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__NOTOC__
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{{SI}}
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{{CMG}}
{{CMG}}; {{AE}} {{Rohan}} {{Hudakarman}}


{{SK}} Fibroxanthoma; Fibrous cortical defect
{{SK}} Fibroxanthoma; Fibrous cortical defect; NOF


==Overview==
==Overview==
Non ossifying fibromas are present in about 30 % of children. The [[incidence]] of Non ossifying fibroma is approximately 1000-2000 per 100,000 individuals worldwide. The age distribution of Non ossifying fibroma is between 5-15 years. Men are more commonly affected than women, with a 1.9:1 ratio. In 1929, Phemister first described the term non ossifying fibroma. The exact [[pathogenesis]] of non ossifying fibroma is not fully understood. Non ossifying fibroma (NOF) typically occur in the [[metaphysis]] of the [[long bones]]. The [[bones]] often involved are [[femur]], [[tibia]], and [[fibula]]. The majority of patients with non ossifying fibroma are [[asymptomatic]]. Some patients with non ossifying fibroma have a complaints of [[Pain|pain,]] [[swelling]] and [[pathological]] [[fracture]]. [[X-ray]] is the [[diagnostic]] study of choice for the [[diagnosis]] of non ossifying fibroma demonstrating soap bubble like [[Lytic|lytic lesion]]. Observation is the mainstay of treatment for non ossifying fibroma. [[Surgery]] in form of [[curettage]] and [[bone grafting]] is reserved for cases with high risk of [[pathological]] [[Bone fracture|fracture]].


*Non ossifying fibroma is a benign fibroblastic mass. It typically arises in the metaphyses of long bones, particularly the distal femur and tibia. It is common, possibly affecting more than 40% of boys and 30% of girls.It is thought to originate at the insertion site of a ligament or tendon and it has been suggested that it may reflect a previous traction injury. Lesions are usually asymptomatic, although they are occasionally associated with pathological fractures.
==Historical Perspective==
*In 1929, Phemister first described the term non ossifying fibroma.<ref>{{Cite journal| author = [[H. L. Jaffe]] & [[L. Lichtenstein]]| title = Non-osteogenic fibroma of bone| journal = [[The American journal of pathology]]| volume = 18| issue = 2| pages = 205–221| year = 1942| month = March| pmid = 19970624}}</ref><ref>{{cite book | last = Peabody | first = Terrance | title = Orthopaedic oncology : primary and metastatic tumors of the skeletal system | publisher = Springer | location = Cham | year = 2014 | isbn = 9783319073224 }}</ref>
*In 1941, Sontag and Pyle reported a [[Radiological|radiologic]] description of non ossifying fibroma.
*In 1942, Jaffe and Lichtenstein described [[clinical findings]], [[anatomic]] aspects and the [[Natural history of disease|natural history]].
 
==Classification==
Non ossifying fibroma can be classified based on [[imaging]] findings.
 
===Enneking (MSTS) Staging System===
*The Enneking surgical staging system (also known as the MSTS system) for benign [[Musculoskeletal system|musculoskeletal]] [[Tumor|tumors]] based on [[radiographic]] characteristics of the [[tumor]] host margin.<ref name="pmid20333492">{{cite journal| author=Jawad MU, Scully SP| title=In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system. | journal=Clin Orthop Relat Res | year= 2010 | volume= 468 | issue= 7 | pages= 2000-2 | pmid=20333492 | doi=10.1007/s11999-010-1315-7 | pmc=2882012 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20333492  }} </ref>
*It is widely accepted and routinely used [[classification]].
 
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px" align="center"
| valign="top" |
|-
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Stages}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Description}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |1
| style="padding: 5px 5px; background: #F5F5F5;" | Latent: Well demarcated borders
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |2
| style="padding: 5px 5px; background: #F5F5F5;" | Active: Indistinct borders
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |3
| style="padding: 5px 5px; background: #F5F5F5;" | Aggressive: Indistinct borders
|}
 
==Pathophysiology==
*The exact [[pathogenesis]] of non ossifying fibroma is not fully understood.
*Various theories have been proposed concerning the [[pathogenesis]] of non ossifying fibroma<ref>{{cite journal |vauthors=Herget GW, Mauer D, Krauß T, El Tayeh A, Uhl M, Südkamp NP, Hauschild O |title=Non-ossifying fibroma: natural history with an emphasis on a stage-related growth, fracture risk and the need for follow-up |journal=BMC Musculoskelet Disord |volume=17 |issue= |pages=147 |date=April 2016 |pmid=27044378 |pmc=4820930 |doi=10.1186/s12891-016-1004-0 |url=}}</ref><ref>{{cite journal |vauthors=Goldin A, Muzykewicz DA, Dwek J, Mubarak SJ |title=The aetiology of the non-ossifying fibroma of the distal femur and its relationship to the surrounding soft tissues |journal=J Child Orthop |volume=11 |issue=5 |pages=373–379 |date=October 2017 |pmid=29081852 |pmc=5643931 |doi=10.1302/1863-2548.11.170068 |url=}}</ref>
**An abnormal development extending from the [[growth plate]].
**An abnormal [[Osteoclast|osteoclastic]] [[resorption]] at the [[subperiosteal]] level during remodeling of the [[metaphysis]].
*Non ossifying fibroma (NOF) typically occur in the [[metaphysis]] of the [[long bones]].<ref>{{cite journal |vauthors=Mankin HJ, Trahan CA, Fondren G, Mankin CJ |title=Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: a biologic and clinical review |journal=Chir Organi Mov |volume=93 |issue=1 |pages=1–7 |date=May 2009 |pmid=19711155 |doi=10.1007/s12306-009-0016-4 |url=}}</ref>
*The [[bones]] often involved are [[femur]], [[tibia]], and [[fibula]].<ref>{{cite journal |vauthors=CUNNINGHAM JB, ACKERMAN LV |title=Metaphyseal fibrous defects |journal=J Bone Joint Surg Am |volume=38-A |issue=4 |pages=797–808 |date=July 1956 |pmid=13331975 |doi= |url=}}</ref>
*Conditions associated:
**[[Jaffe-Campanacci syndrome]]
***[[Congenital syndromes|Congenital syndrome]] of multiple non-ossifying fibromas and [[Café au lait spot|cafe au lait]] [[pigmentation]], [[mental retardation]], [[heart]], [[eyes]] and [[gonads]] ([[hypogonadism]]) involved
**[[Neurofibromatosis]]
**[[Familial]] multifocal NOF
**[[Aneurysmal bone cyst]]
 
==Causes==
 
* There are no established [[causes]] for non ossifying fibroma.<ref>{{cite journal |vauthors=Hatcher CH |title=The Pathogenesis of Localized Fibrous Lesions in the Metaphyses of Long Bones |journal=Ann. Surg. |volume=122 |issue=6 |pages=1016–30 |date=December 1945 |pmid=17858695 |pmc=1618342 |doi= |url=}}</ref>
 
==Differentiating Non Ossifying Fibroma from Other Diseases==
Non ossifying fibroma must be differentiated from following [[bone]] disorders:
{|
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease
! style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Bubbly lytic lesion on x-ray'''
! style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Lakes of Blood on histology'''
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Treatment is curretage and bone grafting
|-
! style="background:#DCDCDC;" align="center" + |Non ossifying fibroma
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | -
|-
! style="background:#DCDCDC;" align="center" + |[[Simple bone cyst|Unicameral bone cyst]]
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | -
|-
! style="background:#DCDCDC;" align="center" + |[[Aneurysmal bone cyst]]
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | +
|-
! style="background:#DCDCDC;" align="center" + |[[Giant cell tumor of bone|Giant cell tumor]]
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[Biopsy]]
| style="background:#F5F5F5;" align="center" + | +
|-
! style="background:#DCDCDC;" align="center" + |[[Chondroblastoma]]
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Biopsy]]
| style="background:#F5F5F5;" align="center" + | +
|-
! style="background:#DCDCDC;" align="center" + |Chondromyxoid Fibroma
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | +
|-
! style="background:#DCDCDC;" align="center" + |[[Osteoblastoma]]
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | +
|-
! style="background:#DCDCDC;" align="center" + |Telangiectatic [[osteosarcoma]]
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
| style="background:#F5F5F5;" align="center" + | -
|}
 
==Epidemiology and Demographics==
*The [[incidence]] of Non ossifying fibroma is approximately 1000 - 2000 per 100,000 individuals worldwide.<ref>{{cite journal |vauthors=Freyschmidt J, Ostertag H, Saure D |title=[Fibrous metaphyseal defect (fibrous cortical defect, non-ossifying fibroma). Paper II: differential diagnosis (author's transl)] |language=German |journal=Rofo |volume=134 |issue=4 |pages=392–400 |date=April 1981 |pmid=6453054 |doi=10.1055/s-2008-1056377 |url=}}</ref>
*[[Adolescent|Adolescents]] and [[children]] are most affected by non ossifying fibroma.
*Non ossifying fibromas are present in about 30 % of [[children]].<ref>Nielsen GP, Kyriakos M. Fibrohistiocytic tumours. In: Fletcher CDM, Bridge J, Hogendorn PCW, Mertens F, editors. WHO Classifications of tumours of bone and soft Tissue. Lyon: IARC Press; 2013. pp. 301–4</ref>
*The [[age]] distribution of Non ossifying fibroma is between 5-15 years.
*[[Men]] are more commonly affected than [[women]], with a 1.9:1 ratio.<ref>{{cite journal |vauthors=Ritschl P, Lintner F, Pechmann U, Brand G |title=Fibrous metaphyseal defect |journal=Int Orthop |volume=14 |issue=2 |pages=205–11 |date=1990 |pmid=2115506 |doi= |url=}}</ref>
*There is no [[racial]] predilection to non ossifying fibroma.
==Risk Factors==
 
* There are no established [[risk factors]] for non ossifying fibroma.
 
==Screening==
 
* There is insufficient evidence to recommend routine screening for non ossifying fibroma.
 
==Natural History, Complications, and Prognosis==
*Common [[complications]] of [[Aneurysmal disease|aneurysmal]] [[bone cyst]] include:<ref>{{cite journal |vauthors=Wodajo FM |title=Top five lesions that do not need referral to orthopedic oncology |journal=Orthop. Clin. North Am. |volume=46 |issue=2 |pages=303–14 |date=April 2015 |pmid=25771324 |doi=10.1016/j.ocl.2014.11.012 |url=}}</ref>
**[[Pathological]] [[Bone fracture|fracture]]
**Premature [[Epiphyseal plate|epiphyseal]] closure
***Limb-length discrepancy
***Angular [[deformity]]
** Rare [[malignant transformation]]
*[[Prognosis]] is generally excellent for non ossifying fibroma.
*Factors that influence the outcome of the non ossifying fibroma include:
**Young age
**Open growth plates
**[[Metaphysis|Metaphyseal]] location


==Diagnosis==
==Diagnosis==
===Chest X-ray===
===Diagnostic Study of Choice===
{| align="right"
|
[[File:NOF xrays.gif|300px|thumb|X ray of Non ossifying fibroma in proximal humerus.[https://radiopaedia.org/cases/non-ossifying-fibroma-4?lang=us Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463]]]
|}
 
*[[X-rays|X-ray]] is the diagnostic study of choice for the diagnosis of non ossifying fibroma.
*[[X-rays|X-ray]] findings include:<ref>{{cite journal |vauthors=Ritschl P, Karnel F, Hajek P |title=Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study |journal=Skeletal Radiol. |volume=17 |issue=1 |pages=8–15 |date=1988 |pmid=3358140 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Drennan DB, Maylahn DJ, Fahey JJ |title=Fractures through large non-ossifying fibromas |journal=Clin. Orthop. Relat. Res. |volume= |issue=103 |pages=82–8 |date=1974 |pmid=4413505 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Ritschl P, Karnel F, Hajek P |title=Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study |journal=Skeletal Radiol. |volume=17 |issue=1 |pages=8–15 |date=1988 |pmid=3358140 |doi= |url=}}</ref>
**[[Metaphyseal]] eccentric "bubbly" [[lytic]] [[lesion]] surrounded by sclerotic rim
**[[Cortex]] may be expanded and thin
**As bone grows, it migrates to [[diaphysis]] and the lesions enlarge.
**Lesions become [[Sclerotic ring|sclerotic]] as patient approaches [[skeletal]] maturity.
 
===History and Symptoms===
*The majority of patients with non ossifying fibroma are [[asymptomatic]].<ref>{{cite book | last = Peabody | first = Terrance | title = Orthopaedic oncology : primary and metastatic tumors of the skeletal system | publisher = Springer | location = Cham | year = 2014 | isbn = 9783319073224 }}</ref>
*Some [[Patient|patients]] with non ossifying fibroma have a positive [[History and Physical examination|history]] of:
**[[Pain]]
**[[Swelling]]
**[[Pathological]] [[fracture]]
 
===Physical Examination===
*Patients with non ossifying fibroma usually appear well.
*Common [[physical examination]] findings of non ossifying fibroma include:<ref>{{cite journal |vauthors=Mallet JF, Rigault P, Padovani JP, Touzet P, Nezelof C |title=[Non-ossifying fibroma in children: a surgical condition?] |language=French |journal=Chir Pediatr |volume=21 |issue=3 |pages=179–89 |date=1980 |pmid=7408072 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Fauré C, Laurent JM, Schmit P, Sirinelli D |title=Multiple and large non-ossifying fibromas in children with neurofibromatosis |journal=Ann Radiol (Paris) |volume=29 |issue=3-4 |pages=369–73 |date=1986 |pmid=3092720 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Hetts SW, Hilchey SD, Wilson R, Franc B |title=Case 110: Nonossifying fibroma |journal=Radiology |volume=243 |issue=1 |pages=288–92 |date=April 2007 |pmid=17392261 |doi=10.1148/radiol.2431040427 |url=}}</ref>
**[[Asymptomatic]]
**[[Pain]] if associated with [[pathological]] [[fracture]]
*Findings associated with [[Jaffe-Campanacci syndrome]] include:<ref>{{cite journal |vauthors=Cherix S, Bildé Y, Becce F, Letovanec I, Rüdiger HA |title=Multiple non-ossifying fibromas as a cause of pathological femoral fracture in Jaffe-Campanacci syndrome |journal=BMC Musculoskelet Disord |volume=15 |issue= |pages=218 |date=June 2014 |pmid=24965055 |pmc=4088300 |doi=10.1186/1471-2474-15-218 |url=}}</ref>
**[[Café-au-lait spot|Café-au-lait spots]]
**Multiple [[nevi]]
**[[Hypogonadism]]
**[[Ocular]] abnormalities


* Eccentric lucent lesion with thinned cortex, which may have a multilocular appearance and often a sclerotic margin.
===Laboratory Findings===
* The lesions spontaneously regress with time and radiographs will show increasing marginal sclerosis followed by progressive ossification of the lesion extending from its diaphyseal aspect.
* The appearance on plain films is characteristic and no further imaging is indicated.
<gallery>
Image:


Nonossifying fibroma 001.jpg
* There are no [[diagnostic]] [[laboratory]] findings associated with non ossifying fibroma.


Image:
===Electrocardiogram===


Nonossifying fibroma 002.jpg
* There are no [[ECG]] findings associated with non ossifying fibroma.


Image:
===X-ray===
*Three views of affected [[bone]] or [[joint]] are recommended.
*[[X-ray]] findings include:<ref>{{cite journal |vauthors=Ritschl P, Karnel F, Hajek P |title=Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study |journal=Skeletal Radiol. |volume=17 |issue=1 |pages=8–15 |date=1988 |pmid=3358140 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Drennan DB, Maylahn DJ, Fahey JJ |title=Fractures through large non-ossifying fibromas |journal=Clin. Orthop. Relat. Res. |volume= |issue=103 |pages=82–8 |date=1974 |pmid=4413505 |doi= |url=}}</ref><ref>{{cite journal |vauthors=Ritschl P, Karnel F, Hajek P |title=Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study |journal=Skeletal Radiol. |volume=17 |issue=1 |pages=8–15 |date=1988 |pmid=3358140 |doi= |url=}}</ref>
**[[Metaphyseal]] eccentric "bubbly" [[lytic]] lesion surrounded by sclerotic rim
**[[Cortex]] may be expanded and thin
**As [[bone]] grows, it migrates to [[diaphysis]] and the [[lesions]] enlarge.
**Lesions become [[Sclerotic ring|sclerotic]] as patient approaches [[skeletal]] maturity.
{| align="right"
|
[[File:NOF CT GIF.gif|300px|thumb|CT of non ossifying fibroma in distal femur.[https://radiopaedia.org/cases/non-ossifying-fibroma-5?lang=us Source: Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 14173]]]
|}


Nonossifying fibroma 003.jpg
===Echocardiography or Ultrasound===


Image:
* There are no [[echocardiography]]/[[ultrasound]]  findings associated with non ossifying fibroma.


Nonossifying fibroma 004.jpg
===CT scan===
*[[Computed tomography]] (CT) is useful in predicting the [[fracture]] risk.<ref>{{cite journal |vauthors=Huzjan R, Vukelic-Markovic M, Brkljacic B, Ivanac G |title=The value of ultrasound in diagnosis and follow-up of fibrous cortical defect |journal=Ultraschall Med |volume=26 |issue=5 |pages=420–3 |date=October 2005 |pmid=16240255 |doi=10.1055/s-2005-857887 |url=}}</ref><ref>{{cite journal |vauthors=Loberant N, Samovsky M, Papura S |title=Gray-scale and Doppler characteristics of fibrous cortical defects in a child |journal=J Clin Ultrasound |volume=31 |issue=7 |pages=369–74 |date=September 2003 |pmid=12923882 |doi=10.1002/jcu.10188 |url=}}</ref><ref>{{cite journal |vauthors=von Falck C, Rosenthal H, Gratz KF, Galanski M |title=Nonossifying fibroma can mimic residual lymphoma in FDG PET: additional value of combined PET/CT |journal=Clin Nucl Med |volume=32 |issue=8 |pages=640–2 |date=August 2007 |pmid=17667441 |doi=10.1097/RLU.0b013e3180a1ad09 |url=}}</ref>
*[[CT scans]] may depict a central lucency.
*[[CT-scans|CT scan]] may confirm a minimally displaced [[fracture]].
*It may also be helpful in [[Preoperative assessment|preoperative]] planning in unusual locations such as the [[Femur neck|femoral neck]].


</gallery>
===MRI===
*[[Magnetic resonance imaging]] (MRI) are similar to those from [[CT scan]].<ref>{{cite book | last = Peabody | first = Terrance | title = Orthopaedic oncology : primary and metastatic tumors of the skeletal system | publisher = Springer | location = Cham | year = 2014 | isbn = 9783319073224 }}</ref>
*[[MRI]] demonstrates a characteristic low signal on both [[T1]] and T2 sequences without enhancement.
*High signal on T2 can be seen with an associated [[stress fracture]].


===Atypical Fibroxanthoma===
===Other Imaging Findings===
<gallery widths=200px>


* There are no other [[imaging]] findings associated with non ossifying fibroma.
{| align="right"
|
[[File:NOF MRI GIF.gif|300px|thumb|MRI of non ossifying fibroma in proximal humerus.[https://radiopaedia.org/cases/non-ossifying-fibroma-4?lang=us Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463]]]
|}


8289.jpg | Atypical Fibroxanthoma <br> [http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=504 <font size="-2">''Used with permission of Dermatology Atlas'</font>]
===Other Diagnostic Studies===
NF8293.jpg | Atypical Fibroxanthoma <br> [http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=504 <font size="-2">''Used with permission of Dermatology Atlas'</font>]
'''Biopsy'''
*[[Biopsy]] finding of non ossifying fibroma includes:<ref>{{cite journal |vauthors=Hoeffel C, Panuel M, Plenat F, Mainard L, Hoeffel JC |title=Pathological fracture in non-ossifying fibroma with histological features simulating aneurysmal bone cyst |journal=Eur Radiol |volume=9 |issue=4 |pages=669–71 |date=1999 |pmid=10354882 |doi=10.1007/s003300050730 |url=}}</ref>
**Bland [[fibroblastic]] component with a few [[histiocytes]], [[myofibroblast]] cells, and [[giant cells]].
**Marked [[proliferation]]<nowiki/>s of [[spindle cells]] arranged in a storiform pattern.
**[[Hemosiderin]] deposits also are found.
**Some [[leukocyte]] [[Infiltration (medical)|infiltration]] may be present.


==Treatment==
===Medical Therapy===
*[[Observation]] is the mainstay of treatment for non ossifying fibroma.<ref>{{cite journal |vauthors=Hoeffel C, Panuel M, Plenat F, Mainard L, Hoeffel JC |title=Pathological fracture in non-ossifying fibroma with histological features simulating aneurysmal bone cyst |journal=Eur Radiol |volume=9 |issue=4 |pages=669–71 |date=1999 |pmid=10354882 |doi=10.1007/s003300050730 |url=}}</ref>
==Observation==   
'''Indications'''
*First line of treatment
*Most [[lesions]] resolve spontaneously and progressively re-ossify as [[child]] enters 2nd and 3rd decade of life


</gallery>
'''Technique'''
*[[Radiograph|Radiographs]] at 6, 12months, then annually until reossified


===Casting===
'''Indication'''
*[[Pathological|Pathologic]] [[fracture]] especially in the [[pediatric]] population.
===Surgery===
'''Indication'''
*[[Unstable]] [[fractures]]
*High risk of [[Pathological|pathologic]] [[fracture]]
'''Technique'''
*The [[surgical]] approach involves exposing the [[fracture]] site and developing a [[Cortical bone|cortical]] window to [[curette]] the [[tumor]].<ref>{{cite journal |vauthors=Herget GW, Mauer D, Krauß T, El Tayeh A, Uhl M, Südkamp NP, Hauschild O |title=Non-ossifying fibroma: natural history with an emphasis on a stage-related growth, fracture risk and the need for follow-up |journal=BMC Musculoskelet Disord |volume=17 |issue= |pages=147 |date=April 2016 |pmid=27044378 |pmc=4820930 |doi=10.1186/s12891-016-1004-0 |url=}}</ref><ref>{{cite journal |vauthors=Andreacchio A, Alberghina F, Testa G, Canavese F |title=Surgical treatment for symptomatic non-ossifying fibromas of the lower extremity with calcium sulfate grafts in skeletally immature patients |journal=Eur J Orthop Surg Traumatol |volume=28 |issue=2 |pages=291–297 |date=February 2018 |pmid=28819829 |doi=10.1007/s00590-017-2028-3 |url=}}</ref>
===Primary Prevention===
* There are no established measures for the [[primary prevention]] of non ossifying fibroma.
===Secondary Prevention===
* There are no established measures for the [[secondary prevention]] of non ossifying fibroma.


==References==
==References==
{{reflist|2}}
{{reflist|2}}


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Latest revision as of 20:55, 9 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2] Huda A. Karman, M.D.

Synonyms and keywords: Fibroxanthoma; Fibrous cortical defect; NOF

Overview

Non ossifying fibromas are present in about 30 % of children. The incidence of Non ossifying fibroma is approximately 1000-2000 per 100,000 individuals worldwide. The age distribution of Non ossifying fibroma is between 5-15 years. Men are more commonly affected than women, with a 1.9:1 ratio. In 1929, Phemister first described the term non ossifying fibroma. The exact pathogenesis of non ossifying fibroma is not fully understood. Non ossifying fibroma (NOF) typically occur in the metaphysis of the long bones. The bones often involved are femur, tibia, and fibula. The majority of patients with non ossifying fibroma are asymptomatic. Some patients with non ossifying fibroma have a complaints of pain, swelling and pathological fracture. X-ray is the diagnostic study of choice for the diagnosis of non ossifying fibroma demonstrating soap bubble like lytic lesion. Observation is the mainstay of treatment for non ossifying fibroma. Surgery in form of curettage and bone grafting is reserved for cases with high risk of pathological fracture.

Historical Perspective

Classification

Non ossifying fibroma can be classified based on imaging findings.

Enneking (MSTS) Staging System

Stages Description
1 Latent: Well demarcated borders
2 Active: Indistinct borders
3 Aggressive: Indistinct borders

Pathophysiology

Causes

  • There are no established causes for non ossifying fibroma.[8]

Differentiating Non Ossifying Fibroma from Other Diseases

Non ossifying fibroma must be differentiated from following bone disorders:

Disease Bubbly lytic lesion on x-ray Lakes of Blood on histology Diagnosis Treatment is curretage and bone grafting
Non ossifying fibroma + - Radiology and biopsy -
Unicameral bone cyst + - Radiology and biopsy -
Aneurysmal bone cyst + + Radiology and biopsy +
Giant cell tumor - - Radiology and Biopsy +
Chondroblastoma - - Biopsy +
Chondromyxoid Fibroma - - Radiology and biopsy +
Osteoblastoma - - Radiology and biopsy +
Telangiectatic osteosarcoma - + Radiology and biopsy -

Epidemiology and Demographics

  • The incidence of Non ossifying fibroma is approximately 1000 - 2000 per 100,000 individuals worldwide.[9]
  • Adolescents and children are most affected by non ossifying fibroma.
  • Non ossifying fibromas are present in about 30 % of children.[10]
  • The age distribution of Non ossifying fibroma is between 5-15 years.
  • Men are more commonly affected than women, with a 1.9:1 ratio.[11]
  • There is no racial predilection to non ossifying fibroma.

Risk Factors

  • There are no established risk factors for non ossifying fibroma.

Screening

  • There is insufficient evidence to recommend routine screening for non ossifying fibroma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

X ray of Non ossifying fibroma in proximal humerus.Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with non ossifying fibroma.

X-ray

CT of non ossifying fibroma in distal femur.Source: Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 14173

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

  • There are no other imaging findings associated with non ossifying fibroma.
MRI of non ossifying fibroma in proximal humerus.Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463

Other Diagnostic Studies

Biopsy

Treatment

Medical Therapy

Observation

Indications

  • First line of treatment
  • Most lesions resolve spontaneously and progressively re-ossify as child enters 2nd and 3rd decade of life

Technique

  • Radiographs at 6, 12months, then annually until reossified

Casting

Indication

Surgery

Indication

Technique

Primary Prevention

Secondary Prevention

References

  1. ↑ H. L. Jaffe & L. Lichtenstein (1942). "Non-osteogenic fibroma of bone". The American journal of pathology. 18 (2): 205–221. PMID 19970624. Unknown parameter |month= ignored (help)
  2. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  3. ↑ Jawad MU, Scully SP (2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin Orthop Relat Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
  4. ↑ Herget GW, Mauer D, Krauß T, El Tayeh A, Uhl M, Südkamp NP, Hauschild O (April 2016). "Non-ossifying fibroma: natural history with an emphasis on a stage-related growth, fracture risk and the need for follow-up". BMC Musculoskelet Disord. 17: 147. doi:10.1186/s12891-016-1004-0. PMC 4820930. PMID 27044378.
  5. ↑ Goldin A, Muzykewicz DA, Dwek J, Mubarak SJ (October 2017). "The aetiology of the non-ossifying fibroma of the distal femur and its relationship to the surrounding soft tissues". J Child Orthop. 11 (5): 373–379. doi:10.1302/1863-2548.11.170068. PMC 5643931. PMID 29081852.
  6. ↑ Mankin HJ, Trahan CA, Fondren G, Mankin CJ (May 2009). "Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: a biologic and clinical review". Chir Organi Mov. 93 (1): 1–7. doi:10.1007/s12306-009-0016-4. PMID 19711155.
  7. ↑ CUNNINGHAM JB, ACKERMAN LV (July 1956). "Metaphyseal fibrous defects". J Bone Joint Surg Am. 38-A (4): 797–808. PMID 13331975.
  8. ↑ Hatcher CH (December 1945). "The Pathogenesis of Localized Fibrous Lesions in the Metaphyses of Long Bones". Ann. Surg. 122 (6): 1016–30. PMC 1618342. PMID 17858695.
  9. ↑ Freyschmidt J, Ostertag H, Saure D (April 1981). "[Fibrous metaphyseal defect (fibrous cortical defect, non-ossifying fibroma). Paper II: differential diagnosis (author's transl)]". Rofo (in German). 134 (4): 392–400. doi:10.1055/s-2008-1056377. PMID 6453054.
  10. ↑ Nielsen GP, Kyriakos M. Fibrohistiocytic tumours. In: Fletcher CDM, Bridge J, Hogendorn PCW, Mertens F, editors. WHO Classifications of tumours of bone and soft Tissue. Lyon: IARC Press; 2013. pp. 301–4
  11. ↑ Ritschl P, Lintner F, Pechmann U, Brand G (1990). "Fibrous metaphyseal defect". Int Orthop. 14 (2): 205–11. PMID 2115506.
  12. ↑ Wodajo FM (April 2015). "Top five lesions that do not need referral to orthopedic oncology". Orthop. Clin. North Am. 46 (2): 303–14. doi:10.1016/j.ocl.2014.11.012. PMID 25771324.
  13. ↑ Ritschl P, Karnel F, Hajek P (1988). "Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study". Skeletal Radiol. 17 (1): 8–15. PMID 3358140.
  14. ↑ Drennan DB, Maylahn DJ, Fahey JJ (1974). "Fractures through large non-ossifying fibromas". Clin. Orthop. Relat. Res. (103): 82–8. PMID 4413505.
  15. ↑ Ritschl P, Karnel F, Hajek P (1988). "Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study". Skeletal Radiol. 17 (1): 8–15. PMID 3358140.
  16. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  17. ↑ Mallet JF, Rigault P, Padovani JP, Touzet P, Nezelof C (1980). "[Non-ossifying fibroma in children: a surgical condition?]". Chir Pediatr (in French). 21 (3): 179–89. PMID 7408072.
  18. ↑ Fauré C, Laurent JM, Schmit P, Sirinelli D (1986). "Multiple and large non-ossifying fibromas in children with neurofibromatosis". Ann Radiol (Paris). 29 (3–4): 369–73. PMID 3092720.
  19. ↑ Hetts SW, Hilchey SD, Wilson R, Franc B (April 2007). "Case 110: Nonossifying fibroma". Radiology. 243 (1): 288–92. doi:10.1148/radiol.2431040427. PMID 17392261.
  20. ↑ Cherix S, Bildé Y, Becce F, Letovanec I, Rüdiger HA (June 2014). "Multiple non-ossifying fibromas as a cause of pathological femoral fracture in Jaffe-Campanacci syndrome". BMC Musculoskelet Disord. 15: 218. doi:10.1186/1471-2474-15-218. PMC 4088300. PMID 24965055.
  21. ↑ Ritschl P, Karnel F, Hajek P (1988). "Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study". Skeletal Radiol. 17 (1): 8–15. PMID 3358140.
  22. ↑ Drennan DB, Maylahn DJ, Fahey JJ (1974). "Fractures through large non-ossifying fibromas". Clin. Orthop. Relat. Res. (103): 82–8. PMID 4413505.
  23. ↑ Ritschl P, Karnel F, Hajek P (1988). "Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study". Skeletal Radiol. 17 (1): 8–15. PMID 3358140.
  24. ↑ Huzjan R, Vukelic-Markovic M, Brkljacic B, Ivanac G (October 2005). "The value of ultrasound in diagnosis and follow-up of fibrous cortical defect". Ultraschall Med. 26 (5): 420–3. doi:10.1055/s-2005-857887. PMID 16240255.
  25. ↑ Loberant N, Samovsky M, Papura S (September 2003). "Gray-scale and Doppler characteristics of fibrous cortical defects in a child". J Clin Ultrasound. 31 (7): 369–74. doi:10.1002/jcu.10188. PMID 12923882.
  26. ↑ von Falck C, Rosenthal H, Gratz KF, Galanski M (August 2007). "Nonossifying fibroma can mimic residual lymphoma in FDG PET: additional value of combined PET/CT". Clin Nucl Med. 32 (8): 640–2. doi:10.1097/RLU.0b013e3180a1ad09. PMID 17667441.
  27. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  28. ↑ Hoeffel C, Panuel M, Plenat F, Mainard L, Hoeffel JC (1999). "Pathological fracture in non-ossifying fibroma with histological features simulating aneurysmal bone cyst". Eur Radiol. 9 (4): 669–71. doi:10.1007/s003300050730. PMID 10354882.
  29. ↑ Hoeffel C, Panuel M, Plenat F, Mainard L, Hoeffel JC (1999). "Pathological fracture in non-ossifying fibroma with histological features simulating aneurysmal bone cyst". Eur Radiol. 9 (4): 669–71. doi:10.1007/s003300050730. PMID 10354882.
  30. ↑ Herget GW, Mauer D, Krauß T, El Tayeh A, Uhl M, Südkamp NP, Hauschild O (April 2016). "Non-ossifying fibroma: natural history with an emphasis on a stage-related growth, fracture risk and the need for follow-up". BMC Musculoskelet Disord. 17: 147. doi:10.1186/s12891-016-1004-0. PMC 4820930. PMID 27044378.
  31. ↑ Andreacchio A, Alberghina F, Testa G, Canavese F (February 2018). "Surgical treatment for symptomatic non-ossifying fibromas of the lower extremity with calcium sulfate grafts in skeletally immature patients". Eur J Orthop Surg Traumatol. 28 (2): 291–297. doi:10.1007/s00590-017-2028-3. PMID 28819829.


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