INTRAABDOMINAL DESMOPLASTIC SMALL-CELL TUMORS - REPORT OF 2 CASES

Two young adults that presented with intra-abdominal desmoplastic smal l cell tumors (DSCT) without any evidence of a primary site are descri bed. Both cases share the clinical characteristic features of this rar e tumor which include predominant intra-abdominal location as initial presentation, nesting pattern of growth, intense desmoplastic reaction , immunohistochemical reactivity for epithelial, neural and muscle mar kers, and highly aggressive behavior, Aggressive chemotherapy with a c isplatin-containing regimen was the main therapy to our patients, Up t o the present, both cases are alive with disease, The survival is 18 a nd 15 months from the initial diagnosis, respectively, Interestingly, one of the cases encountered an episode of cerebral infarction at the territory of the left middle cerebral artery 12 days after the first c ycle of chemotherapy, This is a previously unrecognized manifestation for this tumor type, This causal relationship between chemotherapy and an acute vascular event is the most likely explanation for our patien t's stroke.