Successful resection of a giant recurrent gastrointestinal stromal tumour with imatinib mesylate as neoadjuvant therapy

Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the GI tract. Although a great deal of experience has been obtained with imatinib mesylate in patients with unresectable or metastatic GISTs, its role in the neoadjuvant setting is not well established. In this paper, we describe a case of successful resection of a giant recurrent GIST with imatinib mesylate as neoadjuvant therapy.     

Small gastrointestinal stromal tumor concomitant with early gastric cancer： A case report

The term gastrointestinal stromal tumors (GISTs)is defined diagnostically as the main group of mesenchymal tumors with spindle or epithelioid cells arising from the wall of the gastrointestinal tract with immunohistochemical reactivity for CD117 antibody.Previous studies revealed that cells in GISTs express a growth factor receptor with tyrosine kinase activity (termed c-kit), which is the product of the c-kit protooncogene. The most specific and practical diagnostic criteria for GISTs are: immunohistochemically determined c-kit (CD117) expression; mitotic score; and tumor size.A small GIST concomitant with early gastric cancer is rarely encountered clinically. Herein we have reported a case of a 1.1-cm GIST detected by esophagogastroduo denoscopy concomitant with a Ⅱc type of early gastric cancer (signet ring cell type). It was detected during a routine physical health examination. To our knowledge,this is the first report of a small GIST concomitant with a signet ring cell type of early gastric cancer.     

胃肠道间质瘤32例临床病理分析及免疫组化研究

目的：探讨胃肠道间质瘤的临床病理及免疫组化特点和诊断标准。方法：对32例胃肠道间质瘤进行常规病理检查及免疫组化染色。结果：胃肠道间质瘤由梭形细胞和上皮样细胞组成，大多数病例CD117和CD34标记阳性。结论：胃肠道间质瘤是胃肠道常见的非上皮性肿瘤，缺乏定向分化。CD117、CD34标记阳性对胃肠道间质瘤的诊断具有重要的价值。     

Gastrointestinal stromal tumor of the stomach with a giant abscess penetrating the gastric lumen

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. In large GISTs, cystic degeneration, necrosis and focal hemorrhage that occur inside the tumor can result in gastrointestinal bleeding. We describe a case of a 74-year old male with GIST of the stomach accompanied with a giant abscess that penetrated the gastric lumen. The patient experienced undiagnosed fever for two months prior to hospitalization. Gastrointestinal endoscopy, X-ray series and computed tomography of the patient’s abdomen revealed a gastric submucosal tumor in the fornix, with a fistula to the gastric lumen that was inundated with a great deal of pus. The mass was diagnosed as a GIST from biopsy specimens. The patient was treated by endoscopic drainage of the abscess and intravenous administration of antibiotics. Eventually, a partial gastrectomy was performed. He was also administered Imanitib mesylate as adjuvant therapy. He was followed up for 2 years and no metastasis or recurrence was recognized at the follow- up examinations. This is the first report of a patient with clearly diagnosed GIST with endoscopic evidence of an abscess penetrating into the gastric lumen.     

Gastrointestinal stromal tumour of the rectum： Report of a case and review of literature

Gastrointestinal stromal tumour （GIST） is a rare tumour of the gastrointestinal tract which does not generally originate in the rectum. The authors describe a case of a 70-year-old man who underwent an anterior resection of the rectum for a low-risk GIST. The patient was not given adjuvant chemotherapy with imatinib and is still disease-free 30 mo after surgery. The authors conclude that although rectal GIST is extremely uncommon, it should be included in differential diagnosis when a tumour in the rectum is detected. Biopsy of the tumour is essential, since this makes it possible to reach a sure preoperative diagnosis based on the immunohistological features of the CDl17 and CD34. Although complete surgical resection with negative tumour margins is the principal curative procedure for primary and nonmetastatic tumours, further studies are still needed for the determination of the most effective treatment strategy for patients with rectal GIST.     

DOG1 is useful for diagnosis of KIT-negative gastrointestinal stromal tumor of stomach

Approximately 80%-95%of gastrointestinal stromal tumors(GISTs)show positive staining for KIT,while the other 5%-20%show negative staining.If the tumor is negative for KIT,but is positive for CD34,a histological diagnosis is possible.However,if the tumor is negative for KIT,CD34,S-100,and SMA,a definitive diagnosis is often challenging.Recently,Discovered on GIST-1(DOG1)has received considerable attention as a useful molecule for the diagnosis of GIST.DOG1,a membrane channel protein,is known to be overexpressed in GIST.Because the sensitivity and specificity of DOG1 are higher than those of KIT,positive staining for DOG1has been reported,even in KIT-negative GISTs.KITnegative GISTs most commonly arise in the stomach and are mainly characterized by epithelioid features histologically.We describe our experience with a rare case of a KIT-negative GIST of the stomach that was diagnosed by positive immunohistochemical staining for DOG1 in a patient who presented with severe anemia.Our findings suggest that immunohistochemical staining for DOG1,in addition to gene analysis,is useful for the diagnosis of KIT-negative tumors that are suspected to be GISTs.     

Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

Approximately 10% of patients with gastrointestinal stromal tumors （GIST）develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor （PEComa） in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule （0.8 cm in diameter） was found during surgery for symptomatic gastric neoplasm （15 cm in diameter） arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.     

Clinical Study on Gastrointestinal Stromal Tumors (GIST) in Iceland, 1990–2003

This is a whole population-based study on clinical symptoms, surgical treatment, and outcome of GIST. All mesenchymal tumors in the digestive tract diagnosed from 1990 to 2003 were identified. All reports were reviewed, all tumors were stained with antibodies to c-kit, and the diagnosis of GIST was confirmed. Clinical, pathological, treatment, and outcome data were analyzed. The study included 53 patients with GIST. The mean age at diagnosis was 65.8±13.6 years (SD). Tumor distribution included 62% in the upper, 32% in the middle, and 6% in the lower digestive tract. Mean tumor size was 4.9±4.4 cm (SD). Gastrointestinal (GI) bleeding was the main symptom in 53% (20/38) of symptomatic cases; most presented with acute gastrointestinal bleeding. Complete surgical resection was performed in 87% (46/53) of patients. Eight of the 53 tumors (15.1%) metastasized, 7 of which were nongastric. The disease-specific death rate at 5 years was 85%, and 5-year survival after complete resection was 64.1%. We conclude that GISTs are often found incidentally but GI bleeding is the most common presentation. Five-year survival is better than previously reported and gastric GIST seems to be more benign than nongastric. GIST seems to metastasize mainly intra-abdominally. These authors contributed equally to the study.     

Concomitant gastric adenocarcinoma and stromal tumor in a woman with polymyalgia rheumatica

Gastrointestinal stromal tumors （GISTs） are rare neoplasms （1%） of the gastrointestinal tract and to our knowledge only rare cases of synchronous presentation of gastric carcinomas and GISTs are reported in the literature. A 72-year-old female with a simultaneous presentation of gastric adenocarcinoma and GIST is presented. Moreover, due to polymyalgia rheumatica the patient received corticosteroids as treatment for the last 3 years. The concomitant occurrence of these neoplasms may involve common carcinogenic factors and there could be an association with polymyalgia rheumatica either as a paraneoplastic presentation or due to its treatment with corticosteroids.     

Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome

This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. Recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.     

Rare adult gastric duplication cyst mimicking a gastrointestinal stromal tumor

Gastric duplication cyst is a very rare gastrointestinal tract malformation that accounts for 2%-4%of alimentary tract duplications.Most cases are diagnosed within the first year of life,following presentation of abdominal pain,vomiting,and weight loss and clinical discovery of an abdominal palpable mass.This case report describes a very uncommon symptomatic gastric duplication cyst diagnosed for the first time in adulthood.Only a few other case reports of similar condition exist,and all were identified by endosonography.The current case involves a 52-year-old male who presented with a onemonth history of progressive iron deficiency anemia without overt gastrointestinal bleeding.The patient underwent esophagogastroduodenoscopy,which revealed a 2.0 cm pinkish subepithelial lesion,suspected to be a gastrointestinal stromal tumor(GIST)and source of gastrointestinal bleeding.The endosonography showed inhomogeneous hypoechoic lesions with focal anechoic areas arising from a second and third layer of the gastric wall.Differential diagnoses of GIST,neuroendocrine tumor,or pancreatic heterotopia were made.The lesion was removed using an endoscopic submucosal resection technique.Histopathology revealed an erosive gastric mass composed of a complex structure of dilated gastric glands surrounded by fibro-muscular tissue,fibroblasts,and smooth muscle bundles,which led to the diagnosis of gastric duplication.     

Synchronous well differentiated neuroendocrine tumour and gastrointestinal stromal tumour of the stomach: a case report

Background  

Well differentiated neuroendocrine tumours (carcinoids), arising from cells of the diffuse neuroendocrine system, represent the most commonly encountered gastric endocrine tumours. Gastrointestinal stromal tumours (GISTs), which stem from interstitial Cajal cells located within the wall of the gastrointestinal tract and have a characteristic immunoreactivity for CD117 (c-kit protein), account for the majority of gastrointestinal mesenchymal neoplasms. Simultaneous occurrence of a GIST with a well differentiated neuroendocrine tumour in the stomach is very rare.     

Laparoscopic resection of gastric gastrointestinal stromal tumors presenting as left adrenal tumors

Gastrointestinal stromal tumors (GISTs) are rare gastrointestinal malignancies. They are rarely seen near the urinary tract. In a literature review, only one case of GIST presenting as a left adrenal tumor was reported. We report two documented cases of gastric GISTs mimicking left adrenal tumors which were successfully treated with pure laparoscopic adrenalectomy and wedge resection of the stomach by excising the tumor from the stomach with serial firing of endoscopic gastrointestinal staplers. The surgical margins were clear, and the patients recovered smoothly. No adjuvant therapy with imatinib was prescribed. During the surveillance for 9 mo and 44 mo respectively, no tumor recurrence and metastasis were documented. Laparoscopic tumor excision, when adhering to the principles of surgical oncology, seems feasible and the prognosis is favorable for such tumors.     

Development of multiple myeloma in a patient with gastrointestinal stromal tumor treated with imatinib mesylate： A case report

Gastrointestinal stromal tumors （GISTs） are rare tumors, which represent approximately 1% of the neoplasms of the gastrointestinal tract. These tumors rarely give extra-abdominal metastases. However, their clinical outcome is potentially adverse. In some rare cases, coexistance of GISTs with other malignancies has been reported. Here we present a case of a 74-year old male with GIST, which was managed by surgical resection. Fourteen months later, the patient presented with liver metastases and imatinib mesylated was administered. During treatment, the patient reported skeletal pain and plane X-rays revealed osteolytic bone lesions. Further investigation revealed the presence of multiple myeloma. To the best of our knowledge, this is the first report of the co-existence of multiple myeloma （MM） with GIST.     

A gastrointestinal stromal tumor of the duodenum masquerading as a pancreatic head tumor

Gastrointestinal stromal tumor （GIST） represents the most common kind of mesenchymal tumor that arises from the alimentary tract. GIST is currently defined as a gastrointestinal tract mesenchymal tumor showing CD117 （c-kit protein） positivity at immunohistochemistry. Throughout the whole length of the gastrointestinal tract, GIST arises most commonly from the stomach followed by the small intestine, the colorectum, and the esophagus. Only 3%-5% of GISTs occur in the duodenum, and especially, if GIST arises from the C loop of the duodenum, it can be difficult to differentiate from the pancreas head mass because of its anatomical proximity. Here, we report a case of duodenal GIST, which was assessed as a pancreatic head tumor preoperatively.     

Gastrointestinal stromal tumor presenting with prominent calcification

We present a rare case of a gastrointestinal stromal tumor (GIST) in the stomach with prominent calcification at presentation. A 61-year-old woman visited our hospital because of epigastric discomfort. A spherical calcified lesion with a diameter of about 30 mm was incidentally shown in the left upper quadrant on an abdominal X-ray. Computed tomography demonstrated that the tumor was growing from the upper gastric body, with calcification in the peripheral ring area. A laparoscopic partial gastrectomy was performed, and the resected specimen revealed a well-circumscribed tumor with exophytic growth from the gastric muscularis propria. Microscopic examination revealed spindle-shaped tumor cells with calcification and hemorrhage. Additionally, positive immunoreactivity of the tumor to KIT and CD34 and a low mitotic index resulted in the diagnosis of very low risk GIST. There are a few case reports of heavily calcified GIST, although solitary or punctate calcification of primary GIST has been reported in several case series. Dystrophic calcification of necrotic or degenerative tissue is the supposed cause of primary calcified GISTs. In contrast, appearance of calcification after administration of imatinib mesylate, which may be one indicator of disease response, is possibly caused by a different mechanism.     

Unusual combination of paraneoplastic manifestations in a patient with metastatic gastrointestinal stromal tumor (GIST)

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Activating mutations in tyrosine kinase receptors KIT or platelet-derived growth factor receptor alpha (PDGFRA) are the main mechanisms causing the disease. Patients generally present with non-specific symptoms, while a number of tumors are discovered incidentally and may be metastatic at the time of diagnosis. Aggressive GISTs have a defined pattern of metastasis to the liver or throughout the abdomen, or both. Though GISTs rarely present systemic or isolated paraneoplastic reactions, a few cases have been reported in the literature. We present the case of a 54-year-old patient with metastatic GIST at diagnosis and the emergence of paraneoplastic manifestations during follow-up.     

Synchronous occurrence of gastrointestinal stromal tumors and other primary gastrointestinal neoplasms

INTRODUCTION Gastrointestinal stromal tumors (GISTs) are an un-common mesenchymal neoplasm affecting the GI tract. The synchronous occurrence of mesenchymal tumors and other primary gastrointestinal malignancies has been rarely reported in the literature[…     

A case of gastrointestinal stromal tumour of the ampulla of Vater

Gastrointestinal stromal tumour rarely develops in the duodenal ampulla region. We report here a case of gastrointestinal stromal tumour of the ampulla of Vater found in a 44-year-old Japanese man presenting with biliary obstruction. He died of hepatic failure with diffuse liver metastasis. The postmortem examination showed a large Borrman type III-like tumour in the duodenal ampullary region with direct invasion of the pancreas and extrahepatic bile duct as well as metastases to the liver and regional lymph nodes. The duct orifice was located at the centre of the tumour. Microscopically, the tumour consisted of anaplastic spindle cells with high mitotic activity (90 mitoses per 50 high-power fields). Immunohistochemically, the spindle cells were positive for KIT and CD34. The final diagnosis was high-grade malignant gastrointestinal stromal tumour of the ampulla of Vater. Considering the recent advances in the diagnosis and treatment of gastrointestinal stromal tumour, this neoplasm should be included in the differential diagnosis of the tumours appearing in the duodenal ampulla region.     

Malignant extra-gastrointestinal stromal tumor of the pancreas:Report of two cases and review of the literature

Gastrointestinal stromal tumors(GISTs)are mesenchymal tumors that arise from the gastrointestinal tract.In rare cases,these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract,such as the mesentery,omentum and retroperitoneum.However,pancreatic extra-gastrointestinal stromal tumors are extremely rare,with only 14 previous cases reported.A 61-year-old man with no clinical symptoms had a routine check-up,during which an abdominal mass located in the pancreas tail was detected.Abdominal surgery was performed with resection of the pancreas tail and the spleen,and he was diagnosed with lowrisk GISTs.Another 60-year-old man with no clinical symptoms underwent Computed tomography which revealed a well-demarcated tumor,6 cm in diameter,in the head of the pancreas.He was diagnosed with pancreatic GISTs.Here,we describe two rare cases of pancreatic GISTs and review the cases previously reported in the literature.