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Desmoplastic fibroma

From Wikipedia, the free encyclopedia
Medical condition
Desmoplastic fibroma
SpecialtyOncology

Inmedicine, adesmoplastic fibroma is alow-grademalignant, locally aggressive, fibrous and rare tumor of the bone, affecting children and young adults, potentially resulting incortical bone destruction. It usually affects craniofacial bones, mandible most frequently, long bones (metaphyseal femur, tibia, humerus).[1] The World Health Organization, 2020, reclassified these tumors as specific benign tumors in the category offibroblastic and myofibroblastic tumors.[2]

Although it does not tend tometastatize, it has a high localrecurrence and infiltrative growth.[3] Treatment consists in wide local excision to prevent otherwise frequent recurrences.[4] The role ofradiotherapy andchemotherapy in this tumor still is unclear.[5]

Some cases have been described, in which anosteosarcoma has arisen from a desmoplastic fibroma.[6]

A famous occurrence of this particular form of the disease involved Italo-AustralianRiccardo Torresan in 2011, with 18 cm of femur needing to be removed with the now widely recognized method of "aggressive curettage" being employed.[7]

See also

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References

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  1. ^"Desmoplastic Firboma".
  2. ^Sbaraglia M, Bellan E, Dei Tos AP (April 2021)."The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives".Pathologica.113 (2):70–84.doi:10.32074/1591-951X-213.PMC 8167394.PMID 33179614.
  3. ^Schneider, M.; Zimmermann, A. C.; Depprich, R. A.; Kübler, N. R.; Engers, R.; Naujoks, C. D.; Handschel, J. (2009)."Desmoplastic fibroma of the mandible - review of the literature and presentation of a rare case".Head & Face Medicine.5: 25.doi:10.1186/1746-160X-5-25.PMC 2787487.PMID 19930688.
  4. ^"Desmoplastic fibroma".
  5. ^"Desmoplastic fibroma".
  6. ^Abdelwahab, Ibrahim Fikry; Klein, Michael J.; Hermann, George; Steiner, German C.; Yang, David C. (2002). "Osteosarcoma Arising in a Desmoplastic Fibroma of the Proximal Tibia".American Journal of Roentgenology.178 (3):613–615.doi:10.2214/ajr.178.3.1780613.PMID 11856684.
  7. ^"Desmoplastic Fibroma - BONETUMOR.ORG".

External links

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Classification
Not otherwise specified
Connective tissue neoplasm
Fibromatous
Fibroma/fibrosarcoma
Fibroma/fibromatosis
Histiocytoma/histiocytic sarcoma
Myxomatous
Fibroepithelial
Synovial-like
Lipomatous
Myomatous
General
Smooth muscle
Skeletal muscle
Complex mixed and stromal
Mesothelial


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