Abstract
Hepatic tumors are rare childhood neoplasms with uncertain etiology. We report the cooccurrence of hepatic tumors in 2 boys with fragile X syndrome, one with hepatoblastoma and another with desmoplastic nested spindle cell tumor of liver. The pathogenesis of fragile X syndrome involves silencing of the fragile X mental retardation 1 gene and consequent loss of FMR1 protein. We speculate regarding molecular pathways that might explain the cooccurrence of the 2 conditions. Further examination of a possible functional link between hepatic neoplasia and loss of FMRP is warranted.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 239-241 |
| Number of pages | 3 |
| Journal | Journal of Pediatric Hematology/Oncology |
| Volume | 30 |
| Issue number | 3 |
| DOIs | |
| State | Published - Mar 2008 |
Keywords
- FMRP
- Fragile X syndrome
- Hepatic tumor
- Hepatoblastoma
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Oncology
- Hematology
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Cite this
Wirojanan, J., Kraff, J., Hawkins, D. S., Laird, C., Gane, L. W., Angkustsiri, K., Tassone, F., & Hagerman, R. J. (2008). Two boys with fragile X syndrome and hepatic tumors. Journal of Pediatric Hematology/Oncology, 30(3), 239-241. https://doi.org/10.1097/MPH.0b013e31815f88c9