodes of melena over 3 days. Physical examination revealed pale conjunctiva but normal hemodynamic status. Gastroendoscopy showed one antral submucosal tumor with evidence of an associated bleeder (the bleeder over the mucosal surface of the gastric tumor). An abdominal computed tomography scan showed an antral submucosal tumor, two secondary lesions over segment II/V of the liver and a retroperitoneal tumor. Surgical removal of the tumor and subsequent recovery were uneventful. The gastric GIST with liver metastasis and retroperitoneal paraganglioma were confirmed by pathologic study. The patient was treated with imatinib mesylate for the GIST with liver metastasis, and continued follow-up treatment at our hospital. An abdominal computed tomography scan at 32 months after surgery showed no change in the liver metastatic lesions and no evidence of local recurrence. Another follow-up visit at 33 months after surgery confirmed the stable condition. Of nearly 100 cases reported in the literature, this case is the first to be reported in Taiwan. This case highlights the possibility of this rare syndrome occurring in young female patients with one of the three components of the triad and the need for further diagnostic studies for early identification of tumors when curative surgery is still possible.