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Calcifying aponeurotic fibroma

Disease definition

A rare, superficial fibromatosis characterized by non-malignant, locally invading, fibrosing tumour of differentiated fibroblasts, slowly growing subcutaneously, occurring predominantly distally on the extremities, especially the hands and feet. Histologic examination shows a multinodular pattern with large areas of calcification and fibrosis, and the presence of elongated spindle cells with hyperchromatic plump vesicular nuclei interspersed within fine bands of collagen.

ORPHA:199260

Classification level: Disorder
  • Synonym(s):
    • Juvenile aponeurotic fibromatosis
    • Keasby tumor
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: M72.8
  • ICD-11: EE61
  • OMIM: -
  • UMLS: C0553647
  • MeSH: -
  • GARD: -
  • MedDRA: -

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