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FOXO1-FGFR1 fusion and amplification in a solid variant of alveolar rhabdomyosarcoma
Journal article   Open access   Peer reviewed

FOXO1-FGFR1 fusion and amplification in a solid variant of alveolar rhabdomyosarcoma

Jinglan Liu, Miguel A. Guzman, Donna Pezanowski, Dilipkumar Patel, John Hauptman, Matthew Keisling, Steve J. Hou, Peter R. Papenhausen, Judy M. Pascasio, Hope H. Punnett, …
Modern pathology, v 24(10), pp 1327-1335
01 Oct 2011
PMID: 21666686
url
https://doi.org/10.1038/modpathol.2011.98View
Published, Version of Record (VoR)Maybe Open Access (Publisher Bronze) Open

Abstract

Life Sciences & Biomedicine Pathology Science & Technology
Rhabdomyosarcoma is the most common pediatric soft tissue malignancy. Two major subtypes, alveolar rhabdomyosarcoma and embryonal rhabdomyosarcoma, constitute 20 and 60% of all cases, respectively. Approximately 80% of alveolar rhabdomyosarcoma carry two signature chromosomal translocations, t(2;13)(q35;q14) resulting in PAX3-FOXO1 fusion, and t(1; 13)(p36;q14) resulting in PAX7-FOXO1 fusion. Whether the remaining cases are truly negative for gene fusion has been questioned. We are reporting the case of a 9-month-old girl with a metastatic neck mass diagnosed histologically as solid variant alveolar rhabdomyosarcoma. Chromosome analysis showed a t(8;13;9)(p11.2;q14;9q32) three-way translocation as the sole clonal aberration. Fluorescent in situ hybridization (FISH) demonstrated a rearrangement at the FOXO1 locus and an amplification of its centromeric region. Single-nucleotide polymorphism-based microarray analysis illustrated a co-amplification of the FOXO1 gene at 13q14 and the FGFR1 gene at 8p12p11.2, suggesting formation and amplification of a chimerical FOXO1-FGFR1 gene. This is the first report to identify a novel fusion partner FGFR1 for the known anchor gene FOXO1 in alveolar rhabdomyosarcoma. Modern Pathology (2011) 24, 1327-1335; doi: 10.1038/modpathol.2011.98; published online 10 June 2011

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Pathology
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