Pseudoglandular Schwannoma With <i>FUS::KLF17</i> Fusion: Broadening the Spectrum of <i>FUS</i>‐Associated Tumors

We present a case of a 51-year-old male with a pseudoglandular cellular schwannoma arising from the brachial plexus, which contains the expected molecular aberrations for a schwannoma (chromosome 22q loss encompassing the NF2 and LZTR1 genes) as well as a FUS::KLF17 rearrangement. Pseudoglandular schwannomas are rare morphologic variants of schwannomas that contain gland-like spaces lined by S100-positive, cytokeratin-negative pseudocolumnar Schwann cells. Fusions involving FUS and EWSR are commonly found in myoepithelial tumors of bone and soft tissue. While the spectrum of tumors with fusions involving FUS and EWSR is relatively broad, no cases, to our knowledge, have been reported of schwannomas, let alone the morphologically distinct pseudoglandular schwannoma, containing a FUS rearrangement. This case thus expands the spectrum of FUS rearranged tumors, highlighting the need for documentation of similar cases to understand the clinical significance of this combination.

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Publication Details

Journal
Genes Chromosomes and Cancer
Published
2025-08-01
DOI
https://doi.org/10.1002/gcc.70077
Citations
1
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
Field-Weighted Citation Impact
1.39
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article

Pseudoglandular Schwannoma With FUS::KLF17 Fusion: Broadening the Spectrum of FUS‐Associated Tumors

Diana Bell, Rania Bakkar, Jerome P. Givi, Michelle Afkhami et al.
1 citations
Genes Chromosomes and Cancer
Neurofibromatosis and Schwannoma Cases
1.39
article

Pseudoglandular Schwannoma With FUS::KLF17 Fusion: Broadening the Spectrum of FUS‐Associated Tumors

Diana Bell, Rania Bakkar, Jerome P. Givi, Michelle Afkhami, Daisy Wu
article en
1 citations

Abstract

We present a case of a 51-year-old male with a pseudoglandular cellular schwannoma arising from the brachial plexus, which contains the expected molecular aberrations for a schwannoma (chromosome 22q loss encompassing the NF2 and LZTR1 genes) as well as a FUS::KLF17 rearrangement. Pseudoglandular schwannomas are rare morphologic variants of schwannomas that contain gland-like spaces lined by S100-positive, cytokeratin-negative pseudocolumnar Schwann cells. Fusions involving FUS and EWSR are commonly found in myoepithelial tumors of bone and soft tissue. While the spectrum of tumors with fusions involving FUS and EWSR is relatively broad, no cases, to our knowledge, have been reported of schwannomas, let alone the morphologically distinct pseudoglandular schwannoma, containing a FUS rearrangement. This case thus expands the spectrum of FUS rearranged tumors, highlighting the need for documentation of similar cases to understand the clinical significance of this combination.

Genes Chromosomes and CancerVol. 64(8)
City of Hope (US), University of Pittsburgh Medical Center (US)
Openalex Percentile: Top 17%
Neurofibromatosis and Schwannoma Cases
1.39
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Pseudoglandular Schwannoma With <i>FUS::KLF17</i> Fusion: Broadening the Spectrum of <i>FUS</i>‐Associated Tumors — Diana Bell, Rania Bakkar, et al. · Genes Chromosomes and Cancer (2025) | TGRS Research Map | TGRS