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PRRX1-NCOA1-rearranged fibroblastic tumour: a clinicopathological, immunohistochemical and molecular genetic study of six cases of a potentially under-recognised, distinctive mesenchymal tumour.

Josephine Kam Tai K DermawanElizabeth M AzzatoJudith Jebastin ThangaiahSandra Gjorgova-GjeorgievskiBrian P RubinAndrew L FolpeAbbas AgaimyKaren J Fritchie
Published in: Histopathology (2021)
The morphological features of PRRX1-NCOA1-rearranged fibroblastic tumour overlap with those of RB1-deficient soft-tissue tumours, solitary fibrous tumour, and low-grade fibromyxoid sarcoma/sclerosing epithelioid fibrosarcoma. This differential diagnosis can be resolved with a combination of careful morphological study and the application of a panel of immunostains, although molecular genetic study is most definitive. The natural history of PRRX1-NCOA1-rearranged fibroblastic tumour appears to be quite favourable, although longer-term study of a larger number of cases is warranted.
Keyphrases
  • low grade
  • gene expression
  • bone marrow
  • genome wide
  • radiation therapy
  • preterm infants
  • single molecule