Non-ossifying fibroma explained

Non-ossifying fibroma
Synonyms:Fibroxanthoma

A non-ossifying fibroma (NOF) is a benign bone tumor of the osteoclastic giant cell-rich tumor type.[1] It generally occurs in the metaphysis of long bones in children and adolescents.[2] Typically, there are no symptoms unless there is a fracture.[2] It can occur as part of a syndrome such as when multiple non-ossifying fibromas occur in neurofibromatosis, or Jaffe–Campanacci syndrome in combination with cafe-au-lait spots, mental retardation, hypogonadism, eye and cardiovascular abnormalities.[2]

Diagnosis is by X-ray or MRI, usually when investigating a person for something else.[2] Medical imaging typically shows a well defined radiolucent lesion, with a distinct multilocular appearance, sometimes looking like bubbles.[2] It is usually around 1–2 cm in size, but be as large as 7 cm.[3] They consist of foci consist of collagen rich connective tissue, fibroblasts, histiocytes and osteoclasts.[2] Usually no treatment is required.[1] Surgical curettage and bone grafting may be required if it is large.[3]

It is found in 30–40% of children and adolescents, but rare in adults as most have resolved by this time.[2] They do not become malignant.[2] It affects twice as many males as females.[2] A NOF was identified on the mandible of Qafzeh 9, an early anatomically modern human dated to 90–100 000 yrs B.P.[4]

Signs and symptoms

Most people with non-ossifying fibroma have no symptoms.[1] If the tumor is large, there may be pain over the affected area, a pathological fracture, and the affected limb might not function properly.[1] It can occur as part of a syndrome such as when multiple non-ossifying fibromas occur in neurofibromatosis, or Jaffe–Campanacci syndrome in combination with cafe-au-lait spots, mental retardation, hypogonadism, eye and cardiovascular abnormalities.[2]

Diagnosis

It is usually diagnosed by x-ray or MRI, when investigating another problem.[1] The tumor presents as a well defined radiolucent lesion, with a distinct multilocular appearance, sometimes looking like a "soap bubble".[5] If small and no symptoms, then biopsy is not needed.[1]

Additional images

See also

External links

Notes and References

  1. Book: Soft Tissue and Bone Tumours: WHO Classification of Tumours. 2020. WHO Classification of Tumours Editorial Board. International Agency for Research on Cancer. Lyon (France). 5th. 3. 978-92-832-4503-2. Non-ossifying fibroma. 447–448.
  2. Book: Murali. Sundaram. Tumors and Tumor-Like Lesions of Bone: For Surgical Pathologists, Orthopedic Surgeons and Radiologists. Ilaslan. Hakan. Holden. Darlene M.. 2015. Springer. 978-1-4471-6577-4. Santini-Araujo. Eduardo. 15. en. 2. An imaging approach to bone tumors. Kalil. Ricardo K.. Bertoni. Franco. Park. Yong-Koo.
  3. Book: Paulos . Jaime . Bone Tumors: Diagnosis and Therapy Today . 2021 . Springer . 978-1-4471-7501-8 . 139 . en . Non Ossifying Fibroma. 10.1007/978-1-4471-7501-8_22 . 238034517 .
  4. Coutinho Nogueira D, Dutour O, Coqueugniot H, Tillier A.-m., (2019) Qafzeh 9 mandible (ca 90–100 kyrs BP, Israel) revisited : μ-CT and 3D reveal new pathological conditions, International Journal of Paleopathology, Vol 26, pp.104-110, https://doi.org/10.1016/j.ijpp.2019.06.002
  5. Web site: Ahn . Leah . O'Donnell . Patrick . Non-Ossifying Fibroma - Pathology - Orthobullets . www.orthobullets.com . 3 August 2021.