Gastrointestinal stromal tumor: a clinicopathological study of 74 cases

Abstract

To explore a simplified and reproducible approach for the diagnosis and morphologic prognostication of gastrointestinal stromal tumor (GIST). Eighty-five cases of gastrointestinal mesenchymal tumors including 74 cases of GIST, 8 esophageal smooth muscle tumor, 1 rectal leiomyosarcoma, 1 Schwannoma, and 1 malignant fibrous histiocytoma were studied by histological evaluation along with an immunohistochemistry panel including vimentin, CD117 (c-kit), CD34, SMA, desmin and S-100. Clinicopathological correlation was performed in 31 cases of GIST that had accompanied with the available follow-up data. Among 74 GISTs, 34 arose principally from the stomach, 30 from the small intestine, and 10 other cases found in the esophagus, retroperitoneum, mesenterium and omentum. The patients' age ranged from 23 to 80 years (mean 52.5 years), with 45 males and 29 females. Histologically, the tumors composed of either spindle or oval to round cells arranged in interlacing fascicles forming whorls or cellular clusters, cytoplasm generally abundant and eosinophilic. There were 48 cases of spindle cell type, 10 cases of epithelioid cell type and 16 cases of mixed cell type. All 74 cases of GIST were positive for CD117 in a cell membranous pattern, however, some variable staining patterns of CD117 had been noticed in a few cases. In addition, 54 GISTs were also positive for CD34 (72.9%), 25 cases positive for SMA, 5 cases positive for S-100 and 5 cases positive for desmin. According to the Fletcher's scheme, GISTs in this study were divided into 4 subcategories including groups of very low risk of aggressive behavior (3 cases), of low risk (15 cases), of intermediate risk (36 cases) and of high risk (20 cases) respectively. Kaplan-Meier survival analysis of 31 GIST cases whom had been followed up for 16 to 72 months showed a statistically significant difference present among the subcategories (P < 0.01). GISTs predominantly occur in the middle and old age patients, more common in male, and positive CD117 staining is considered to be the defining marker to differentiate GIST from other mesenchymal tumors of the GI tract. Positive CD34 immun-staining, plus a CD117 positivity, strengthens further a diagnosis of GIST. Subclassification of GISTs using Fletcher's scheme appears to be simple, reproducible, and correlates well with the clinical behavior of the tumor.