Detection and clinical significance of bone marrow involvement in patients with rhabdomyosarcoma
Language English Country Germany Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Rhabdomyosarcoma, Alveolar chemistry genetics pathology MeSH
- Child MeSH
- Rhabdomyosarcoma, Embryonal genetics pathology MeSH
- Forkhead Box Protein O1 MeSH
- Forkhead Transcription Factors analysis MeSH
- Infant MeSH
- Bone Marrow chemistry pathology MeSH
- Humans MeSH
- Neoplasm Recurrence, Local pathology MeSH
- Adolescent MeSH
- Young Adult MeSH
- MyoD Protein genetics MeSH
- Biomarkers, Tumor analysis MeSH
- Reverse Transcriptase Polymerase Chain Reaction methods MeSH
- Child, Preschool MeSH
- Prognosis MeSH
- Recombinant Fusion Proteins analysis MeSH
- Rhabdomyosarcoma chemistry genetics mortality pathology MeSH
- PAX3 Transcription Factor MeSH
- PAX7 Transcription Factor analysis MeSH
- Paired Box Transcription Factors analysis MeSH
- Check Tag
- Child MeSH
- Infant MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Forkhead Box Protein O1 MeSH
- Forkhead Transcription Factors MeSH
- FOXO1 protein, human MeSH Browser
- MyoD Protein MeSH
- MyoD1 myogenic differentiation protein MeSH Browser
- Biomarkers, Tumor MeSH
- PAX3 protein, human MeSH Browser
- PAX7 protein, human MeSH Browser
- Recombinant Fusion Proteins MeSH
- PAX3 Transcription Factor MeSH
- PAX7 Transcription Factor MeSH
- Paired Box Transcription Factors MeSH
Childhood rhabdomyosarcoma (RMS) has two major histological subtypes: alveolar (aRMS) and embryonal. The aim of the study was to monitor minimal disseminated disease (MDD) using real-time quantitative reverse-transcription PCR (RQ-RT-PCR) of the PAX3-FKHR, PAX7-FKHR fusion genes and myoD1 gene. We prepared an assay using RQ-RT-PCR for a quantitative assessment of MDD in aRMS by using hydrolysis probe for quantification of PAX3-FKHR, PAX7-FKHR and myoD1 genes and beta-2-microglobulin housekeeping gene. Primary tumor samples (44), samples of local recurrences (26) from 48 patients with aRMS were examined by nested RT-PCR and RQ-RT-PCR techniques. Additionally, bone marrow samples (115), peripheral blood progenitor cell samples (27), and peripheral blood samples (25) from 33 aRMS patients were tested. PAX3/7-FKHR and myoD1 transcripts proved to be a sensitive tool for detection of MDD in RMS. We were able to identify 15/25 patients with bone marrow (BM) involvement at the time of presentation using RQ-RT-PCR. We analyzed PAX3-FKHR or PAX7-FKHR expression during the course of the disease. The RQ-RT-PCR results correlated well with nested RT-PCR results (p < 0.0001). The presence of metastases is the most adverse prognostic factor in RMS, and bone marrow is a frequent site of the tumor dissemination in RMS, especially in aRMS. Our results detecting the fusion transcripts or myoD1 transcript in the BM or peripheral blood suggest that patients with positive findings are at high risk of the tumor progression.
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