Desmoplastic fibroma

Desmoplastic fibroma
a,b) Male with desmoplastic fibroma in first toe
SpecialtyOncology

In medicine, a desmoplastic fibroma is a benign, but 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).[1] The World Health Organization, 2020, reclassified these tumors as specific benign tumors in the category of fibroblastic and myofibroblastic tumors.[2]

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

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

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.[7]

See also

References

  1. ↑ "Desmoplastic Firboma". Archived from the original on 2021-09-22. Retrieved 2021-09-28.
  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". Archived from the original on 2018-10-13. Retrieved 2021-09-28.
  5. ↑ "Desmoplastic fibroma". Archived from the original on 2021-09-22. Retrieved 2021-09-28.
  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". Archived from the original on 2017-07-23. Retrieved 2021-09-28.
Classification

Giovanni

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