Non-ossifying fibroma

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Non-ossifying fibroma
Other namesFibroxanthoma
File:NOF 1.jpg
X-ray of nonossifying fibroma of distal tibia.

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, intellectual disabilities, 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

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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, intellectual disabilities, hypogonadism, eye and cardiovascular abnormalities.[2]

Diagnosis

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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

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File:MaturenonossifyingfibromaCT.PNG
An ossified non-ossifying fibroma on CT

See also

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References

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  1. ^ a b c d e f Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
  2. ^ a b c d e f g h i j Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
  3. ^ a b Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
  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. ^ Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
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  • Error creating thumbnail: File missing Media related to Lua error in Module:Commons_link at line 62: attempt to index field 'wikibase' (a nil value). at Wikimedia Commons
  • Wheeless' Textbook of Orthopaedics: Nonossifying Fibroma