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Updated: Mar 4, 2026

An Orthotopic Sciatic Nerve Xenograft for Neurofibromatosis Type 1 Neurofibromas
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Fibronectin 1 (FN1)-rearranged Mesenchymal Neoplasms: An Updated Review.

Jun Nishio1, Yoshiro Chijiiwa2, Yuki Shinohara2

  • 1Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, Fukuoka, Japan; nishio@fdcnet.ac.jp.

Cancer Genomics & Proteomics
|March 2, 2026
PubMed
Summary

Fibronectin 1 (FN1) gene rearrangements are found in various chondroid matrix tumors. This review details the clinical, histological, and molecular features of FN1-rearranged mesenchymal neoplasms.

Keywords:
FN1calcified chondroid mesenchymal neoplasmcalcifying aponeurotic fibromainflammatory myofibroblastic tumorlipofibromatosisphosphaturic mesenchymal tumorreviewsoft tissue chondromasynovial chondromatosis

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Area of Science:

  • Oncology
  • Genetics
  • Pathology

Background:

  • Fibronectin 1 (FN1) gene encodes extracellular matrix glycoprotein fibronectin.
  • FN1 rearrangements are identified in chondroid matrix-producing tumors like synovial chondromatosis and phosphaturic mesenchymal tumors.
  • Recent findings link FN1 fusions to lipofibromatosis and inflammatory myofibroblastic tumors.

Purpose of the Study:

  • To provide an updated overview of FN1-rearranged mesenchymal neoplasms.
  • To discuss the clinical, histological, and molecular genetic characteristics of these tumors.
  • To explore the relationships between different FN1-rearranged neoplasms.

Main Methods:

  • Literature review of clinical, histological, and molecular data.
  • Analysis of World Health Organization (WHO) classifications.
  • Synthesis of current knowledge on FN1-rearranged mesenchymal neoplasms.

Main Results:

  • FN1 rearrangements characterize a spectrum of mesenchymal neoplasms.
  • These tumors often exhibit overlapping histological features.
  • Current classifications suggest FN1-rearranged lesions are typically benign or intermediate grade.

Conclusions:

  • FN1-rearranged mesenchymal neoplasms represent a distinct group of tumors.
  • Understanding their molecular genetics is crucial for accurate diagnosis and classification.
  • Further research can elucidate the precise biological behavior and therapeutic implications.