desmoplastic fibroma

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In medicine, a desmoplastic fibroma is a low-grade malignant, locally aggressive, fibrous and rare tumor of the bone, affecting children and young adults, potentially resulting in cortical bone destruction. It usually affects craniofacial bones, mandible most frequently, long bones (metaphyseal femur, tibia, humerus).{{cite web |title=Desmoplastic Firboma |url=http://www.tumorsurgery.org/tumor-education/bone-tumors/types-of-bone-tumors/desmoplastic-fibroma.aspx}} The World Health Organization, 2020, reclassified these tumors as specific benign tumors in the category of fibroblastic and myofibroblastic tumors.{{cite journal | vauthors = Sbaraglia M, Bellan E, Dei Tos AP | title = The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives | journal = Pathologica | volume = 113 | issue = 2 | pages = 70–84 | date = April 2021 | pmid = 33179614 | pmc = 8167394 | doi = 10.32074/1591-951X-213 | url = }}

Although it does not tend to metastatize, it has a high local recurrence and infiltrative growth.{{cite journal |title=Desmoplastic fibroma of the mandible - review of the literature and presentation of a rare case |journal=Head & Face Medicine |volume=5 |pages=25 |pmc=2787487 |year=2009 |last1=Schneider |first1=M. |last2=Zimmermann |first2=A. C. |last3=Depprich |first3=R. A. |last4=Kübler |first4=N. R. |last5=Engers |first5=R. |last6=Naujoks |first6=C. D. |last7=Handschel |first7=J. |pmid=19930688 |doi=10.1186/1746-160X-5-25 |doi-access=free }} Treatment consists in wide local excision to prevent otherwise frequent recurrences.{{cite web |title=Desmoplastic fibroma |url=http://www.pathologyoutlines.com/topic/bonedesmoplasticfibroma.html}} The role of radiotherapy and chemotherapy in this tumor still is unclear.{{cite web |title=Desmoplastic fibroma |url=http://www.tumorsurgery.org/tumor-education/bone-tumors/types-of-bone-tumors/desmoplastic-fibroma.aspx}}

Some cases have been described, in which an osteosarcoma has arisen from a desmoplastic fibroma.{{cite journal |title=Osteosarcoma Arising in a Desmoplastic Fibroma of the Proximal Tibia |journal=American Journal of Roentgenology|volume=178|issue=3|pages=613–615|doi=10.2214/ajr.178.3.1780613|pmid=11856684|year=2002 |last1=Abdelwahab |first1=Ibrahim Fikry |last2=Klein|first2=Michael J.|last3=Hermann|first3=George|last4=Steiner|first4=German C.|last5=Yang|first5=David C.}}

A famous occurrence of this particular form of the disease involved Italo-Australian Riccardo Torresan in 2011, with 18 cm of femur needing to be removed with the now widely recognized method of "aggressive curettage" being employed.{{cite web|url=http://www.bonetumor.org/tumors-fibrous-tissue/desmoplastic-fibroma|title=Desmoplastic Fibroma - BONETUMOR.ORG}}