PPP6R3-USP6 amplification: Novel oncogenic mechanism in malignant nodular fasciitis

Ruifeng Guo; Xiaoke Wang; Margaret M. Chou; Yan Asmann; Doris E. Wenger; Alyaa Al-Ibraheemi; Diana W. Molavi; Albert Aboulafia; Long Jin; Karen Fritchie; Jennifer L. Oliveira; Robert B. Jenkins; Jennifer J. Westendorf; Jie Dong; Andre M. Oliveira

doi:10.1002/gcc.22366

PPP6R3-USP6 amplification: Novel oncogenic mechanism in malignant nodular fasciitis

Ruifeng Guo, Xiaoke Wang, Margaret M. Chou, Yan Asmann, Doris E. Wenger, Alyaa Al-Ibraheemi, Diana W. Molavi, Albert Aboulafia, Long Jin, Karen Fritchie, Jennifer L. Oliveira, Robert B. Jenkins, Jennifer J. Westendorf, Jie Dong, Andre M. Oliveira

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Abstract

Nodular fasciitis (NF) is a clonal self-limited neoplastic proliferation characterized by rearrangements of the USP6 locus in most examples. To our knowledge well-documented malignant behavior has never been previously observed in NF. In this report we present an unusual case of NF with classical histologic features that showed a protracted clinical course characterized by multiple recurrences and eventual metastatic behavior over a period of 10 years. Molecular analyses revealed the presence and amplification of the novel PPPR6-USP6 gene fusion, which resulted in USP6 mRNA transcriptional upregulation. These findings further support the oncogenic role of the USP6 protease in mesenchymal neoplasia and expand the biologic potential of NF.

Original language	English (US)
Pages (from-to)	640-649
Number of pages	10
Journal	Genes Chromosomes and Cancer
Volume	55
Issue number	8
DOIs	https://doi.org/10.1002/gcc.22366
State	Published - Aug 1 2016

ASJC Scopus subject areas

Genetics
Cancer Research

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Guo, R., Wang, X., Chou, M. M., Asmann, Y., Wenger, D. E., Al-Ibraheemi, A., Molavi, D. W., Aboulafia, A., Jin, L., Fritchie, K., Oliveira, J. L., Jenkins, R. B., Westendorf, J. J., Dong, J., & Oliveira, A. M. (2016). PPP6R3-USP6 amplification: Novel oncogenic mechanism in malignant nodular fasciitis. Genes Chromosomes and Cancer, 55(8), 640-649. https://doi.org/10.1002/gcc.22366

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title = "PPP6R3-USP6 amplification: Novel oncogenic mechanism in malignant nodular fasciitis",

abstract = "Nodular fasciitis (NF) is a clonal self-limited neoplastic proliferation characterized by rearrangements of the USP6 locus in most examples. To our knowledge well-documented malignant behavior has never been previously observed in NF. In this report we present an unusual case of NF with classical histologic features that showed a protracted clinical course characterized by multiple recurrences and eventual metastatic behavior over a period of 10 years. Molecular analyses revealed the presence and amplification of the novel PPPR6-USP6 gene fusion, which resulted in USP6 mRNA transcriptional upregulation. These findings further support the oncogenic role of the USP6 protease in mesenchymal neoplasia and expand the biologic potential of NF.",

author = "Ruifeng Guo and Xiaoke Wang and Chou, {Margaret M.} and Yan Asmann and Wenger, {Doris E.} and Alyaa Al-Ibraheemi and Molavi, {Diana W.} and Albert Aboulafia and Long Jin and Karen Fritchie and Oliveira, {Jennifer L.} and Jenkins, {Robert B.} and Westendorf, {Jennifer J.} and Jie Dong and Oliveira, {Andre M.}",

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doi = "10.1002/gcc.22366",

language = "English (US)",

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T1 - PPP6R3-USP6 amplification

T2 - Novel oncogenic mechanism in malignant nodular fasciitis

AU - Guo, Ruifeng

AU - Wang, Xiaoke

AU - Chou, Margaret M.

AU - Asmann, Yan

AU - Wenger, Doris E.

AU - Al-Ibraheemi, Alyaa

AU - Molavi, Diana W.

AU - Aboulafia, Albert

AU - Jin, Long

AU - Fritchie, Karen

AU - Oliveira, Jennifer L.

AU - Jenkins, Robert B.

AU - Westendorf, Jennifer J.

AU - Dong, Jie

AU - Oliveira, Andre M.

PY - 2016/8/1

Y1 - 2016/8/1

N2 - Nodular fasciitis (NF) is a clonal self-limited neoplastic proliferation characterized by rearrangements of the USP6 locus in most examples. To our knowledge well-documented malignant behavior has never been previously observed in NF. In this report we present an unusual case of NF with classical histologic features that showed a protracted clinical course characterized by multiple recurrences and eventual metastatic behavior over a period of 10 years. Molecular analyses revealed the presence and amplification of the novel PPPR6-USP6 gene fusion, which resulted in USP6 mRNA transcriptional upregulation. These findings further support the oncogenic role of the USP6 protease in mesenchymal neoplasia and expand the biologic potential of NF.

AB - Nodular fasciitis (NF) is a clonal self-limited neoplastic proliferation characterized by rearrangements of the USP6 locus in most examples. To our knowledge well-documented malignant behavior has never been previously observed in NF. In this report we present an unusual case of NF with classical histologic features that showed a protracted clinical course characterized by multiple recurrences and eventual metastatic behavior over a period of 10 years. Molecular analyses revealed the presence and amplification of the novel PPPR6-USP6 gene fusion, which resulted in USP6 mRNA transcriptional upregulation. These findings further support the oncogenic role of the USP6 protease in mesenchymal neoplasia and expand the biologic potential of NF.

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JO - Genes Chromosomes and Cancer

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PPP6R3-USP6 amplification: Novel oncogenic mechanism in malignant nodular fasciitis

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