In
medicine
Medicine is the science and Praxis (process), practice of caring for patients, managing the Medical diagnosis, diagnosis, prognosis, Preventive medicine, prevention, therapy, treatment, Palliative care, palliation of their injury or disease, ...
, 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
A bone is a rigid organ that constitutes part of the skeleton in most vertebrate animals. Bones protect the various other organs of the body, produce red and white blood cells, store minerals, provide structure and support for the body, an ...
destruction. It usually affects craniofacial bones, mandible most frequently, long bones (metaphyseal femur, tibia, humerus). The World Health Organization, 2020, reclassified these tumors as specific benign tumors in the category of
fibroblastic and myofibroblastic tumors
Fibroblastic and myofibroblastic tumors (FMTs) are tumors which develop from the mesenchymal stem cells which differentiate into fibroblasts (the most common cell type in connective tissue) and/or the myocytes/ myoblasts that differentiate into ...
.
Although it does not tend to
metastatize, it has a high local
recurrence and infiltrative growth. Treatment consists in wide local excision to prevent otherwise frequent recurrences. The role of
radiotherapy
Radiation therapy or radiotherapy (RT, RTx, or XRT) is a treatment using ionizing radiation, generally provided as part of cancer therapy to either kill or control the growth of malignant cells. It is normally delivered by a linear particle ...
and
chemotherapy
Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
in this tumor still is unclear.
Some cases have been described, in which an
osteosarcoma
An osteosarcoma (OS) or osteogenic sarcoma (OGS) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchyme, mesenchymal origin (and thus a sarcoma) and that exhi ...
has arisen from a desmoplastic fibroma.
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.
See also
*
Desmoplasia
In medicine, desmoplasia is the growth of fibrous connective tissue. It is also called a desmoplastic reaction to emphasize that it is secondary to an insult (medical), insult. Desmoplasia may occur around a neoplasm, causing dense fibrosis arou ...
References
External links
Types of neoplasia
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