Soft tissue sarcoma subtypes exhibit distinct patterns of acquired uniparental disomy.

[dedifferentiated liposarcoma]

Soft tissue sarcomas (STS ) are heterogeneous mesenchymal tumors with diverse subtypes . STS can be classified into two main categories according to the type of genomic alteration : recurrent translocation driven STS , and non-recurrent translocations . However , little has known about acquired uniparental disomy in STS .In this study , we analyzed SNP microarray data to determine the frequency and distribution patterns of acquired uniparental disomy (aUPD ) in major soft tissue sarcoma (STS ) subtypes using CNAG and R softwares .We identified recurrent aUPD regions specific to alveolar rhabdomyosarcoma with the most frequent at 11 p 15 .4 , gastrointestinal stromal tumor at 1 p 36 .11 -p 35 .3 , leiomyosarcoma at 17 p 13 .3 -p 13 .1 , myxofibrosarcoma at 1 p 35 .1 -p 34 .2 and 16 q 23 .3 -q 24 .1 , and pleomorphic liposarcoma at 13 q 13 .2 -q 13 .3 and 13 q 14 .11 -q 14 .2 . In contrast , specific recurrent aUPD regions were not identified in dedifferentiated liposarcoma , Ewing sarcoma , myxoid /round cell liposarcoma , and synovial sarcoma . Strikingly total , centromeric and segmental aUPD regions are more frequent in STS that do not exhibit recurrent translocation events .Our study yields a detailed map of aUPD across 9 diverse STS subtypes and suggests the potential location of several novel tumor suppressor genes and oncogenes .

Diseases
Validation

Diseases presenting "tumor suppressor genes" symptom

cowden syndrome

dedifferentiated liposarcoma

esophageal adenocarcinoma

lymphangioleiomyomatosis

oral submucous fibrosis

pleomorphic liposarcoma

proteus syndrome

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