Gastrointestinal Stromal Tumors ; diagnosis ; epidemiology ; surgery ; Humans ; Stomach ; pathology

GIST is a rare neoplasm, the majority of GISTs are located in the stomach and small intestine. Most GISTs are diagnosed histopathologically after resection because of submucosal location. A 37-year-old female patient presented with a 2-weeks history of generalized weakness, nausea accompanied by intermittent passage of black, tarry stools. Esophagogastroduodenoscopy and ERCP showed a large round mass measuring 5 cm in diameter in the ampulla of Vater with ulcer crack. Endoscopic multiple biopsies from the mass including ulcer base were taken. Light microscopic findings showed spindle-shaped and epitheloid tumor cells having high cellularity and frequent mitotic figures. On immunohistochemical stainings, the tumor cells were positive for CD34 and smooth muscle actin. Based on these preoperative findings, a diagnosis of malignant GIST of the ampulla of Vater was made probably. After operation, immunohistochemical studies revealed positive reaction for c-kit and vimentin, as well as focally reactive for CD34 and smooth muscle actin. We report a case of GIST in the ampulla of Vater presenting with melena that was diagnosed preoperatively and postoperatively.
Adult ; *Ampulla of Vater ; Common Bile Duct Neoplasms/*diagnosis/pathology ; Female ; Gastrointestinal Stromal Tumors/*diagnosis/pathology ; Humans

At the end of 2012, Chinese Society of Clinical Oncology (CSCO) amended and revised the expert consensus of gastrointestinal stromal tumor (GIST) 2011. This article is respectively review hot topics regarding surgical intervention during the revision, including principles and indications of biopsy and surgery.
Biopsy ; Consensus Development Conferences as Topic ; Gastrointestinal Neoplasms ; diagnosis ; pathology ; surgery ; Gastrointestinal Stromal Tumors ; diagnosis ; pathology ; surgery ; Humans

BACKGROUND/AIMS: The incidence of duodenal tumors has increased by health surveillance. However, preoperative diagnosis of subepithelial duodenal tumors remains difficult because of the wide variety of pathologies and the location of the tumors. We analyzed endoscopic, radiological, and pathological features of subepithelial benign duodenal tumors (BDTs), which were treated by surgical resection. METHODS: Five patients with subepithelial BDTs treated by surgical resection were analyzed retrospectively. We compared the preoperative and postoperative diagnosis and evaluated the clinical presentations, endoscopic and radiological findings, surgical treatments, pathological results, and outcomes of these patients. RESULTS: All the patients underwent successful surgical resection. There were two cases of gastrointestinal stromal tumors (GISTs) treated with segmental duodenectomy, one case of carcinoid tumor treated with antrectomy, one case of gangliocytic paraganglioma treated with ampullectomy, and a lipoma removed by mass excision. The two GISTs were in the duodenal third and fourth segment close to the pancreas, and it was difficult to exclude pancreatic tumors by imaging studies. All the patients remained healthy for more than three years. CONCLUSIONS: Subepithelial BDTs are rare and difficult to diagnosis. Awareness and preoperative diagnosis of subepithelial BDTs can lead to minimally invasive treatment, including endoscopic or local surgical resection.
Carcinoid Tumor ; Diagnosis ; Duodenum ; Gastrointestinal Stromal Tumors ; Humans ; Incidence ; Lipoma ; Pancreas ; Paraganglioma ; Pathology ; Retrospective Studies

With deeper understanding of gastrointestinal stromal tumor(GIST), more and more patients are diagnosed as GIST. Although the prognosis of early GIST is satisfactory after complete surgical resection, there are still many problems in the treatment of advanced GIST. Variety of treatment options has been used in the treatment of GIST, such as surgery, targeted drug therapy, and surgery plus imatinib therapy. However, post-operative recurrence, imatinib-resistance, multi-targeted drug resistance are still challenges. Many clinical evidences show that a reasonable management strategy can improve the prognosis of patients with advanced GIST. All the doctors should have a clear mind to carry out appropriate interventions. Advanced GIST should not be simply considered to be either medical or surgical disease, but rather must be systematically managed by multidisciplinary team approach combining surgical oncology, medical oncology, pathology, and interventional medicine. This review will advocate suitable treatment strategies based on the most recent progresses in systemic treatment for advanced GIST and our clinical experience to achieve early detection, early prevention, proper management, and therefore improve the survival of these patients.
Gastrointestinal Stromal Tumors ; diagnosis ; drug therapy ; pathology ; surgery ; Humans ; Neoplasm Metastasis

OBJECTIVETo investigate the clinicopathological and molecular genetic characteristics of gastrointestinal stromal tumor (GISTs) with significant cystic changes, and to assess their biological behavior.

METHODSClinicopathological features of 7 patients with cystic GISTs treated at the Zhongshan Hospital of Fudan University from February 2005 to January 2010 were summarized retrospectively. The mutations status of c-kit and PDGFR-α were analyzed.

RESULTSThere were 2 males and 5 females aged from 46 to 76 years old. Primary site of GISTs included stomach(n=4), duodenum(n=1), and small intestinal(n=2). Tumor size ranged from 6 to 16 cm with obviously cystic changes. Tumor cells were found in the solid components under microscope, of which epithelioid cell type were found in 4 case and spindle cell type in 3 cases. The mitotic figures were no more than 3/50 HPF in all the patients. According to the NIH criteria, 4 were high-risk and 3 were low-risk. Based on morphological characteristics, 3 cases were as borderline tumor, 3 moderate-risk, and 1 moderate-risk. Gene mutation of exon 11 of c-kit were identified in 3 cases. During the follow up ranging from 9 to 80 months, all the 7 patients had cancer-free survival.

CONCLUSIONThe biological behavior of cystic GIST is indolent with a low risk of malignancy and favorable prognosis.

Aged ; Female ; Gastrointestinal Stromal Tumors ; diagnosis ; pathology ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies

We describe an unusual case of extragastrointestinal stromal tumor (EGIST) presenting as a scrotal mass. A 71-year-old man presented with a gradually enlarging scrotal mass with a 20-year duration. Physical examination revealed a huge (as large as volleyball), round, nontender mass occupying the whole scrotum, which was resected completely. Clinical and radiological findings did not comply with any other primary site disease. Under histological examination, the tumor showed a spindle cell pattern with low cellularity, absence of necrotic and mitotic features. immunohistochemical analysis revealed the tumor reactive for CD117 and CD34, while negative for smooth muscle actin, desmin and S-100 protein. To our knowledge, this is the first reported case of an EGIST involving the scrotum.
Aged ; Antigens, CD34 ; analysis ; Gastrointestinal Stromal Tumors ; diagnosis ; Humans ; Immunohistochemistry ; Male ; Proto-Oncogene Proteins c-kit ; analysis ; Scrotum ; pathology ; Sex Cord-Gonadal Stromal Tumors ; chemistry ; pathology

Incidental gastrointestinal subepithelial mass is increasing with national cancer screening endoscopy. Most of gastrointestinal subepithelial mass are small-sized and asymptomatic tumor with benign nature, but gastrointestinal stromal tumor should be ruled-out because of its malignant behavior. Although conventional endoscopy alone can differentiate the nature of subepithelial mass, more accurate diagnosis can be achieved with endoscopic ultrasonography and its guided biopsy. In this review, differential diagnosis and treatment strategy of incidental gastrointestinal subepithelial mass would be presented.
Diagnosis, Differential ; Endoscopy, Gastrointestinal ; Gastrointestinal Stromal Tumors/*diagnosis/pathology/ultrasonography ; Humans ; Intestinal Neoplasms/diagnosis/pathology/ultrasonography ; Stomach Neoplasms/diagnosis/pathology/ultrasonography ; Tomography, X-Ray Computed

Neurofibromatosis type 1 (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous disorder characterized by abnormal skin pigmentation (cafe au lait spots and axillary freckling), cutaneous and plexiform neurofibromas, skeletal dysplasias, and Lisch nodules (pigmented iris hamartomas). Gastrointestinal stromal tumors (GISTs) are the most common tumors of mesenchymal origin in the gastrointestinal tract, mesentery, omentum, and retroperitoneum. Here, we report a case of GIST in the ileum of a 76-year-old woman previously diagnosed as NF-1. She was admitted due to sudden onset of abdominal pain. Contrast enhanced CT scan revealed a moderately defined, peripherally enhanced soft tissue mass of about 8.8 x 7.3 cm, originating from the small bowel in the left of the abdomen. Surgical excision was performed and the tumor was found to be composed of tumor cells that were positive for c-kit protein. The patient started imatinib treatment a month later, but stopped medication due to dyspepsia after a few months and eventually progressed after 18 months.
Risk Factors ; Neurofibromatosis 1/*diagnosis/pathology/surgery ; Humans ; Gastrointestinal Stromal Tumors/*diagnosis/pathology/surgery ; Female ; Aged ; Abdominal Pain

OBJECTIVETo investigate the clinical characteristics, surgical procedures and prognosis of duodenal gastrointestinal stromal tumors (GISTs).

METHODSThe clinical data of 18 GIST patients, collected from 1995 to 2004, were retrospectively analyzed.

RESULTSThe lesions of duodenal GISTs mainly located in the descending duodenum (13/18), unusually in the horizontal part (2/18), ascending part (1/18), and the bulb (2/18). Pathological examination revealed 1 case of benign tumor, 2 cases of borderline tumors and 15 cases of malignant tumors, Microscopically, the tumors were composed of spindle cells (14 cases), epithelial cell (1 case), and mixed cell types (3 cases). The clinical manifestations were non-specific, mostly was melena (7/18), as well as abdominal pain (6/18), fullness (5/18), and anemia (3/18). The diagnoses were performed by upper gastrointestinal radiography, gastroscopy, endoscopic ultrasonography and CT scan. All of the 18 patients received surgical treatment, including 9 pancreaticoduodenectomies, 5 local resections, 3 segmental resections of duodenum, and 1 distal subtotal gastrectomy. 1 and 3 year survival rates were 100% and 86.7% respectively.

CONCLUSIONMost duodenal GISTs are malignant, and the choices of surgical procedures are mainly determined by the location and size of the tumors.

Adult ; Aged ; Duodenal Neoplasms ; diagnosis ; pathology ; surgery ; Female ; Gastrointestinal Stromal Tumors ; diagnosis ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies