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.     

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.     

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.     

Patterns of extension of gastrointestinal stromal tumors (GIST) treated with imatinib (Gleevec?) by 18F-FDG PET/CT

Background and aim: currently it is recognized the usefulness of 18F-FDG PET in assessing response to therapy with imatinib (Gleevec?) in the gastrointestinal tract sarcomas (GIST). To facilitate the follow-up of these studies is important to know the patterns of metastatic spread. The aim of this paper is to describe patterns observed in the 18F-FDG PET/CT.Method: retrospective study included 29 patients who underwent 18F-FDG PET/CT after being diagnosed with unresectable or metastatic GIST. In total, 87 PET/CT studies were performed (1-6 controls per patient) with a mean time of follow-up 6-36 months. We analyzed the location of the lesions evidenced in PET, CT and fusion. Images were evaluated visually and semiquantitatively (SUV). In cases in which has been considered necessary, additional images have been undertaken: PET delayed imaging, intravenous contrast CT and inspiratory chest CT.Results: the most common primary site was the stomach (41%), small bowel (35%), and rectum (24%). Significant changes in the location of metastatic disease between pre-treatment and the monitoring were observed, with the appearance of more extra-abdominal disease.Conclusions: individualization of protocol studies and interpretation of PET, CT and fused images were required for evaluation of treatment response to imatinib. Hybrid 18F-FDG PET/CT provides an accurate determination of the extent of GIST. While the most common metastatic site is the liver and peritoneum, in the following cases are common extra-abdominal disease.     

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.     

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.     

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.     

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.     

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.     

Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor： A case report

Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.     

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.     

Liver transplantation for metastatic neuroendocrine tumor： A case report and review of the literature

Neuroendocrine tumors are divided into gastrointestinal carcinoids and pancreatic neuroendocrine tumors. The WHO has updated the classification of these lesions and has abandoned the term "carcinoid". Both types of tumors are divided into functional and non-functional tumors. They are characterized by slow growth and frequent metastasis to the liver and may be limited to the liver for long periods. The therapeutic approach to hepatic metastases should consider the number and distribution of the liver metastases as well as the severity of symptoms related to hormone production and tumor bulk. Surgery is generally considered as the first line therapy. In patients with unresectable liver metastases, alternative treatments are dependent on the type and the growth rate. Initial treatments consist of long acting somatostatin analogs and/or interferon. Streptozocin-based chemotherapy is usually reserved for symptomatic patients with rapidly advancing disease, but generally the therapy is poorly tolerated and its effects are short-lived. Locoregional therapy directed such as hepatic-artery embolization and chemoembolization, radiofrequency thermal ablation and cryosurgery, is often used instead of systemic therapy, if the disease is limited to the liver. However, liver transplantation should be considered in patients with neuroendocrine metastases to the liver that are not accessible to curative or cytoreductive surgery and if medical or locoregional treatment has failed and if there are life threatening hormonal symptoms. We report a case of liver transplantation for metastatic neuroendocrine tumor of unknown primary source and provide a detailed review of the world literature on this controversial topic.     

Collision tumor of the rectum： A case report of metastatic gastric adenocarcinoma plus primary rectal adenocarcinoma

INTRODUCTION The term collision tumor refers to two coexisting, but independent tumors[1]. Malignant neoplasms originating from two or more distinct topographic organs may form a collision tumor. A possible explanation for this is field cancerization, whi…