TY - JOUR
T1 - The emerging PRRX1-NCOA fibroblastic neoplasm
T2 - a combined reappraisal of published tumors and two new cases
AU - Chen, Chien Heng
AU - Chang, Kung Chao
AU - Chuang, Chieh Han
AU - Fu, Jing Tong
AU - Huang, Hsuan Ying
N1 - Funding Information:
This work was sponsored in part by the Chang Gung Hospital (CMRPG8L0371 to HYH).
Funding Information:
The authors also thank Kaohsiung Chang Gung genomic (CLRPG8G0591) and tissue bank (CLRPG8F1701 and CLRPG8F1702) core laboratories for technical assistance.
Publisher Copyright:
© 2021, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2022/7
Y1 - 2022/7
N2 - Nuclear receptor coactivator (NCOA) family gene fusions have been increasingly discovered in diverse mesenchymal neoplasms, while PRRX1-NCOA-fused fibroblastic tumors still remain insufficiently characterized. We herein present two additional PRRX1-NOCA1-positive cases sharing lobulated hypocellular growth of innocuous spindle-to-stellate cells in a fibromyxoid stroma enriched with polymorphous vessels. A constellation of low cellularity, alternating myxocollagenous matrix, bland cytomorphology, and, especially, unusual collagenous rosettes in one case were morphologically reminiscent of low-grade fibromyxoid sarcoma. In both cases, immunoprofiles were similarly nondescript and negative for all diagnostic markers, including MUC4, emphasizing the diagnostic value of molecular testing. Review of published and current cases highlights a striking predominance of PRRX1-NCOA1, unusual collagenous rosettes, and favorable behavior in this emerging fibroblastic tumor type.
AB - Nuclear receptor coactivator (NCOA) family gene fusions have been increasingly discovered in diverse mesenchymal neoplasms, while PRRX1-NCOA-fused fibroblastic tumors still remain insufficiently characterized. We herein present two additional PRRX1-NOCA1-positive cases sharing lobulated hypocellular growth of innocuous spindle-to-stellate cells in a fibromyxoid stroma enriched with polymorphous vessels. A constellation of low cellularity, alternating myxocollagenous matrix, bland cytomorphology, and, especially, unusual collagenous rosettes in one case were morphologically reminiscent of low-grade fibromyxoid sarcoma. In both cases, immunoprofiles were similarly nondescript and negative for all diagnostic markers, including MUC4, emphasizing the diagnostic value of molecular testing. Review of published and current cases highlights a striking predominance of PRRX1-NCOA1, unusual collagenous rosettes, and favorable behavior in this emerging fibroblastic tumor type.
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U2 - 10.1007/s00428-021-03219-x
DO - 10.1007/s00428-021-03219-x
M3 - Article
C2 - 34647172
AN - SCOPUS:85116976469
VL - 481
SP - 111
EP - 116
JO - Virchows Archiv - Abteilung A Pathologische Anatomie
JF - Virchows Archiv - Abteilung A Pathologische Anatomie
SN - 0945-6317
IS - 1
ER -