Primary gastrointestinal stromal tumor of the liver： A case report

We report a case of primary gastrointestinal stromal tumor （GIST） of the liver. A 17-year-old man with a solid mass in the anterior segment of the right liver was asymptomatic with negative laboratory examinations with the exception of positive HBV. Contrast-enhanced ultrasound （CEUS） revealed a hypervascular lesion in the arterial phase and hypoechoic features during the portal and late phases. However, enhanced spiral computed tomography （CT） showed hypoattenuation in all three phases. Following biopsy, immunohistochemical evaluation demonstrated positive CDl17. Different imaging features of primary GISTs of the liver are due to pathological properties and different working systems between CEUS and enhanced spiral CT.     

Angiographic findings of gastrointestinal stromal tumor

AIM:To discuss the angiographic features of gastrointestinalstromal tumor(GIST)and to evaluate their diagnostic role.METHODS:Twelve patients with pathologically proved GISTunderwent angiography(DSA)1 wk before operation,usingPuck and digital subtraction DSA.The origin,size,morphologyand angiographic appearance of the lesions were reviewed.RESULTS:Two tumors arose from stomach,8 from jejunum,and 2 from ileum.Seven cases were benign and 5 weremalignant.Obviously thickened supplying arteries weredetected in 8 tumors,and early-developed veins were foundin 3.Two types of angiographic changes of GIST were observed.Four cases had twisted irregular neoplastic vessels withpartially coarse and indistinct margins,which were allmalignant.Eight cases had ball-like neoplastic vessels withuniform tumor staining,of which 7 were benign and 1 wasmalignant.CONCLUSION:Angiography facilitates localization anddiagnosis of GIST,helps define their size,range and location,and is especially valuable to patients suffering from melenawith unknown reasons.     

Inflammatory pseudotumor of the liver in association with a gastrointestinal stromal tumor：A case report

Inflammatory pseudotumor of the liver is a rare benign lesion that can mimic a malignant liver nioplasm. A case of inflammatory pseudotumor of the liver found in association with a malignant gastrointestinal stromal tumor (GIST) of the small bowel was reported. The inflammatory pseudotumor was misdiagnosed as a metastasis from the GIST by frozen section. A correct diagnosis was made only after histopathological examination of the paraffin section of the resescted specimen. This case is psrticularly interesting because of the association of the two rare pathological entities and the diagnostic dilemma that arose from the similarity of their histological appearances. To our knowledge,this association has not been reported in the literature.     

Inflammatory pseudotumor of the liver in association with a gastrointestinal stromal tumor: A case report

Inflammatory pseudotumor of the liver is a rare benignlesion that can mimic a malignant liver neoplasm.A caseof inflammatory pseudotumor of the liver found inassociation with a malignant gastrointestinal stromal tumor(GIST)of the small bowel was reported.The inflammatorypseudotumor was misdiagnosed as a metastasis fromthe GIST by frozen section.A correct diagnosis was madeonly after histopathological examination of the paraffinsection of the resected specimen.This case is particularlyinteresting because of the association of the two rarepathological entities and the diagnostic dilemma that arosefrom the similarity of their histological appearances.Toour knowledge,this association has not been reported inthe literature.     

Hepatopancreatoduodenectomy for metastatic duodenal gastrointestinal stromal tumor

BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.     

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.     

Intestinal Kaposi''''s sarcoma may mimic gastrointestinal stromal tumor in HIV infection

Diffuse intestinal Kaposi's sarcoma shares macroscopicand histopathologic features with gastrointestinal stromatumors. Correct diagnosis may pose a clinical challengeWe describe the case of a young HIV-1-infected Africanlady without advanced immunodeficiency, who presentedwith a diffuse spindle cell tumor of the gut. Initiadiagnosis was of a gastrointestinal stromal tumor, basedon endoscopy and histopathology. Further evaluationrevealed evidence for human herpesvirus 8 (HHV8) andthe diagnosis had to be changed to diffuse intestinaKaposi's sarcoma. Antiretroviral triple therapy togethewith chemotherapy was commenced, and has led to therapid remission of intestinal lesions. With a backgroundof HIV infection, the presence of HHV8 as the causativeagent of Kaposi's sarcoma should be determined, asdistinct treatment is indicated.     

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.     

Treatment of patients with advanced gastrointestinal stromal tumor of small bowel： Implications of imatinib mesylate

AIM: To examine the impact of imatinib mesylate (Glivec) on patient survival and response and its safety,and the correlation of the response rate with the kit gene mutation status. METHODS: Thirty-three of 74 (44.6%) small bowel gastrointestinal stromal tumor (GIST) patients who developed recurrence after curative resection and not treated with Glivec were classified as group A patients. Twenty-two advanced small bowel GIST patients treated with Glivec were classified as group B patients. Clinicopathological features, post-recurrence and overall survival rates were compared. Each tumor in group B patients was investigated for mutations of kit or plateletderived growth factor alpha (PDGFRA). The mutation type was correlated with clinical outcomes. The antitumor effect and safety of Glivec in group B patients were also assessed. RESULTS: Advanced small bowel GIST patients treated with Glivec had substantially longer post-recurrence survival and higher overall survival rates than those not treated with Glivec. A total of 15 patients had a partial response (PR) (67.8%). Activated mutations of c-kit were found in 16 of 19 tested patients and no PDGFRA mutant was identified. In 13 patients with GISTs harboring exon 11 kit mutations, the partial response rate (PR) was 69.3%, whereas two of three patients with tumors containing an exon 9 kit mutation had an overall response rate (ORR) of 66.7% (not significant). CONCLUSION: Glivec significantly prolongs the post-recurrence and overall survival of Asian patients with advanced GISTs. Glivec induces a sustained objective response in more than half of Asian patients with advanced small bowel GISTs. Activated mutations of kit exon 11 are detectable in the vast majority of GISTs. There is no difference in the PR rate for patients whose GISTs have kit exon 9 and exon 11 mutations.     

Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen＇s disease

Von Recklinghausen’s disease is an autosomal dominant hereditary disease associated with a wide number of neoplasms. We report a case of a 47-year-old Caucasian male affected by Von Recklinghausen’s disease who developed a malignant somatostatinoma of the papilla major and minor associated with jejunal gastrointestinal stromal tumour with uncertain behaviour. At laparotomy, multiple hepatic metastases were evident. Whipple pancreaticoduodenectomy, jejunal resection, extensive lymphadenectomy and multiple hepatic wedge resections were performed. The patient was alive without recurrence after 24 mo. This is the fourth case reported in the world literature of a patient with Von Recklinghausen’s disease associated with periampullary somatostatinomas and jejunal stromal tumor. In patients with Von Recklinghausen’s disease who complain of gastrointestinal symptoms, a high suspicion index for periampullary endocrine tumours and/or gastrointestinal stromal tumour is required. An aggressive surgical approach seems to give long term survival also in metastatic patients.     

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.     

Coexistence of hepatocellular carcinoma and gastrointestinal stromal tumor： A case report

Malignant gastrointestinal stromal tumors(GIST)are raremesenchymal tumors originating from the wall of thegastrointestinal tract.Their coexistence with other tumorsoriginating from other germ layers is unique.We havereported a case of a 63-year-old GIST patient presentingas an epigastric mass associated with hepatic tumor.Histologically,the mesenteric tumor was composed ofspindle cells showing both neural and smooth muscledifferentiation.Immunohistochemical examinationshowed positive staining for CD117,vimentin,S-100,and SMA,while CD34 antigen was negative.The hepatictumor was diagnosed as hepatocellular carcinoma(HCC).To the best of our knowledge,this is the first case ofGIST and HCC coexistence.The rarity of the case,however,should not lead to ignoring such a possibility indifferential diagnosis.     

Radiofrequency ablation for colorectal cancer liver metastases initially greater than 25 mm but downsized by neo-adjuvant chemotherapy is associated with increased rate of local tumor progression

Background

Radiofrequency ablation (RFA) is a valid treatment for liver metastases from colorectal cancer (CRLM) smaller than 25 mm and unsuitable for surgical resection. Tumor size is predictive for local tumor progression (LTP). The aim of this study was to evaluate whether RFA is indicated for lesions >25 mm at presentation but <25 mm after chemotherapy.

Single HCC smaller than 2 cm: surgery or ablation

Purpose

For hepatocellular carcinoma (HCC), surgical resection and radiofrequency ablation (RFA) are accepted as effective treatments. To clarify the long-term outcome in patients with small HCC, we analyzed data from a nationwide survey of Japan.

Methods

Between 2000 and 2003, a total of 2,550 patients who had undergone resection (n = 1,235) or RFA (n = 1,315) for single small HCC (≤2 cm) were registered to the database of the Liver Cancer Study Group of Japan (LCSGJ).

Results

After a median follow-up period of 37 months, disease-free survival after resection was significantly better than after RFA (1-year, 91 vs. 87%; 2-year, 46 vs. 25%; P = 0.001), but overall survival after resection and RFA were similar (98 vs. 99%; 94 vs. 95%, P = 0.28). In the patients of Child–Pugh class A, disease-free survival was significantly better after resection (n = 1,056) than after RFA (n = 965) (P = 0.001), while overall survival was not significantly different (P = 0.16). In the patients of Child–Pugh class B, both disease-free and overall survival were almost similar (P = 0.63 and P = 0.66) after resection (n = 136) and RFA (n = 303).

Conclusions

For single small HCC (≤2 cm), surgical resection provides better disease-free survival than does RFA. Longer follow-up is needed to regard this indication as conclusive.     

Is exon mutation analysis needed for adjuvant treatment of gastrointestinal stromal tumor?

Gastrointestinal stromal tumors(GISTs) are the most common soft tissue sarcoma of the gastrointestinal tract,resulting from an activating mutation of stem cell factor receptor(KIT),and an activating mutation of the homologous platelet-derived growth factor receptor alpha(PDGFRA) kinase.Most GISTs(90%-95%) are KIT-positive.About 5% of GISTs are truly negative for KIT expression.GISTs have been documented to resistant conventional chemotherapeutics.Due to the KIT activation that occurs in the majority of the cases,KIT inhibition is the primary treatment approach in the adjuvant treatment of metastatic GISTs.Imatinib mesylate is an oral agent that is a selective protein tyrosine kinase inhibitor of the KIT protein tyrosine kinase,and it has demonstrated clinical benefit and objective tumor responses in most GIST patients in phase Ⅱ and Ⅲ trials.The presence and the type of KIT or PDGFRA mutation are predictive of response to imatinib therapy in patients with advanced and metastatic disease.Molecular analysis in phaseⅠ-Ⅱ trials revealed significant differences in objective response,progression-free survival,and overall survival between GISTs with different kinase mutations.The aim of this letter is to touch on the need for exon mutation analysis for adjuvant treatment with imatinib in GIST patients.     

Intestinal Kaposi＇s sarcoma may mimic gastrointestinal stromal tumor in HIV infection

Diffuse intestinal Kaposi's sarcoma shares macroscopic and histopathologic features with gastrointestinal stromal tumors. Correct diagnosis may pose a clinical challenge.We describe the case of a young HIV-1-infected African lady without advanced immunodeficiency, who presented with a diffuse spindle cell tumor of the gut. Initial diagnosis was of a gastrointestinal stromal tumor, based on endoscopy and histopathology. Further evaluation revealed evidence for human herpesvirus 8 (HHV8) and the diagnosis had to be changed to diffuse intestinal Kaposi's sarcoma. Antiretroviral triple therapy together with chemotherapy was commenced, and has led to the rapid remission of intestinal lesions. With a background of HIV infection, the presence of HHV8 as the causative agent of Kaposi's sarcoma should be determined, as distinct treatment is indicated.     

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.     

Direct endoscopic biopsy for subepithelial tumor larger than 20 mm after removal of overlying mucosa

Objective: An accurate diagnosis of a subepithelial tumor (SET) using endoscopic ultrasound (EUS) without tissue acquisition is difficult. Treatment plan for a SET may be influenced by endoscopic tissue diagnosis. We aimed to clarify the clinical outcomes of direct endoscopic biopsy for SET after removal of the overlying mucosa.

Methods: We evaluated the medical records of 15 patients. All patients underwent direct endoscopic biopsy for a SET larger than 20?mm (involving proper muscle layer) after removal of the overlying mucosa. The rate of achieving an accurate diagnosis and the treatment decision after the procedure were evaluated.

Results: The patients’ mean age was 55.1?±?14.7 years. The patient population predominantly comprised men (9/15, 60%). The mean tumor size was 24.3?±?7.8?mm. The mean biopsy number was 3.5?±?1.7. No major complications occurred with the procedure. The mean procedure time was 15?±?7.4?min. An accurate diagnosis was achieved in 93.3% of patients (14/15). The main pathological diagnoses after direct endoscopic SET biopsy were leiomyoma (33.3%, 5/15) and ectopic pancreas (33.3%, 5/15) followed by gastrointestinal stromal tumor (GIST) (13.3%, 2/15) and schwannoma (13.3%, 2/15). The treatment plan was influenced by the result of biopsy in 80% of patients (9/15), and unnecessary surgical resection was avoided.

Conclusions: Direct endoscopic SET biopsy after removal of the overlying mucosa using an endoscopic conventional snare was a useful diagnostic tool with high diagnostic accuracy and low risk of complications.