Extragastrointestinal Stromal Tumors: A Clinical Study Of 21 Cases

[Objective]To investigate the clinical features,diagnosis,treatment and prognostic factors of the extragastrointestinal stromal tumors(EGIST).[Methods]Retrospectively analyze the clinical data of 21 patients with EGIST treated in our hospital from January 2000 to June 2008.All the patients were followed up from 6 months to 62 months(Median:22 months),and the follow-up rate was 85.7%(18/21).[Results]The EGIST incidence was 4.7%of GIST.EGIST occurred in 11 men and 10 women,who ranged in age from 38 to 75 years(median:57 years).The course of the disease were up to a maximum of 4 years and the shortest was accidentally discovered in an operation.The most common clinical presentations of EGIST were abdomen mass(9 cases ),abdominal pain or discomfort(10 cases),abnormal defecation(2 cases),1 case was discoveried at the admission examination of periampullary carcinoma,1 case was accidentally discoveried in a rectal cancer operation.Of the 21 patients,10 cases arose from retroperitoneum,5 cases from the mesentery,5 cases from the omentum,1 case from the liver.The diameter of tumor ranged from 1 to16 centimetres(median:8 cm).The B ultrasonic examination found 19 cases and the CT found 20 cases.Of the morphology of the cancer cell,17 cases were the spindle cell-predominant type,1 case was the epithelioid cell-predominant type,3 cases were the mixed type. Immunohistochemical staining showed the positive rate of the CD117,CD34,SMA, S100,HHF35 were 100%,76.2%,19.3%,9.6%,4.8%,respectively.The 21 patients with EGIST were all accepted surgical resection.4 cases relapsed and 1 patient received Imatinib therapy after recurrence,the result was partial remission.The overall 1-,3-,5-year survival rates were 77.8%,38.9%,5.6%,respectively,and the median survival time was 26 months.[Conclusions]EGIST is a rare disease with poor prognosis.The diagnosis mainly depends on the imaging examination,the histopathological examination and the immunohistochemical techniques.Extension resection is one of the most effective measures to EGIST,and Imatinib is a highly effective target drug to EGIST.The prognosis has something to do with the location and size of tumor,but nothing with the sex and age of the patients.