Long-term survival after enucleation of a giant esophageal gastrointestinal stromal tumor

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms of the gastrointestinal tract. Less than 1% occurs in the esophagus. Surgery is the primary treatment for patients with GISTs. We report a 29-year-old male was admitted after the detection of a posterior mediastinal mass during work-up with routine examination. He did not have any disease-related symptoms. The physical examination was unremarkable. Chest computed tomographic scan, the barium esophagogram and endoscopic esophageal ultrasound showed benign neoplasm. The patient was performed an enucleation surgery through the right posterolateral thoracotomy. The pathology revealed a 13.0 cm × 12.0 cm × 5.0 cm mass. The tumor was CD117 (C-kit), PDGFRA and DOG1 positive. These findings were consistent with a GIST of the esophagus. So the diagnosis of GIST of esophagus was confirmed. The pathological diagnosis of low grade of GIST of esophagus was confirmed. The patient has no evidence of recurrence and is in good clinical conditions up-to date, five years after surgery.     

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.     

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.     

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[…     

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.     

Synchronous adenocarcinoma and gastrointestinal stromal tumors in the stomach

AIM:To review the clinicopathological characteristics of concurrent gastrointestinal stromal tumors(GISTs) and gastric adenocarcinoma.METHODS:We retrospectively analyzed eight cases of synchronous adenocarcinoma and GIST in the stomach that had been surgically resected with curative intent between March 2003 and December 2008 in Xinhua hospital and Ruijin hospital.The adenocarcinoma was determined to be the primary tumor based on the histological features.The GIST cells were diffusely and strongly positive for CD34 and CD117.RESULTS:The patients were six men and two women aged 47-80 years(average,68.6 years).GIST was preoperatively detected in only one patient.The average sizes of the gastric adenocarcinomas and GISTs were 6.000 ± 2.6186 cm and 1.825 ± 1.4370 cm,respectively.All GISTs were very low-or low-risk lesions that were detected during evaluation,staging,operation or follow-up for gastric adenocarcinoma.CONCLUSION:We hypothesized that the stomach was influenced by the same unknown carcinogen,resulting in a simultaneous proliferation of different cell lines(epithelial and stromal cell).     

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.     

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.     

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.     

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.     

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.     

Coexistence of gastrointestinal stromal tumors with other neoplasms

Background The purpose of this study was to assess the prevalence of other neoplasms in patients with gastrointestinal stromal tumors (GISTs) and to compare clinical and histopathological data in patients with a GIST and accompanying neoplasms and in patients with GIST only. Methods The analysis encompassed 82 patients with a GIST from among 330 300 patients whose surgical specimens, biopsies, and autopsies were evaluated between January 1989 and June 2006. A subgroup of patients with other types of neoplasms was selected. Results Other neoplasms in patients with a GIST were diagnosed in 22 of the 82 (26.8%) patients. The most common accompanying neoplasms were colorectal (nine cases) and gastric (four cases) adenocarcinoma, as well as pancreatic adenocarcinoma (three cases). There was a tendency toward more common localization of a GIST in the small intestine in patients with other neoplasms than in patients with a GIST alone (P < 0.09). Tumors with very low risk of aggressive behavior were more frequent in patients with a GIST accompanied by other neoplasms than in the other group (P < 0.05). No phenotypic differences in GIST cells were found between the two groups. Conclusions In almost 27% of the study population, GISTs coexisted with other neoplasms. A greater proportion of patients with a GIST localized in the small intestine and/or characterized by a very low risk of aggressive behavior and accompanying other neoplasms, compared with a GIST alone, most likely reflects the fact that in the first group, GISTs tended to be an incidental finding during surgery. The results were affected by patient selection and the type of tissue material available.     

Imatinib mesylate neoadjuvant treatment for rectal malignant gastrointestinal stromal tumor

Surgical treatments including radical resection and local excision remain the main treatment for primary rectal gastrointestinal stromal tumors （GISTs）. However, since patients with high-grade rectal GISTs have a higher risk of tumor recurrence and a shorter life expectancy, neoadjuvant treatment is necessary. In this case report, the efficacy of imatinib mesylate （IM） as a neoadjuvant therapy was assessed in an old man with malignant rectal GIST. The patient received IM preoperative treatment for a short period of one and a half months; at the end of the IM treatment, computed tomography scanning showed a markedly reduced tumor size and cystic changes of the tissue. At that time, a function sphincter-sparing surgery was performed. The histological examination of the resected specimen detected no tumor cells, but residual blood vessels and scattered inflammatory lymphocytes. After surgery, the patient has been followed up without additional IM treatment and remained disease-free for 57 mo. This case indicates that IM neoadjuvant therapy can dramatically improve the prognosis of rectal malignant GIST.     

Rectal GIST Presenting as a Submucosal Calculus

This case report presents an incidental finding of a rectal GIST (gastrointestinal stromal tumor) presenting as a submucosal calculus, not previously reported. A 53-year-old man without a significant medical history presented with abdominal pain in the left lower quadrant, and with constipation. Upon rectal examination, a hard submucosal swelling was palpated 4 cm from the anus, at 3 o'clock, in the left rectum wall. X-ray photos, computerized tomography (CT)-scan and a magnetic resonance imaging (MRI) scan clearly showed a calculus. Excision revealed a turnip-like lesion, 3.1 x 2.3 x 1.8 cm. Analysis showed it was a rectal GIST, a rare mesenchymal tumor of the gastrointestinal tract, which expressed CD117 (or c-kit, a marker of kit-receptor tyrosine kinase) and CD34. Calcification is not a usual clinicopathological feature of GISTs [1-3], and although a number of rectal GISTs have been reported [4-9], we have found no cases so far of rectal GIST presenting as a submucosal calculus.In general, GISTs are rare mesenchymal tumors of the gastrointestinal tract (nerve tissue, smooth muscle). Histology and immunohistochemistry discriminate gastrointestinal stromal tumors from leiomyomas and neurinomas. The most important location is the stomach; the rectal location is rare. Usually, the classic signs of malignancy such as cellular invasion and metastasis are missing. A set of histologic criteria stratifies GIST for risk of malignant behavior such as mitotic activity and tumor size, cellular pleomorphism, developmental stage of the cell and quantity of cytoplasma [7,13]. Tumors with a high mitotic activity and size above 5 cm are considered malignant. Recent pharmacological advances such as tyrosine kinase inhibitors have determined c-kit (i.e., CD117) as the most important marker, amongst others. C-kit positive tumors respond extremely well to chemotherapy with Imatinib (Glivec, Gleevec) [10-12].     

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.     

Long-term survival of a patient after resection of a gastrointestinal stromal tumor arising from the pancreas

BACKGROUND:Gastrointestinal stromal tumors (GISTs) may arise in any part of the gastrointestinal tract;extragastrointestinal locations are extremely rare.Only a few cases of extragastrointestinal stromal tumor arising from the pancreas were reported.None of the reports described a long-term follow-up of the patients.METHOD:This report describes an interesting and unusual case of GIST arising from the pancreas.RESULTS:A 74-year-old female presented with a palpable abdominal mass.CT scan showed a large mass 1...     

Isolated abdominal wound metastasis from a gastrointestinal stromal tumor

BACKGROUND: Gastrointestinal stromal tumors (GIST) frequently recur even after complete resection. The typical pattern of failure from GISTs is both local and distant with hepatic and peritoneal metastases being most common. Isolated abdominal-wall recurrence from GISTs has not been previously described. AIM OF THE STUDY: To report an isolated abdominal-wound recurrence in the absence of widespread disease in a patient with GIST. METHODS: Case report of a GIST and isolated abdominal-wound recurrence after laparoscopic-assisted en bloc resection. RESULTS: Elderly male patient presented with an isolated abdominal wall incisional recurrence 18 mo after gastric resection and adjuvant imatinib mesylate therapy for a high-grade GIST. CONCLUSIONS: Complete resection of gastrointestinal stromal tumors followed by imatinib therapy may alter the extent of recurrence.     

Laparoscopic endoscopic cooperative surgery as a minimally invasive treatment for gastric submucosal tumor

Laparoscopic wedge resection is a useful procedure for treating patients with submucosal tumor (SMT) including gastrointestinal stromal tumor (GIST) of the stomach. However, resection of intragastric-type SMTs can be problematic due to the difficulty in accurately judging the location of endoluminal tumor growth, and often excessive amounts of healthy mucosa are removed; thus, full-thickness local excision using laparoscopic and endoscopic cooperative surgery (LECS) is a promising procedure for these cases. Our experience with LECS has confirmed this procedure to be a safe, feasible, and minimally invasive treatment method for gastric GISTs less than 5 cm in diameter, with outcomes similar to conventional laparoscopic wedge resection. The important advantage of LECS is the reduction in the resected area of the gastric wall compared to that in conventional laparoscopic wedge resection using a linear stapler. Early gastric cancer fits the criteria for endoscopic resection; however, if performing endoscopic submucosal dissection is difficult, the LECS procedure might be a good alternative. In the future, LECS is also likely to be indicated for duodenal tumors, as well as gastric tumors. Furthermore, developments in endoscopic and laparoscopic technology have generated various modified LECS techniques, leading to even less invasive surgery.     

Enucleation for gastrointestinal stromal tumors at the esophagogastric junction：Is this an adequate solution？

The authors discussed the proposal by Coccolini and colleagues to treat gastrointestinal stromal tumors (GISTs) at the esophagogastric junction with enucleation and,if indicated,adjuvant therapy,reducing the risks related to esophageal and gastroesophageal resection.They concluded that,because the prognostic impact of a T1 high-mitotic rate on esophageal GIST is worse than that of a T1 high-mitotic rate on gastric GIST,enucleation may not be an adequate surgery for esophagogastric GISTs with a high mitotic ...     

胃肠间质瘤个体化治疗策略的探讨

胃肠间质瘤（GIST）是最常见的胃肠道间叶源性肿瘤，多见于胃和小肠，GIST多具有恶性潜能，生物学行为可以从良性到高度恶性不等。GIST的预后与肿瘤的发病部位、瘤体大小、核分裂数以及肿瘤是否破裂密切相关，伊马替尼辅助治疗明显延长了GIST患者的生存时间。近年对于GIST患者实施个体化治疗的理念也得到了越来越多的重视，本文就GIST个体化治疗的策略研究进行阐述。