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Related Concept Videos

Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...
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  3. Prrx1 -rearranged Fibroblastic Tumors : A Clinicopathologic And Molecular Study Of 18 Cases Including A Novel Prrx1::ep300 Fusion.
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  3. Prrx1 -rearranged Fibroblastic Tumors : A Clinicopathologic And Molecular Study Of 18 Cases Including A Novel Prrx1::ep300 Fusion.

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PRRX1 -rearranged Fibroblastic Tumors : A Clinicopathologic and Molecular Study of 18 Cases Including a Novel

Carina A Dehner1,2, Jorge Torres-Mora3, Judith Jebastin Thangaiah3

  • 1Department of Pathology, Indiana University School of Medicine, Indianapolis, IN.

The American Journal of Surgical Pathology
|October 2, 2025

View abstract on PubMed

Summary
This summary is machine-generated.

This study details 18 rare PRRX1-rearranged tumors, finding they exhibit indolent behavior and characteristic morphology. Molecular analysis revealed PRRX1::NCOA1 and PRRX1::EP300 fusions, aiding in diagnosis of these fibroblastic neoplasms.

Keywords:
fibroblastic tumor

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Area of Science:

  • Pathology
  • Oncology
  • Genetics

Background:

  • PRRX1-rearranged tumors are rare fibroblastic neoplasms with limited documented cases.
  • Understanding their clinical and molecular characteristics is crucial for accurate diagnosis and patient management.

Purpose of the Study:

  • To expand the understanding of PRRX1-rearranged tumors by analyzing the largest case series to date.
  • To characterize the clinical behavior, morphology, and molecular alterations of these uncommon neoplasms.

Main Methods:

  • Retrospective analysis of 18 PRRX1-rearranged tumor cases.
  • Morphological and immunohistochemical evaluation (S100, SOX10, RB expression).
  • Molecular analysis to identify gene fusions (PRRX1::NCOA1, PRRX1::EP300).

Main Results:

  • The series included 18 patients (median age 35 years) with tumors in various locations.
  • Clinical follow-up (50% of cases) indicated indolent behavior without recurrence or metastasis.
  • Morphology showed well-circumscribed tumors with distinctive vessels, uniform cells, and minimal atypia.
  • Immunohistochemistry revealed focal S100/SOX10 expression in some cases and complete loss of RB expression in nearly half.
  • All tumors harbored PRRX1::NCOA1 or a novel PRRX1::EP300 fusion.

Conclusions:

  • PRRX1-rearranged tumors are rare, indolent neoplasms with distinctive morphological and molecular features.
  • The study expands the molecular spectrum of these tumors, identifying PRRX1::NCOA1 and PRRX1::EP300 fusions.
  • Accurate diagnosis is aided by recognizing these features and differentiating them from aggressive neoplasms.