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Related Experiment Video

Updated: Jan 7, 2026

Lateral Molar Approach-Driven Transoral Endoscopic Procedure for Benign Infratemporal Fossa Tumor Resection
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Published on: August 15, 2025

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Calcifying Aponeurotic Fibroma: A Review and Update.

Jun Nishio1, Yuki Shinohara2, Mikiro Koga3

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

In Vivo (Athens, Greece)
|January 2, 2026
PubMed
Summary

Calcifying aponeurotic fibroma (CAF) is a rare tumor in children and adolescents. This review details its clinical, imaging, and molecular features, aiding diagnosis and management.

Keywords:
Calcifying aponeurotic fibromacalcified chondroid mesenchymal neoplasmepidermal growth factorfibronectin 1fusion genelipofibromatosisreview

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Area of Science:

  • Oncology
  • Pathology
  • Radiology

Background:

  • Calcifying aponeurotic fibroma (CAF) is a rare, benign, yet locally aggressive mesenchymal tumor.
  • It predominantly affects the distal extremities of pediatric and adolescent populations.
  • CAF typically manifests as a slow-growing, painless mass with prolonged duration.

Purpose of the Study:

  • To provide an updated overview of calcifying aponeurotic fibroma (CAF).
  • To cover clinical, radiological, morphological, immunohistochemical, and molecular genetic features.
  • To discuss the differential diagnosis of this uncommon condition.

Main Methods:

  • Review of clinical presentations and diagnostic imaging (radiography, MRI).
  • Histopathological and immunohistochemical analysis of tumor characteristics.
  • Molecular genetic studies identifying gene fusions (e.g., FN1-EGF).

Main Results:

  • CAF presents as a poorly circumscribed soft-tissue mass with calcifications.
  • MRI reveals ill-defined subcutaneous masses with characteristic signal intensities and enhancement patterns.
  • Tumor cells show variable expression of SMA, MSA, CD99, ERG, and EGF, with a specific FN1-EGF gene fusion.

Conclusions:

  • CAF diagnosis requires integrating clinical, imaging, histological, and molecular data.
  • Surgical excision is the primary treatment, but local recurrence is frequent due to infiltrative growth.
  • Understanding CAF's multifaceted features is crucial for accurate diagnosis and effective management.