Non-ossifying fibroma: Difference between revisions
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{{Infobox medical condition (new) |
{{Infobox medical condition (new) |
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| name = Non-ossifying fibroma |
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| synonyms = '''Fibroxanthoma''' |
| synonyms = '''Fibroxanthoma''' |
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| image = NOF 1.jpg |
| image = NOF 1.jpg |
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<!-- Definition and symptoms --> |
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A '''non-ossifying fibroma''' (NOF) is a [[benign]] [[bone tumor]].<ref name=WHOblue2020>{{cite book|title=Soft Tissue and Bone Tumours: WHO Classification of Tumours|url=https://books.google.com/books?id=kaR9zQEACAAJ|year=2020|editor=WHO Classification of Tumours Editorial Board|publisher=International Agency for Research on Cancer|location=Lyon (France)|edition=5th|volume=3|isbn=978-92-832-4503-2|chapter=Non-ossifying fibroma| |
A '''non-ossifying fibroma''' ('''NOF''') is a [[benign]] [[bone tumor]] of the osteoclastic giant cell-rich tumor type.<ref name=WHOblue2020>{{cite book|title=Soft Tissue and Bone Tumours: WHO Classification of Tumours|url=https://books.google.com/books?id=kaR9zQEACAAJ|year=2020|editor=WHO Classification of Tumours Editorial Board|publisher=International Agency for Research on Cancer|location=Lyon (France)|edition=5th|volume=3|isbn=978-92-832-4503-2|chapter=Non-ossifying fibroma| |
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pages=447–448}}</ref> It generally occurs in the [[metaphysis]] of long bones in children and adolescents.<ref name="Santini2">{{cite book|last1=Murali|first1=Sundaram|url=https://books.google.com/books?id=qGmnCQAAQBAJ|title=Tumors and Tumor-Like Lesions of Bone: For Surgical Pathologists, Orthopedic Surgeons and Radiologists|last2=Ilaslan|first2=Hakan|last3=Holden|first3=Darlene M.|date=2015|publisher=Springer|isbn=978-1-4471-6577-4|editor-last=Santini-Araujo|editor-first=Eduardo|page=15|language=en|chapter=2. An imaging approach to bone tumors|quote=|editor-last2=Kalil|editor-first2=Ricardo K.|editor-last3=Bertoni|editor-first3=Franco|editor-last4=Park|editor-first4=Yong-Koo}}</ref> Typically, there are no symptoms unless there is a [[fracture]].<ref name="Santini2"/> 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 spot]]s, [[mental retardation]], [[hypogonadism]], eye and cardiovascular abnormalities.<ref name="Santini2"/> |
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However, it is controversial whether it represents a true [[neoplasm]] or rather a developmental disorder of growing bone. Radiographically, the tumor presents as a well marginated radiolucent lesion, with a distinct multilocular appearance. These foci consist of [[collagen]] rich [[connective tissue]], [[fibroblast]]s, [[histiocyte]]s and [[osteoclast]]s. They originate from the [[growth plate]], and are located in adjacent parts of the [[metaphysis]] and [[diaphysis]] of long bones, most often of the legs. No treatment is needed in asymptomatic patients and spontaneous remission with replacement by bone tissue is to be expected. |
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Diagnosis is by [[X-ray]] or [[MRI]], usually when investigating a person for something else.<ref name="Santini2"/> [[Medical imaging]] typically shows a well defined radiolucent lesion, with a distinct multilocular appearance, sometimes looking like bubbles.<ref name="Santini2"/> It is usually around 1–2 cm in size, but be as large as 7 cm.<ref name="Paulos2021">{{cite book |last1=Paulos |first1=Jaime |title=Bone Tumors: Diagnosis and Therapy Today |date=2021 |publisher=Springer |isbn=978-1-4471-7501-8 |pages=139 |url=https://link.springer.com/chapter/10.1007/978-1-4471-7501-8_22 |language=en |chapter=Non Ossifying Fibroma|doi=10.1007/978-1-4471-7501-8_22 |s2cid=238034517 }}</ref> They consist of foci consist of [[collagen]] rich [[connective tissue]], [[fibroblast]]s, [[histiocyte]]s and [[osteoclast]]s.<ref name="Santini2"/> Usually no treatment is required.<ref name=WHOblue2020/> Surgical curettage and bone grafting may be required if it is large.<ref name="Paulos2021"/> |
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Multiple nonossifying fibromas occur in [[Jaffe-Campanacci syndrome]] in combination with [[cafe-au-lait spot]]s, [[mental retardation]], [[hypogonadism]], ocular and cardiovascular abnormalities. |
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| ⚫ | It is found in 30–40% of children and adolescents, but rare in adults as most have resolved by this time.<ref name="Santini2"/> They do not become [[malignant]].<ref name="Santini2"/> It affects twice as many males as females.<ref name="Santini2"/> A NOF was identified on the mandible of [[Skhul and Qafzeh hominins#Qafzeh 9 and 10|Qafzeh 9]], an early anatomically modern human dated to 90–100 000 yrs B.P.<ref name=Coutinho>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</ref> |
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==Signs and symptoms== |
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Most people with non-ossifying fibroma have no symptoms.<ref name=WHOblue2020/> If the tumor is large, there may be pain over the affected area, a [[pathological fracture]], and the affected limb might not function properly.<ref name=WHOblue2020/> 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 spot]]s, [[mental retardation]], [[hypogonadism]], eye and cardiovascular abnormalities.<ref name="Santini2"/> |
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==Diagnosis== |
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It is usually diagnosed by [[x-ray]] or [[MRI]], when investigating another problem.<ref name=WHOblue2020/> The tumor presents as a well defined radiolucent lesion, with a distinct multilocular appearance, sometimes looking like a "soap bubble".<ref name="Ahn">{{cite web |last1=Ahn |first1=Leah |last2=O'Donnell |first2=Patrick |title=Non-Ossifying Fibroma - Pathology - Orthobullets |url=https://www.orthobullets.com/pathology/8027/non-ossifying-fibroma |website=www.orthobullets.com |access-date=3 August 2021}}</ref> If small and no symptoms, then biopsy is not needed.<ref name=WHOblue2020/> |
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==Additional images== |
==Additional images== |
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[[Category:Osteopathies]] |
[[Category:Osteopathies]] |
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{{musculoskeletal-disease-stub}} |
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Latest revision as of 09:42, 4 November 2023
| Non-ossifying fibroma | |
|---|---|
| Other names | Fibroxanthoma |
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| X-ray of nonossifying fibroma of distal tibia. | |
| Specialty | Rheumatology  |
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
[edit]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
[edit]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
[edit]
See also
[edit]References
[edit]- ^ a b c d e f WHO Classification of Tumours Editorial Board, ed. (2020). "Non-ossifying fibroma". Soft Tissue and Bone Tumours: WHO Classification of Tumours. Vol. 3 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 447–448. ISBN 978-92-832-4503-2.
- ^ a b c d e f g h i j Murali, Sundaram; Ilaslan, Hakan; Holden, Darlene M. (2015). "2. An imaging approach to bone tumors". In Santini-Araujo, Eduardo; Kalil, Ricardo K.; Bertoni, Franco; Park, Yong-Koo (eds.). Tumors and Tumor-Like Lesions of Bone: For Surgical Pathologists, Orthopedic Surgeons and Radiologists. Springer. p. 15. ISBN 978-1-4471-6577-4.
- ^ a b Paulos, Jaime (2021). "Non Ossifying Fibroma". Bone Tumors: Diagnosis and Therapy Today. Springer. p. 139. doi:10.1007/978-1-4471-7501-8_22. ISBN 978-1-4471-7501-8. S2CID 238034517.
- ^ 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
- ^ Ahn, Leah; O'Donnell, Patrick. "Non-Ossifying Fibroma - Pathology - Orthobullets". www.orthobullets.com. Retrieved 3 August 2021.
External links
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Media related to Nonossifying fibroma at Wikimedia Commons- Wheeless' Textbook of Orthopaedics: Nonossifying Fibroma
