Nonossifying fibroma: Difference between revisions

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==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 radiologic description of non ossifying fibroma.
*In 1942, Jaffe and Lichtenstein described clinical findings, anatomic aspects and the natural history.


The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
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].
 
OR
 
[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].
 
OR
 
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
 
OR
 
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].
 
OR
 
The staging of [malignancy name] is based on the [staging system].


OR
===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.


There is no established system for the staging of [malignancy name].
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==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 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 syndrome of multiple non-ossifying fibromas and cafe au lait pigmentation, mental retardation, heart, eyes, gonads 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].
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>
 
OR
 
Common causes of [disease] include [cause1], [cause2], and [cause3].
 
OR
 
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
 
OR
 
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
 
==Differentiating ((Page name)) from Other Diseases==
[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].
 
OR
 
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].


==Epidemiology and Demographics==
==Epidemiology and Demographics==
Line 155: Line 109:


==Risk Factors==
==Risk Factors==
There are no established risk factors for [disease name].
There are no established risk factors for non ossifying fibroma.
 
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].
 
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].
There is insufficient evidence to recommend routine screening for non ossifying fibroma.
 
OR
 
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==
Line 193: Line 127:
==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].
*X-ray is the diagnostic study of choice for the diagnosis of non ossifying fibroma.
 
*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
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
**As bone grows, it migrates to diaphysis and the lesions enlarge.
 
**Lesions become sclerotic as patient approaches skeletal maturity.  
OR
 
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
There are no established criteria for the diagnosis of [disease name].


===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 patients with non ossifying fibroma have a positive 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 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].
There are no diagnostic laboratory findings associated with non ossifying fibroma.
 
OR
 
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
 
OR
 
[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].
There are no ECG findings associated with non ossifying fibroma.
 
OR
 
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 as patient approaches skeletal maturity.  
OR
 
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].


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound  findings associated with [disease name].
There are no echocardiography/ultrasound  findings associated with non ossifying fibroma.
 
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].
 
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 may confirm a minimally displaced fracture.  
 
*It may also be helpful in preoperative planning in unusual locations such as the 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].
There are no other imaging findings associated with non ossifying fibroma.
 
OR
 
[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].


===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 proliferations 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 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 reossify as child enters 2nd and 3rd decade of life


OR
'''Technique'''
*Radiographs at 6, 12months, then annually until reossified


Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
===Casting===
 
'''Indication'''
OR
*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 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.
 
OR


Surgery is the mainstay of treatment for [disease or malignancy].
'''Technique'''
*The surgical approach involves exposing the fracture site and developing a 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===
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].
There are no established measures for the primary prevention of non ossifying fibroma.
 
OR
 
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].
There are no established measures for the secondary prevention of non ossifying fibroma.
 
OR
 
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].


==References==
==References==

Revision as of 21:03, 17 January 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]

Synonyms and keywords: Fibroxanthoma; Fibrous cortical defect

Overview

Historical Perspective

  • In 1929, Phemister first described the term non ossifying fibroma.[1][2]
  • In 1941, Sontag and Pyle reported a radiologic description of non ossifying fibroma.
  • In 1942, Jaffe and Lichtenstein described clinical findings, anatomic aspects and the 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 tumors based on radiographic characteristics of the tumor host margin.[3]
  • It is widely accepted and routinely used classification.
Stages Description
1 Latent: Well demarcated borders
2 Active: Indistinct borders
3 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:[4][5]
    • An abnormal development extending from the growth plate.
    • An abnormal osteoclastic resorption at the subperiosteal level during remodeling of the metaphysis.
  • Non ossifying fibroma (NOF) typically occur in the metaphysis of the long bones.[6]
  • The bones often involved are femur, tibia, and fibula.[7]
  • Conditions associated:
    • Jaffe-Campanacci syndrome
      • Congenital syndrome of multiple non-ossifying fibromas and cafe au lait pigmentation, mental retardation, heart, eyes, gonads involved
    • Neurofibromatosis
    • Familial multifocal NOF
    • Aneurysmal bone cyst

Causes

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

Epidemiology and Demographics

The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.

OR

In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

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

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

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

  • X-ray is the diagnostic study of choice for the diagnosis of non ossifying fibroma.
  • X-ray findings include:[9][10][11]
    • 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 as patient approaches skeletal maturity.

History and Symptoms

  • The majority of patients with non ossifying fibroma are asymptomatic.[12]
  • Some patients with non ossifying fibroma have a positive 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:[13][14][15]
    • Asymptomatic
    • Pain if associated with pathological fracture
  • Findings associated with Jaffe-Campanacci syndrome include:[16]
    • Café-au-lait spots
    • Multiple nevi
    • Hypogonadism
    • Ocular abnormalities

Laboratory Findings

There are no diagnostic laboratory findings associated with non ossifying fibroma.

Electrocardiogram

There are no ECG findings associated with non ossifying fibroma.

X-ray

  • Three views of affected bone or joint are recommended.
  • X-ray findings include:[17][18][19]
    • 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 as patient approaches skeletal maturity.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with non ossifying fibroma.

CT scan

  • Computed tomography (CT) is useful in predicting the fracture risk.[20][21][22]
  • CT scans may depict a central lucency.
  • CT may confirm a minimally displaced fracture.
  • It may also be helpful in preoperative planning in unusual locations such as the femoral neck.

MRI

  • Magnetic resonance imaging (MRI) are similar to those from CT scan.[23]
  • 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.

Other Imaging Findings

There are no other imaging findings associated with non ossifying fibroma.

Other Diagnostic Studies

Biopsy

  • Biopsy finding of non ossifying fibroma includes:[24]
    • Bland fibroblastic component with a few histiocytes, myofibroblast cells, and giant cells.
    • Marked proliferations of spindle cells arranged in a storiform pattern.
    • Hemosiderin deposits also are found.
    • Some leukocyte infiltration may be present.

Treatment

Medical Therapy

  • Observation is the mainstay of treatment for non ossifying fibroma.[25]

Observation

Indications

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

Technique

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

Casting

Indication

  • Pathologic fracture especially in the pediatric population.

Surgery

Indication

  • Unstable fractures
  • High risk of pathologic fracture

Technique

  • The surgical approach involves exposing the fracture site and developing a cortical window to curette the tumor.[26][27]

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

  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. ↑ 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.
  10. ↑ Drennan DB, Maylahn DJ, Fahey JJ (1974). "Fractures through large non-ossifying fibromas". Clin. Orthop. Relat. Res. (103): 82–8. PMID 4413505.
  11. ↑ 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.
  12. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ 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.
  18. ↑ Drennan DB, Maylahn DJ, Fahey JJ (1974). "Fractures through large non-ossifying fibromas". Clin. Orthop. Relat. Res. (103): 82–8. PMID 4413505.
  19. ↑ 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.
  20. ↑ 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.
  21. ↑ 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.
  22. ↑ 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.
  23. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  24. ↑ 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.
  25. ↑ 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.
  26. ↑ 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.
  27. ↑ 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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