Nonossifying fibroma: Difference between revisions

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__NOTOC__
__NOTOC__
{{SI}}
{{SI}}
{{CMG}}; AE {{Rohan}}
{{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]].


==Historical Perspective==
==Historical Perspective==
[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].
*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.
The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
*In 1942, Jaffe and Lichtenstein described [[clinical findings]], [[anatomic]] aspects and the [[Natural history of disease|natural history]].
 
In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
 
In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
 
There have been several outbreaks of [disease name], including -----.
 
In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].


==Classification==
==Classification==
There is no established system for the classification of [disease name].
Non ossifying fibroma can be classified based on [[imaging]] findings.
 
OR


[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
===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]].


OR
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px" align="center"
 
| valign="top" |
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
|-
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Stages}}
 
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Description}}
OR
|-
 
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |1
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
| style="padding: 5px 5px; background: #F5F5F5;" | Latent: Well demarcated borders
 
|-
OR
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |2
 
| style="padding: 5px 5px; background: #F5F5F5;" | Active: Indistinct borders
If the staging system involves specific and characteristic findings and features:
|-
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |3
 
| style="padding: 5px 5px; background: #F5F5F5;" | Aggressive: Indistinct borders
OR
|}
 
The staging of [malignancy name] is based on the [staging system].
 
OR
 
There is no established system for the staging of [malignancy name].


==Pathophysiology==
==Pathophysiology==
The exact pathogenesis of [disease name] is not fully understood.
*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>
OR
**An abnormal development extending from the [[growth plate]].
 
**An abnormal [[Osteoclast|osteoclastic]] [[resorption]] at the [[subperiosteal]] level during remodeling of the [[metaphysis]].
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
*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>
OR
*Conditions associated:
 
**[[Jaffe-Campanacci syndrome]]
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
***[[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]]
OR
**[[Familial]] multifocal NOF
 
**[[Aneurysmal bone cyst]]
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
 
OR
 
 
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
 
OR
 
The progression to [disease name] usually involves the [molecular pathway].
 
OR
 
The pathophysiology of [disease/malignancy] depends on the histological subtype.


==Causes==
==Causes==
Disease name] may be caused by [cause1], [cause2], or [cause3].


OR
* 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>


Common causes of [disease] include [cause1], [cause2], and [cause3].
==Differentiating Non Ossifying Fibroma from Other Diseases==
 
Non ossifying fibroma must be differentiated from following [[bone]] disorders:
OR
{|
 
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
! 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'''
OR
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
 
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Treatment is curretage and bone grafting
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
|-
 
! style="background:#DCDCDC;" align="center" + |Non ossifying fibroma
==Differentiating ((Page name)) from Other Diseases==
| style="background:#F5F5F5;" align="center" + | +
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
| style="background:#F5F5F5;" align="center" + | -
 
| style="background:#F5F5F5;" align="center" + |[[Radiology]] and [[biopsy]]
OR
| style="background:#F5F5F5;" align="center" + | -
 
|-
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
! 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==
==Epidemiology and Demographics==
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
*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.
OR
*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.
In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
*[[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.
OR
 
In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
 
 
 
Patients of all age groups may develop [disease name].
 
OR
 
The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
 
OR
 
[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
 
OR
 
[Chronic disease name] is usually first diagnosed among [age group].
 
OR
 
[Acute disease name] commonly affects [age group].
 
 
 
There is no racial predilection to [disease name].
 
OR
 
[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
 
 
 
[Disease name] affects men and women equally.
 
OR
 
[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
 
 
 
The majority of [disease name] cases are reported in [geographical region].
 
OR
 
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
 
==Risk Factors==
==Risk Factors==
There are no established risk factors for [disease name].
OR


The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
* There are no established [[risk factors]] for non ossifying fibroma.
 
OR
 
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
 
OR
 
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.


==Screening==
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].


OR
* There is insufficient evidence to recommend routine screening for non ossifying fibroma.
 
According to the [guideline name], screening for [disease name] is not recommended.
 
OR
 
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*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]]
OR
**Premature [[Epiphyseal plate|epiphyseal]] closure
 
***Limb-length discrepancy
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
***Angular [[deformity]]
 
** Rare [[malignant transformation]]
OR
*[[Prognosis]] is generally excellent for non ossifying fibroma.
 
*Factors that influence the outcome of the non ossifying fibroma include:
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
**Young age
**Open growth plates
**[[Metaphysis|Metaphyseal]] location


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
{| align="right"
 
|
OR
[[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]]]
 
|}
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
 
OR


The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
*[[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>
OR
**[[Metaphyseal]] eccentric "bubbly" [[lytic]] [[lesion]] surrounded by sclerotic rim
 
**[[Cortex]] may be expanded and thin
There are no established criteria for the diagnosis of [disease name].
**As bone grows, it migrates to [[diaphysis]] and the lesions enlarge.
**Lesions become [[Sclerotic ring|sclerotic]] as patient approaches [[skeletal]] maturity.


===History and Symptoms===
===History and Symptoms===
The majority of patients with [disease name] are asymptomatic.
*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:
OR
**[[Pain]]
 
**[[Swelling]]
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
**[[Pathological]] [[fracture]]


===Physical Examination===
===Physical Examination===
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
*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>
OR
**[[Asymptomatic]]
 
**[[Pain]] if associated with [[pathological]] [[fracture]]
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
*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]]
OR
**Multiple [[nevi]]
 
**[[Hypogonadism]]  
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
**[[Ocular]] abnormalities
 
OR
 
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].


===Laboratory Findings===
===Laboratory Findings===
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
OR
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].


OR
* There are no [[diagnostic]] [[laboratory]] findings associated with non ossifying fibroma.
 
[Test] is usually normal among patients with [disease name].
 
OR
 
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
 
OR
 
There are no diagnostic laboratory findings associated with [disease name].


===Electrocardiogram===
===Electrocardiogram===
There are no ECG findings associated with [disease name].


OR
* There are no [[ECG]] findings associated with non ossifying fibroma.
 
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].


===X-ray===
===X-ray===
There are no x-ray findings associated with [disease name].
*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>
OR
**[[Metaphyseal]] eccentric "bubbly" [[lytic]] lesion surrounded by sclerotic rim
 
**[[Cortex]] may be expanded and thin
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
**As [[bone]] grows, it migrates to [[diaphysis]] and the [[lesions]] enlarge.
 
**Lesions become [[Sclerotic ring|sclerotic]] as patient approaches [[skeletal]] maturity.
OR
{| align="right"
 
|
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
[[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]]]
|}


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound  findings associated with [disease name].
OR


Echocardiography/ultrasound  may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
* There are no [[echocardiography]]/[[ultrasound]]  findings associated with non ossifying fibroma.
 
OR
 
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound  may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===CT scan===
===CT scan===
There are no CT scan findings associated with [disease name].
*[[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.  
OR
*[[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]].
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===MRI===
===MRI===
There are no MRI findings associated with [disease name].
*[[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.
OR
*High signal on T2 can be seen with an associated [[stress fracture]].
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Other Imaging Findings===
===Other Imaging Findings===
There are no other imaging findings associated with [disease name].


OR
* There are no other [[imaging]] findings associated with non ossifying fibroma.
 
{| align="right"
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
|
[[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]]]
|}


===Other Diagnostic Studies===
===Other Diagnostic Studies===
There are no other diagnostic studies associated with [disease name].
'''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>
OR
**Bland [[fibroblastic]] component with a few [[histiocytes]], [[myofibroblast]] cells, and [[giant cells]].
 
**Marked [[proliferation]]<nowiki/>s of [[spindle cells]] arranged in a storiform pattern.
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
**[[Hemosiderin]] deposits also are found.
 
**Some [[leukocyte]] [[Infiltration (medical)|infiltration]] may be present.
OR
 
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].


==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*[[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


OR
'''Technique'''
*[[Radiograph|Radiographs]] at 6, 12months, then annually until reossified


Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
===Casting===
 
'''Indication'''
OR
*[[Pathological|Pathologic]] [[fracture]] especially in the [[pediatric]] population.
 
The majority of cases of [disease name] are self-limited and require only supportive care.
 
OR
 
[Disease name] is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR
 
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].


===Surgery===
===Surgery===
Surgical intervention is not recommended for the management of [disease name].
'''Indication'''
 
*[[Unstable]] [[fractures]]
OR
*High risk of [[Pathological|pathologic]] [[fracture]]
 
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
 
OR
 
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
 
OR


The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
'''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>
OR
 
Surgery is the mainstay of treatment for [disease or malignancy].


===Primary Prevention===
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].


OR
* There are no established measures for the [[primary prevention]] of non ossifying fibroma.
 
There are no available vaccines against [disease name].
 
OR
 
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
OR
 
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].


===Secondary Prevention===
===Secondary Prevention===
There are no established measures for the secondary prevention of [disease name].
OR


Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
* There are no established measures for the [[secondary prevention]] of non ossifying fibroma.


==References==
==References==

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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