Duodenal Stromal Tumor: Report of a Case

Gastrointestinal stromal tumors are rare tumors of the gastrointestinal (GI) tract that arise from primitive mesenchymal cells. Gastrointestinal stromal tumors account for approximately 80% all of gastrointestinal mesenchymal tumors. Duodenal stromal tumors (DSTs) manifest with unexplained melena, pain, bleeding, anemia, sometimes a partial duodenal obstruction and, rarely, with obstructive jaundice. If the tumor is successfully treated, its prognosis is usually good because of its non-aggressive nature. If resected, the prognosis is favorable in a majority of cases, and it is much better than in carcinomas of the duodenum. In this article, we report a case of DST originating from the first and second portion of the duodenum. Our patient did not have any problems postoperatively and remained symptom-free at 18 months after surgery.     

Gastrointestinal Stromal Tumor of the Stomach: Report of a Case

We report herein the case of a 70-year-old woman found to have a gastrointestinal stromal tumor (GIST) of the stomach. Preoperative X-ray and endoscopic examination revealed a hemispheric submucosal tumor with central depression in the anterior wall of the gastric fornix. The tumor, which was 3 cm in diameter, was resected by a laparoscopy-assisted procedure. Histologic examination revealed that it was composed of spindle-shaped cells with elongated nuclei, and few mitoses. Most of the tumor cells showed immunoreactivity for vimentin and CD34, but not for α-smooth muscle actin, desmin, or S-100 protein. The PCNA index was 40.5%. Thus, the GIST did not show differentiation toward smooth muscle or neural cells. A gastrectomy was not performed because the small size of the tumor, and the paucity of the mitoses indicated that it was benign. Nevertheless, careful and long-term follow-up is needed to monitor for signs of possible local recurrence or distant metastases. Received: February 9, 2000 / Accepted: September 26, 2000     

Gastrointestinal Stromal Tumor of the Rectum Resected by Laparoscopic Surgery: Report of a Case

A 53-year-old man visited our hospital with the chief complaint of pain on urination. On digital rectal examination, a rigid immobile tumor mass with a smooth surface was palpated on the anterior wall on the right side of the rectum near the anal canal. Computed tomography (CT) and magnetic resonance imaging (MRI) of the pelvis revealed a heterogeneous tumor mass measuring 6.5 cm in diameter, which occupied the cavity of the lesser pelvis. This rectal tumor was diagnosed to be a gastrointestinal stromal tumor (GIST) based on the results of a transrectal needle biopsy. A laparoscopic abdominoperineal resection of the rectum was performed to remove the mass. The intraoperative findings showed an ambiguous boundary between the tumor and the rectum but clear boundaries between the tumor and the peripheral organs, and the use of a laparoscope allowed for a good separation by providing a good visual field. The bleeding volume was approximately 80 ml and the operative time was 320 min. The macroscopic findings of excised specimens of the mass showed the tumor, measuring 6.5 × 5.5 × 5.0 cm, to be growing extrinsically from the anterior wall on the right side of the rectum. A histological examination of the excised specimens revealed at most 5 mitoses per 50 high-power fields (×400). The tumor mass was diagnosed to be a GIST of low-grade malignancy based on these findings. The postoperative course was favorable, and there were no postoperative complications. The patient was discharged on the 8th hospital day. Laparoscopic surgery is a minimally invasive surgical procedure for rectal GIST, which is excellent in terms of esthetics. Laparoscopic surgery is therefore considered to be useful for a resection of the rectum, because the magnifying effect allows surgical maneuvers with a favorable visual field within the pelvis.     

Ectopic Pancreas of the Gastric Antrum Contiguous to a Gastrointestinal Stromal Tumor Manifesting as Upper Gastrointestinal Bleeding: Report of a Case

A 54-year-old woman was referred to us for investigation of recurrent episodes of melena. Gastroduodenal endoscopic examination revealed a hemorrhagic, polypoid tumor, about 3 cm in diameter, in the posterior wall of the gastric antrum, near the greater curvature. The lesion had a smooth surface with ulceration, and was fixed to the sublying planes. The source of the bleeding was the mucosa overlying the tumor. We performed a distal subtotal gastrectomy with Roux-en-Y anastomosis. Histopathological examination of the resected specimen revealed an ectopic pancreas and a gastrointestinal stromal tumor contiguous to the ectopic pancreatic tissue in the gastric antrum. The patient was discharged after an uneventful postoperative course and has not experienced any recurrence of symptoms since.     

Pedunculated Giant Gastrointestinal Stromal Tumor of the Stomach Showing Extragastric Growth: Report of a Case

We report an unusual case of a pedunculated gastrointestinal stromal tumor (GIST) of the stomach with extragastric growth. An 84-year-old woman was referred to our hospital for investigation of anemia. Barium meal examination showed a defect occupying the lesser curvature of the stomach from the middle of the corpus to the fornix and an abdominal computed tomography scan, showed a lesion, 20cm in diameter, touching the surface of the spleen. Laparotomy revealed a tumor located between the liver and spleen. As the tumor appeared to be connected with the posterior wall of the gastric corpus by a pedicle, it was resected with a pedunculated lesion of the stomach. Based on the pathological findings, the tumor was diagnosed as a GIST of uncommitted type, with low-grade malignancy.     

Left Extended Hepatectomy for a Metastatic Gastrointestinal Stromal Tumor After a Disease-Free Interval of 17 Years: Report of a Case

Gastrointestinal stromal tumors (GISTs), although rare, are frequently diagnosed with liver metastasis. These metastatic GISTs are poorly responsive to conventional chemotherapy; however, recent studies report improved survival after complete surgical resection of liver metastases. On the other hand, few reports describe the treatment of delayed liver metastasis after resection of a primary GIST. We report the case of a 55-year-old woman found to have liver metastasis from a GIST after a 17-year disease-free interval. The patient underwent a left extended hepatectomy for a complete resection of the metastatic GIST and is alive and well 30 months later. To our knowledge, this is the longest disease-free interval reported in the literature, and emphasizes the importance of considering late metastasis when evaluating patients with a history of GIST. Thus, surgical resection of delayed liver metastasis from a GIST should be considered as primary therapy.     

Secondary c-kit Mutation in a Recurrent Gastrointestinal Stromal Tumor Under Long-Term Treatment with Imatinib Mesylate: Report of a Case

Gastrointestinal stromal tumors (GISTs) commonly harbor oncogenic mutations of the c-kit receptor gene, which are targets for imatinib mesylate. However, imatinib resistance is an increasing clinical problem. We herein present such a case with a recurrent GIST, in association with the development of a secondary mutation in the c-kit gene. A 67-year-old man, who had a GIST of the stomach with multiple liver metastases, underwent a partial gastrectomy, distal pancreatectomy, and partial hepatectomy. After surgery, he was treated with imatinib. However, during the approximately 4-year treatment period, a recurrence of the GIST in the liver was detected, for which a partial hepatectomy was again performed. The primary GIST constitutively had a deletion mutation in exon 11. In addition, the recurrent hepatic tumor developed a secondary point mutation (Val654Ala) in exon 13, which may be responsible for the imatinib resistance.     

Successful Resection of an Advanced Duodenal Gastrointestinal Stromal Tumor After Down-Staging with Imatinib: Report of a Case

The diagnosis of gastrointestinal stromal tumor (GIST) relies on a combination of the following criteria: anatomic location, typical histopathology, and the presence of CD 117-antigen (the tyrosine kinase receptor, c-kit) or CD 34-antigen. Imatinib mesylate, a specific tyrosine kinase inhibitor, is highly efficient against locally advanced or metastatic GIST. We report a case of unresectable duodenal GIST, which we were able to resect with curative intent after down-staging treatment with a dosage of imatinib 400 mg daily for 8 months. We performed Whipple's procedure combined with en bloc resection of the right kidney and adrenal gland. The patient was recurrence free at his 24-month follow-up examination. Down-staging treatment may be worthwhile in selected patients, but further prospective studies of imatinib in this setting are necessary. We think that imatinib should be continued postoperatively, as the risk of recurrence in these patients may be high.     

Sunitinib Treatment for Multiple Brain Metastases from Jejunal Gastrointestinal Stromal Tumor: Case Report

Gastrointestinal stromal tumors (GISTs) are rare malignant tumors and only a few reported cases of brain metastases can be found. Introduction of a new molecular targeted agent, imatinib mesylate in the last decade has dramatically changed the treatment strategy and prognosis. However, imatinib is usually ineffective for brain metastasis from GISTs. The authors present the case of multiple brain metastases from jejunal GIST. The brain metastasis in the right prefrontal gyrus was detected 20 months after resection of the primary lesion when left hemiparesis began although the patient was on imatinib. Then the patient began taking sunitinib instead of imatinib, and the lesion shrunk and the symptom improved. However, after the dose reduction due to side effects, a new brain metastasis was found and this time, stereotactic radiation was effectively done. Sunitinib is one of the promising receptor tyrosine kinase inhibitors used for metastatic renal cell carcinomas or imatinib-refractory GISTs. Sunitinib is thought to penetrate blood-brain barrier, and recent reports indicate effectiveness to brain metastasis. To the authors'' knowledge, this is the first report of brain metastases from jejunal GIST responding to sunitinib therapy.     

Co-existence of Gastrointestinal Stromal Tumors with Malign Epithelial Tumors: a Report of Two Cases

Aim: To emphasize the importance of a detailed observation for incidental simultaneous tumoral masses during surgery for gastrointestinal stromal tumors (GISTs) at any location in the gastrointestinal system. Case presentations: Case 1: a 39 years old female patient with an esophageal squamous cell carcinoma and a synchronous small intestinal GIST discovered incidentally during esophagectomy.

Case 2: a 73 years old female patient with a gastric GIST and a synchronous colorectal cancer detected incidentally during gastrectomy. In both cases, immunohistochemical examinations of the resected specimens confirmed the coexistences of GISTs and epithelial malignancies.

Conclusion: The coexistences of GISTs with epithelial tumors have been increasing in recent years. In any case of a GIST or gastrointestinal adenocarcinoma, the surgeon should be alert to recognize a possible coexistent tumor with different histological origin.     

Gastrointestinal Stromal Tumor of the Rectum: An Analysis of Seven Cases

Purpose Gastrointestinal stromal tumors (GISTs) rarely originate in the rectum. We investigated the clinicopathologic characteristics of rectal GISTs. Methods We analyzed the medical records of seven patients who underwent surgery for GIST of the rectum between 1998 and 2003. Results There were two men and five women with a median age of 55 years (range, 41–72 years) at the time of diagnosis. The median follow-up period was 23 months (range, 7–75 months). The chief symptoms were hematochezia, constipation, and anal pain. All patients underwent curative resection; in the form of abdominoperineal resection in five patients, transanal excision in one, and Hartmann's operation with prostatectomy in one. The median tumor size was 6.6 cm (range, 1–12 cm). Four patients received adjuvant radiation therapy. Local recurrence developed in two patients; 54 months and 23 months after surgery, respectively. Conclusion The common symptoms of rectal GIST were the same as those of other rectal tumors. Curative surgical resection should be done, but further studies are necessary to investigate better adjuvant treatment strategies for patients with rectal GISTs     

Effect of Imatinib (STI571) on Metastatic Gastrointestinal Stromal Tumors: Report of a Case

The prognosis for advanced gastrointestinal stromal tumor (GIST) is poor. However, recent reports from Europe have described the effects of imatinib against metastatic GIST. We herein report the case of a Japanese patient treated with imatinib for advanced GIST. Imatinib at 400mg daily was given to an adult with multiple liver and peritoneal metastases 17 months after undergoing a GIST resection. The sum of the diameter of all target lesions decreased from 37.7 to 10.9cm at 6 months. Tinnitus (grade 2), which has not been reported elsewhere as an adverse effect, developed at 2 months. However, it did not require any treatment. Other adverse effects, nausea (grade 2) and anemia (grade 2), resolved spontaneously. Our results are consistent with previous reports that show imatinib to be effective for the treatment of metastatic GIST, and also suggest that imatinib at 400mg daily for more than 7 months is well tolerated in Japanese adults.     

Complete Remission of Recurrent Gastrointestinal Stromal Tumors After Treatment with Imatinib: Report of a Case

A 49-year-old man underwent partial resection of the jejunum for an abdominal tumor, which was histologically confirmed to be a gastrointestinal stromal tumor (GIST). Immunohistochemistry revealed that the tumor cells were positive for c-kit, p52, and MIB-1. He underwent resection of a total of 83 recurrent tumors over the next 36 months. A computed tomography (CT) scan done a few months later showed multiple tumor recurrences. The patient was started on imatinib mesylate 400 mg/day, and 3 months later, a CT image showed an increase in tumor size but a decrease in tumor density. Subsequent CT scans showed a marked decrease in tumor size 3 months later and no evidence of tumor recurrence 9 and 12 months after the commencement of imatinib treatment. The patient remains in complete remission 31 months after the start of treatment.     

A Ruptured Gastrointestinal Stromal Tumour of the Transverse Mesocolon: a Case Report

We discuss the long history of a patient still alive with a primary gastrointestinal stromal tumour (GIST) of the transverse mesocolon: initially it presented as an acute complication, namely a haemorrhagic shock caused by rupture of the tumour and it recurred twice locally in the years following primary resection. Each time, a macroscopical-ly complete resection could be achieved. Six years after the resection of the primary tumour, a liver metastasis was discovered, which was removed by radio frequency ablation (RFA). Eight months later, the patient developed a new liver metastasis and a retropancreatic mass deemed unresectable. He is treated with imatinib mesylate (Glyvec®, Novartis, Vilvoorde, Belgium) since and has had a stable disease for 6 months.

Gastrointestinal stromal tumours outside the gastrointestinal tract are rare. Until a few years ago the only possible therapy was surgical resection. Nowadays radio frequency ablation can be a solution for irresectable liver metastases, and imatinib mesylate can bring improvement for advanced and metastasized tumours. Whether this could also be useful as an adjuvant therapy has not yet been investigated, but it could be important knowing that 38% of the tumours recur locally and 15% metastasize.

The most important prognostic factors for gastrointestinal stromal tumours are grading and complete resection.     

Emergency Pancreatoduodenectomy (Whipple Procedure) for Massive Upper Gastrointestinal Bleeding Caused by a Diffuse B-Cell Lymphoma of the Duodenum: Report of a Case

We herein report a rare case of a massive upper gastrointestinal (GI) bleeding, caused by high-grade diffuse B-cell lymphoma of the duodenum, secondary to immunoproliferative small intestinal disease (IPSID) and treated with an emergency partial pancreatoduodenectomy. A 42-year-old man was admitted to our hospital because of hematemesis. Upper GI endoscopy was unrevealing because of the copious bleeding. Initially, the patient underwent conservative treatment, thus resulting in the temporary cessation of the bleeding. Later, the hemorrhage massively relapsed. An urgent abdominal ultrasound raised the suspicion of a large, possibly bleeding, neoplasm of the duodenum, which was finally confirmed by abdominal computed tomography. The patient underwent an emergency laparotomy, during which a partial pancreatoduodenectomy was performed (Whipple procedure). Histologically, the tumor was a high-grade B-cell lymphoma of the duodenum. The nearby small intestinal mucosa was suggestive of IPSID. A massive upper GI hemorrhage from a high-grade B-cell non-Hodgkin lymphoma of the duodenum, which develops secondary to IPSID, is a very rare clinical demonstration of this disease. Our case is one of the few reports in the English literature, for which the Whipple procedure has been performed as a curative treatment.     

Predicting the Antitumor Effects of STI571 by Analysis of c-kit Gene Mutations in Gastrointestinal Stromal Tumors of the Stomach: Report of a Case

We report a case of a large gastrointestinal stromal tumor (GIST), greater than 5 cm in diameter, in the stomach. Microscopically, high levels of mitosis were observed, indicative of a high-grade malignancy. We analyzed the c-kit gene mutations by a replication competent retrovirus assay and DNA sequencing, which revealed a c-kit mutation in exon 11. Liver metastases were detected 7 months after surgery. Patients with an exon 11 mutation of the c-kit gene are reported to have a high response to STI571 (imatinib mesylate, Glivec). Accordingly, a 1-month course of STI571 treatment clearly changed the characterization of the metastatic tumors radiographically. Thus, it may be important to analyze c-kit gene mutations in patients presenting with GISTs to predict the effectiveness of STI571 in suppressing GISTs, especially tumors thought to have malignant potential.     

Transsacral Approach to Resect a Gastrointestinal Stromal Tumor in the Rectum: Report of Two Cases

Gastrointestinal stromal tumors (GISTs) rarely arise in the rectum. Whereas a local resection with negative margins is generally considered adequate for resectable GISTs, a wide resection is usually indicated for rectal lesions because of the technical impossibility of local resection. We report the cases of two patients who underwent resection of a rectal GIST using a transsacral approach. Both patients had an uneventful postoperative course, and no evidence of recurrence has been identified. The transsacral approach appears to be less invasive and should be considered as the treatment of choice for a rectal GIST.     

Laparoscopic Wedge Resection for Gastrointestinal Stromal Tumors of the Stomach: Initial Experience

PURPOSE: Surgery for gastrointestinal stromal tumors (GIST) of the stomach is now frequently performed using a laparoscopic approach. We investigated the feasibility and effectiveness of laparoscopy in the management of GIST of the stomach. METHODS: We reviewed the records of 12 consecutive patients who underwent laparoscopic surgery for GIST between April 2000 and April 2004, and compared their short-term outcomes with those of patients who underwent open surgery. All laparoscopic wedge resections were done using stapling devices and 3-4 trocars, often with the aid of intraoperative gastroscopy. We examined all patients preoperatively using various diagnostic modalities, including endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA). A laparoscopic approach was not indicated if the tumor was located near the cardia or pylorus or if it was >=5 cm in diameter. RESULTS: A specific diagnosis of GIST was obtained preoperatively by EUS-FNA in 10 of the 12 patients. The median diameter of the lesion was 2.7 cm (range, 1.5-4.8 cm). Although intraoperative complications were encountered in two patients, conversion to open surgery was not required, and we were able to perform complete tumor excision with negative surgical margins in all patients. The median operative time was 100 min (range, 65-180 min), similar to that for open surgery. First flatus was passed earlier, and the interval to resuming oral intake was shorter than after open surgery. No major postoperative complications such as leakage developed, and the median postoperative hospital stay was 7 days (range, 5-12 days). All diagnoses made by EUS-FNA were confirmed by immunohisto-pathological evaluation of resected specimens. CONCLUSION: Laparoscopic wedge resection is a feasible treatment option for GISTs of the stomach if the lesion is <5 cm in diameter.     

A Spontaneously Ruptured Gastric Stromal Tumor Presenting as Generalized Peritonitis: Report of a Case

Among the diverse clinical presentations of gastrointestinal stromal tumor (GIST), spontaneous rupture with peritonitis is extremely rare. We report herein the unusual case of a 75-year-old man found to have a spontaneously ruptured gastric stromal tumor after presenting with generalized peritonitis. The patient was brought to the emergency department of our hospital by ambulance, with generalized severe abdominal pain. On examination, his abdomen was extensively distended with generalized severe rebound tenderness. Abdominal computed tomography scan showed a giant mass arising from the anterior gastric wall with an irregular internal low-density area and a small amount of ascites. An emergency laparotomy revealed a ruptured gastric tumor with dissemination of its necrotic tissue throughout the peritoneal cavity. The tumor was excised together with normal gastric tissue around its base. The tumor, which was 15 × 11 × 4.4 cm in size, had a coarse laceration over its well-capsulated smooth serosal surface with massive necrosis and clotted blood inside. Immunohistochemical examination revealed positive reactivity to C-kit protein, which was consistent with the newly introduced diagnostic criteria of GIST. The patient had an uneventful postoperative course and remains well. Received: November 8, 1999 / Accepted: November 20, 2000     

Primary Stromal Tumor of the Omentum: Report of a Case

We report the case of a primary extragastrointestinal stromal tumor (EGIST) found in the omentum of a 65-year-old woman. The resected specimen, which measured 6 cm at its largest point, consisted of an outer solid part and inner uniloculated cysts. Microscopically, the tumor was characterized by interlacing bundles of elongated spindle cells, with the nuclei showing a focal palisading pattern; however, skenoid fibers were not observed anywhere and mitoses were absent. Immunohistochemically, the tumor was negative for smooth-muscle actin, desmin, and S-100 protein, but it was positive for CD117 and CD34. The microscopic features were consistent with a gastrointestinal stromal tumor.