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| Leiomyosarcoma or Gastrointestinal Stromal Tumor | ||
| Etiology •Unknown | ||
| Pathogenesis •Unknown •While it is difficult to distinguish between some benign mesenchymal tumors and low grade sarcomas, there is no convincing evidence that intestinal sarcomas arise from their benign counterparts. •While some tumors show evidence of clearcut differentiation, most commonly smooth muscle, many show evidence of of two or more differentiated cell lines prompting the current practice to call these tumors "Gastointestinal Stromal Tumors"(GIST) and then specifiy the particular line of differentiation identifies. •The adoption of GIST also suggests that these tumors may arise from a multipotential progenitor cell., | ||
| Epidemiology •Incidence increases between age 30-60yrs. and then levels off. •Equal incidence in men and women, although some series report a female predominance. •No geographic preference | ||
| General Gross Description •Malignant GIST tumors showing leiomyomatous differentiation are most commonly present in the muscular wall of the Jejeunum and Ileum, and much less commonly oin the large intestine. •The tumors are generally well circumscribed, with a white firm cut surface. •Large tumors may show central necrosis and hemorrhage. •Some tumors may be endophytic, bulging into the lumen of the intestine. •Smaller tumors have an intact overlying mucosal surface. •Many tumors are exophytic and bulge from the serosal surface into the peritoneal cavity or adjoining tissue. •Regional lymph nodes are usually unimpressive, as only 15% contain metastatic tumor. •Adjoining peritoneal surfaces and the liver are most likely to contain metastatic tumor. •Examples: | ||
| General Microscopic Description •Malignant GIST tumors most commonly have a spindle cell histology. •The most reliable parameter for a diagnosis of malignancy apart from the demonstration of metastases is mitotic rate. •All GIST tumors with >5 mitoses/10hpf should be considered to be malignant. •All GIST tumors with 1-4 mitoses/10hpf may have malignant potential as 40% of these tumors will eventually demonstrate metastases. •Tumors over 4cm in diameter should be considered to have malignant potential although this is a less reliable guide than mitotic activity. •The most reliable way to subclassify these tumors is the presence of specific markers on immunoperoxidase examination: smooth muscle-Vimentin+,Actin+,Desmin+; Fibroblastic-Vimentin+; Neural-S-100+, Vimentin+, and Neurofilament+; vascular-CD-34+ or Factor 8+ •Electrom Microscopy is a useful adjunct to identify specific cellular organelles. •Examples: | ||
| References •DeVita VT et al eds.: Cancer: Priciples and Practice of Oncology. Philadelphia, JB Lippincott, 4th ed. 1993, pp915-928 •Gastrointestinal Pathology, Fenoglio-Preiser CM, etal.Raven Press New York 1989 pp543-585 This link will directly take you to the relevant new literature Leiomyosarcoma or Gastrointestinal Stromal Tumor
| Synopsis by: Martin Nadel M.D. (T64000M88903)[450]
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