A case of duodenal gastrointestinal stromal tumor treated by pancreas-sparing partial duodenectomy

An 79-year-old man admitted our hospital for abdominal mass. Computed tomography showed a tumor measuring about 10 cm in diameter without any metastasis lesion and any sings of local infiltration. Gastroduodenal endoscopy revealed the presence of a submucosal tumor in the third portion of the duodenum, and biopsy revealed tumor cells stained positive for c-kit. These findings were consistent with a GIST and we performed a partial resection of the duodenum sparing the pancreas. Gastrointestinal stromal tumors (GIST) were mainly located in the stomach and the small intestine. Duodenal localization is rare. Surgical approach for GISTs should basically be a partial resection. However, for GISTs located in the duodenum, the partial resection was sometimes difficult and pancreaticoduodenectomy (PD) may be needed, depending on the tumor size and the location of the tumor close to the papilla Vater. Since GIST grew expansively, rarely involving lymph nodes, PD may be an excessive procedure to treat the disease. For this reason pancreas-sparing partial duodenectomy has been introduced for the treatment of duodenal GIST.     

A case of ruptured gastrointestinal stromal tumor of the duodenum with intraabdominal bleeding

We report a case of ruptured gastrointestinal stromal tumor (GIST) of the duodenum with intraabdominal bleeding. A 50-year-old man was admitted to our hospital because of sudden epigastralgia. Enhanced computed tomography (CT) showed a tumor measuring about 14 cm in diameter on the ventral side of the right kidney and intrapelvic fluid collection. We performed an emergency operation. At laparotomy, the tumor was originated from the 2nd portion of the duodenum without attaching to the retroperitoneum. We resected the tumor radically. Histopathologically, the tumor arose from the proper muscle layer of the duodenum, and was positive for c-kit and negative for CD34 and α-SMA on immunostaining. Then the tumor was diagnosed as GIST of the duodenum. Adjuvant therapy by imatinib 400 mg/day is now being done because this case is thought to be clinically malignant GIST.     

A case of gastric gastrointestinal stromal tumor resected after chemotherapy with imatinib

We report a case of a residual stomach gastrointestinal stromal tumor (GIST) successfully treated with resection. We were able to curatively resect after treatment with imatinib. A 33-year-old man underwent distal gastrectomy for duodenal ulcer perforation at the age of 18. He began to experience back pain, and the diagnosis of mild gastritis was made over observation in near medicine, but he was admitted to our hospital because his symptoms continued for 5 months. Abdominal CT and MRI showed a protruding lesion of approximately 17 cm in the left upper-abdomen. Surgery was performed, but the tumor had directly invaded the surrounding organs. To find the definite diagnosis, a biopsy was performed. The histopathological diagnosis was c-kit-positive GIST. Administration of imatinib 400 mg/day was commenced. After 6 months of treatment, CT revealed a roughly 68% reduction in the tumor's diameter. The radical operation was considered feasible and total gastrectomy was performed. The postoperative course was uneventful. Neoadjuvant therapy with imatinib may become a useful means of conserving improve organ and complete resection rate increase with tumor reduction.     

Three cases of giant rectal gastrointestinal stromal tumor

The frequency of rectal gastrointestinal stromal tumor (GIST) is relatively low. We have experienced three cases of giant rectal GIST. Case 1 was treated with sunitinib after imatinib failed by Stevens-Johnson syndrome as neoadjuvant therapy. Case 2 was treated with imatinib as neoadjuvant therapy. These neoadjuvant therapies had no effect on tumor size. All patients underwent an abdominoperineal resection. The mean major axis was 11 .7 cm. Immnohistochemical staining showed that CD34 and KIT were positive. The term of follow-up is short, but no recurrences have been found in all cases. It has been reported that imatinib as neoadjuvant therapy is useful for radical resection in cases of giant rectal GIST. Furthermore, neoadjuvant therapy seems to be one of the treatment options for locally advanced rectal GIST. However, in cases of GIST patients not responding to imatinib, we should perform a surgical resection immediately.     

A case of gastric gastrointestinal stromal tumor operated after low-dose chemotherapy with imatinib mesylate

A 76-year-old man was admitted to our hospital because of tarry motions. Endoscopic findings showed an ulcer on a large submucosal tumor in the stomach. Abodminal CT scan showed a protruding lesion of approximately 13 cm at the lumen of the gastric body. FDG-PET imaging revealed FDG uptake in the gastric body and abdominal cavity. We diagnosed it as GIST with peritoneal dissemination clinically, and treatment with 300 mg of imatinib mesylate was started in December 2006. The main tumor was reduced(reduction rate of 27%)and FDG-PET imaging revealed a decrease in FDG uptake in the main tumor and all disseminated tumors after 5 months of treatment. However, the drug was discontinued for arthritis(grade 3). Partial gastrectomy with sampling peritoneal nodules was performed in June 2007. The present case suggests that low-dose chemotherapy with imatinib mesylate may be useful as a preoperative therapy for a minimal surgery.     

A gastrointestinal stromal tumor in the stomach: usefulness of computed tomographic volumetry

We report herein the case of a 70-year-old man who was found to have a gastrointestinal stromal tumor (GIST) in the stomach following sigmoid colon resection. Preoperative gastroscopic and barium examinations revealed a submucosal tumor, measuring 10cm, on the upper part of the stomach. Using computed tomography (CT) images (i.e., computed tomographic volumetry) the doubling time of this tumor was calculated, accurately, as 3.3 months, which suggested a high growth rate and malignancy. A laparotomy and partial gastric resection were performed. Histologically, the tumor consisted of spindle-shaped cells with oval nuclei. In immunohistochemical studies, the tumor cells were positive with respect to c-kit, CD34, and vimentin, but negative with respect to smooth muscle actin and S-100 protein. There were 15–16 mitoses per 50 high-power fields (HPFs), and the Ki-67 antigen (MIB-1) index was 25.5% in the most active areas, which also indicated malignancy. The final pathological diagnosis of this tumor was malignant GIST. The patient was found to have hepatic metastasis 27 months after the surgery, and he subsequently received a hepatic subsegmentectomy. To our knowledge, there are very few reports concerning the growth rate of GISTs. Computed tomographic volumetry is useful for the follow-up of small or irregularly shaped gastric submucosal tumors, and for making decisions regarding surgical intervention.     

A case of failed laparoscopic and endoscopic cooperative surgery (LECS) for gastrointestinal stromal tumor of the stomach

Recently, laparoscopic and endoscopic cooperative surgery (LECS) for submucosal gastric tumor has been developed to avoid an excessive surgical resection of gastric wall, which causes a deformity of the stomach and reduced oral intake. We report here a case of failed LECS for gastric submucosal tumor, and discuss the cause of failure. An 89-year-old woman underwent LECS for an intraluminal type submucosal tumor involving the upper and posterior gastric wall. Specifically, two sites of the gastric wall were fixed with subumbilical and left latero-abdominal wall approaches and two trocars with balloons were introduced into the stomach. Endoscopic submucosal resection was performed circumferentially around the tumor. Then, we tried to lift the tumor using an Endo-loop?. However, the amount of lifting was not sufficient for a safe surgical resection because the surgical field was extremely narrow. Therefore, we converted LECS to open surgery. In conclusion, it is important to consider clinical factors such as body size, tumor size and tumor location when planning LECS. Particularly, the intraabdominal suturing technique instead of using a linear stapler is desirable for a tumor involving the posterior wall.     

A patient with metastatic gastrointestinal stromal tumor who responded to STI571

We report a case of gastrointestinal stromal tumor (GIST) with multiple hepatic metastases that responded to tyrosine kinase inhibitor STI571. A 30-year-old woman underwent total gastrectomy on July 10, 1998, with a diagnosis of submucosal tumor of the stomach. Pathological analysis of the primary lesion revealed strong expression of c-kit, and it was diagnosed as GIST. The patient underwent tumor excision due to peritoneal recurrence on May 1, 2000 and November 13, 2000. On August 8, 2001, multiple liver metastases were detected by abdominal CAT scan. Treatment with STI571 at a dose of 400 mg/day for 28 days was initiated on September 14, 2000. CAT scan showed rapid tumor shrinkage after 3 weeks of treatment (reduction rate of 56%) and the response continued after 7 weeks of treatment (reduction rate of 71%). Thus, we evaluated the response as PR. Leukocytopenia, edema, diarrhea and nausea were observed; however, all toxicities were mild and tolerable. This case suggests the efficacy of STI571 for metastatic GIST.     

Hand-assisted laparoscopic surgery for a large gastrointestinal stromal tumor of the stomach

We report two cases of large gastrointestinal stromal tumor (GIST) of the stomach that were successfully treated by hand-assisted laparoscopic surgery (HALS). Two patients, a 56-year-old woman and a 60-year-old man, were admitted to our department for the treatment of a large submucosal tumor of the stomach. After gastrointestinal endoscopy, ultrasonography, computed tomography, and magnetic resonance imaging, we suspected that the masses, measuring 7.0 cm and 8.0 cm in diameter, respectively, were GISTs in the stomach. However, preoperatively, we could not rule out the possibility of malignant neoplasms, because they had been bleeding or gradually growing. Hand-assisted laparoscopic wedge resection was safely performed for the diagnosis and treatment of the submucosal tumor of the stomach. The immunohistochemical diagnosis in both patients was GIST of the stomach with intermediate-grade malignancy. HALS may be a good indication for large GISTs of the stomach that are difficult to diagnose preoperatively, whether they are malignant or benign, because it is safe and minimally invasive, promoting rapid recovery.     

Complete response achieved in a case of gastric gastrointestinal stromal tumor by administration of imatinib mesilate with concurrent relatively high-dose steroid therapy to control side effects

We present a 53-year-old man who underwent proximal gastrectomy for gastric gastrointestinal stromal tumor (GIST) in August 2005. Imatinib mesilate (imatinib) 400 mg/day was started in February 2006 for left adrenal metastasis. Tumor size markedly decreased by April 2006, but medication had to be discontinued due to a pruritic rash. A distal pancreatectomy, splenectomy, and left adrenectomy were performed in August for recurrent adrenal metastases. Imatinib 200 mg/day started postoperatively was discontinued immediately due to fever and pruritus. Local recurrence with peritoneal dissemination was found in February 2007. Imatinib 100 mg/day with prednisolone (PDL) 5 mg/day was discontinued in two days due to pruritic dermatitis. Imatinib 200 mg/day with PDL 40 mg/day was restarted in April due to an increase in tumor size. Treatment continued without obvious side effects, and PDL dosage was tapered to 10 mg/day. The tumor was no longer visible on CT in May 2008, and complete response is being maintained as of August 2009.     

Totally laparoscopic resection for a large gastrointestinal stromal tumor of stomach

A debate is currently ongoing about whether a large gastrointestinal stromal tumor (GIST) should be treated by the laparoscopic approach because of the increased risk of tumor rupture during manipulation of the tumor with laparoscopic instruments and the resultant peritoneal tumor dissemination. Herein, we report a case of a large GIST of the stomach which was successfully treated by the laparoscopic approach. A 57 year old female patient visited our institution complaining of postprandial epigastric discomfort. An esophagogastroduodenoscopy and an abdominal computed tomography scan revealed a 10×8 cm sized submucosal tumor at the greater curvature side of the gastric antrum. The patient underwent laparoscopic distal gastrectomy with intracorporeal Billroth-II reconstruction without any breakage of the tumor. Her postoperative course was uneventful and she was discharged on the 7th postoperative day. Even a large GIST of the stomach can safely be treated by the laparoscopic approach when it is performed with proper techniques by an experienced surgeon.     

A case report of adenocarcinoma of the lung in which a partial response was achieved by carboplatin

A 56-year-old housewife was incidentally discovered to have an abnormal shadow in the right B6 area upon a chest X-ray film being taken. A transcutaneous lung biopsy of the mass revealed adenocarcinoma of the lung (WHO classification). A brain computed tomography (CT) scan demonstrated multiple brain metastasis. Following whole brain irradiation, carboplatin (CBDCA) (450 mg/m2) was administered by intravenous drip infusion on March 7, 1986. After three weeks of initial treatment with CBDCA, the size of the tumor in the primary site was found to have decreased on the chest X-ray film by more than 50%, following which, similar doses of CBDCA were administered twice, and reduced (330 mg/m2) doses three times every three to four weeks. She showed a partial response and, for seven months after the beginning of the CBDCA treatment, no progression was seen on the chest X-ray film. It is suggested that there is a need for further phase II studies of CBDCA against non-small cell lung cancer.     

A case of amelanotic anorectal malignant melanoma mimicking gastrointestinal stromal tumor

A 56-year-old female patient was admitted, complaining of hematochezia. She was preoperatively diagnosed with poorly-differentiated carcinoma of anorectum with metastases in the lung and mesorectal lymph nodes, and underwent abdominoperineal resection of the rectum. The immunohistochemistry of the rectal tumor showed positive for vimentin and c-kit, and negative for AE1/AE3, S-100, a-SMA, LCA and CD34, which was compatible with gastrointestinal stromal tumor (GIST). Regardless of the administration of imatinib mesylate, multiple metastases in the brain, bone, adrenal glands and inguinal lymph node proceeded in a short term. An excisional biopsy of the inguinal lymph node was performed and immunohistochemistry of the specimen showed positive for S-100, melan-A, HMB45 and tyrosinase. Therefore, we concluded that amelanotic anorectal melanoma (AMM) metastasized to the lymph node, and rechecked the immuno histochemistry of the anorectal tumor. The anorectal tumor showed positive for melan-A, HMB45 and tyrosinase, but negative for S-100. As far as we know, there are few reports of AMM with S-100 negative and c-kit positive. In such cases, making a differential diagnosis between AMM and GIST of the anorectal region can be very confusing.     

Duodenal gastrointestinal stromal tumor presenting with acute upper gastrointestinal bleeding treated with segmental resection

Gastrointestinal stromal tumours (GISTs) are considered to derive from the interstitial cells of Cajal or their precursors and are defined by their expression of c-kit protein (CD117) that is positive in 95% percent of cases. These are rare mesenchymatous tumours, while they represent the most common mesenchymal tumours of the alimentary tract. The majority of GISTs develop in the stomach and small intestine and more rarely in the rectum, colon, esophagus and mesentery; only 3-5% of all GISTs are located in the duodenum. The presenting symptoms include early satiation, dysphagia, bloating, abdominal pain and gastrointestinal bleeding, either acute or chronic. Surgery remains the mainstay of treatment for localized, non-metastatic, resectable GISTs. We present a case of duodenal gastrointestinal stromal tumour of the third portion of the duodenum that presented with acute upper gastrointestinal bleeding treated with segmental duodenal resection.     

An extraordinary case in whom gastrointestinal stromal tumor and low-grade malignant lymphoma are seen together in the stomach

Introduction Gastrointestinal system (GIS) is the most common site of involvement of all primary extranodal lymphomas. Gastric lymphoma constitutes 3–6% of all primary stomach malignancies. Stomach is also the commonest site of involvement of gastrointestinal stromal tumors (GIST). We would like to report these rare synchronous tumors in the same patient. Case A 68-year-old male was admitted to the internal medicine clinics with the complaints of abdominal distension. Physical examination was normal. On abdominal computed tomography a 12 × 14 × 22 cm sized giant tumoral mass was detected in left hypochondrium. A total gastrectomy was performed. Two distinct neoplasms were detected; one of which was located in the posterior wall of the stomach with the size of 24 × 16 × 13 cm, and the other one was localized in the fundus of the stomach and its size was 6 × 5 × 2 cm. Pathological evaluation revealed the diagnosis of GIST at the posterior wall and low-grade malignant lymphoma from the mass localized in the fundus of the stomach. Discussion Two primary tumors are not seen so often together in the stomach. Adenocarcinoma and associated tumors including gastric lymphoma (especially MALT lymphoma), carcinoid, leiomyosarcoma and rhabdomyosarcoma constitute most of the reported series. Rarely adenocarcinoma and associated GIST cases were reported. It is important to report concurrent gastric lymphoma and GIST case since it is extremely rare in the English literature.     

Clinical outcome in gastrointestinal stromal tumor patients who interrupted imatinib after achieving stable disease or better response

BACKGROUND: Imatinib has been found to be effective in the treatment of patients with gastrointestinal stromal tumors (GIST). We sought to evaluate the clinical outcome of imatinib interruption in GIST patients who had achieved stable disease (SD) or showed better response to imatinib therapy. METHODS: From July 2001 to December 2004, we prospectively collected clinical data from 62 consecutive patients with advanced GIST, of whom 58 (93.5%) achieved SD or better response to imatinib therapy and were included in this study. Imatinib therapy was interrupted in 14 of the 58 patients (interruption group, INT), after a median time of 11.9 months. Progression-free survival (PFS) after imatinib interruption was calculated and imatinib-refractory PFS and overall survival (OS) were compared between the INT group and the 44 patients who continued imatinib treatment (continuation group, CONT). RESULTS: After a median follow-up of 17.9 months following imatinib interruption, nine patients (64%) had progressive disease (PD) with a median PFS from the date of imatinib interruption of 10.0 months. Median PFS dated from the time of imatinib initiation in the INT group was 21.8 months (95% CI, 17.3-26.3 months), but was not reached in the CONT group (P=0.029). Following imatinib reintroduction in the INT group, 88% of patients achieved disease control. There were no statistically significant differences in imatinib-refractory PFS (P=0.405) and OS (P=0.498) between the groups. CONCLUSION: In GIST patients controlled with imatinib, treatment might be interrupted, at least temporarily, when clinically warranted.     

Hypoxia-inducible factor-1alpha expression and angiogenesis in gastrointestinal stromal tumor of the stomach

Hypoxia inducible factor (HIF)-1 is reported to transactivate expression of vascular endothelial growth factor (VEGF), which is an important angiogenic factor. The aim of this study was to elucidate the clinical significance of HIF-1alpha expression in gastrointestinal stromal tumors (GIST). Specimens obtained from 53 patients who underwent surgical resection for GIST of the stomach were used in this study. Specimens were examined immunohistochemically for HIF-1alpha, VEGF, and Ki-67 expression. Tumor microvessel density (MVD) was determined immunohistochemically with anti-CD31 antibody and was estimated by averaging the counts from three high-power fields in the area showing the greatest neovascularization. HIF-1alpha expression was detected in 17 (32.1%) of 53 lesions and was correlated significantly with tumor size, liver metastasis, VEGF expression, and MVD. Prognosis was significantly poorer in patients with tumors expressing HIF-1alpha than in patients with tumors lacking HIF-1alpha expression. HIF-1alpha may play a role in angiogenesis and tumor progression of GIST through regulation of VEGF.     

Expression of vascular endothelial growth factor and angiogenesis in gastrointestinal stromal tumor of the stomach

Vascular endothelial growth factor (VEGF) is associated with the malignant potential of several types of carcinoma. The aim of this study was to elucidate the clinical significance of VEGF expression in gastrointestinal stromal tumor (GIST). METHODS: Specimens obtained from 53 patients who had underwent surgical resection for GIST of the stomach were used in this study. Specimens were examined immunohistochemically for VEGF expression and Ki-67 expression. Tumor microvessel density (MVD) was determined immunohistochemically with anti-CD31 antibody, and was estimated by averaging the counts from three high-power fields in the area showing the greatest neovascularization. RESULTS: VEGF expression was detected in 14 (26.4%) of the 53 lesions and correlated significantly with tumor size, liver metastasis, Ki-67 labeling index, and MVD. Prognosis was significantly poorer than in patients with tumors expressing VEGF than in patients with tumors lacking VEGF expression. Multiple logistic regression analysis for 10-year survival showed VEGF expression and high mitotic rate to be independent predictor of a poor outcome. CONCLUSIONS: Angiogenesis associated with VEGF may play an important role in the progression of GIST. VEGF expression may serve as an indicator of a poor prognosis.