Abstract |
This case report documents the first karyotypic, fluorescence in situ hybridization, and genetic analysis of an angiomatoid fibrous histiocytoma that arose and recurred in the arm of a 5.5-year-old girl. Complex rearrangements between chromosomes 2, 12, 16, and 17 were noted, as well as deletion in the long arm of chromosome 11. Flow cytometry revealed a normal cell population. The t(12;16) site was further investigated using reverse transcriptase-polymerase chain reaction. We found that the FUS (also known as TLS) gene from 16p11 combined with the ATF-1 gene from 12q13 to generate a chimeric FUS/ATF-1. The FUS gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma and in acute myeloid leukemia with t(16;21)(p11;q22), while the ATF-1 gene is rearranged in the t(12;22)(q13;q12) found recurrently in clear cell sarcomas ( malignant melanoma of soft parts). Thus, the FUS/ATF-1 gene in angiomatoid fibrous histiocytoma is predicted to code for a protein that is very similar to the chimeric EWS/ATF-1 found in clear cell sarcoma.
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Authors | B L Waters, I Panagopoulos, E F Allen |
Journal | Cancer genetics and cytogenetics
(Cancer Genet Cytogenet)
Vol. 121
Issue 2
Pg. 109-16
(Sep 2000)
ISSN: 0165-4608 [Print] United States |
PMID | 11063792
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Activating Transcription Factor 1
- DNA, Complementary
- DNA-Binding Proteins
- Heterogeneous-Nuclear Ribonucleoproteins
- RNA-Binding Protein EWS
- RNA-Binding Protein FUS
- Ribonucleoproteins
- Transcription Factors
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Topics |
- Activating Transcription Factor 1
- Amino Acid Sequence
- Artificial Gene Fusion
- Base Sequence
- Child
- Chromosomes, Human, Pair 12
- Chromosomes, Human, Pair 16
- DNA, Complementary
- DNA-Binding Proteins
- Female
- Hemangioma
(genetics)
- Heterogeneous-Nuclear Ribonucleoproteins
- Histiocytoma, Benign Fibrous
(genetics)
- Humans
- In Situ Hybridization, Fluorescence
- Karyotyping
- Molecular Sequence Data
- RNA-Binding Protein EWS
- RNA-Binding Protein FUS
- Ribonucleoproteins
(genetics)
- Soft Tissue Neoplasms
(genetics)
- Transcription Factors
(genetics)
- Translocation, Genetic
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