Difference between revisions of "Chondromyxoid fibroma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Bone ChondromyxoidFibroma MP2 CTR.jpg
| Width      =
| Caption    = Chondromyxoid fibroma. [[H&E stain]].
| Synonyms  =
| Micro      = spindle cells or stellate cells in a myxoid or chondroid stroma, lobules with hypocellular centers and hypercellular peripheries, +/-[[giant cells]] in the hypercellular periphery, scattered calcifications, no true hyaline cartilage formation, no mitotic activity
| Subtypes  =
| LMDDx      = [[chondroblastoma]], [[chondrosarcoma]], [[phosphaturic mesenchymal tumour]] (case report)
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[bone]] ([[metaphysis]]) - see ''[[bone tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx = teenager/young adult
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Chondromyxoid fibroma''' is a rare benign [[Chondro-osseous tumours|chondro-osseous tumour]] typically found in the [[metaphysis]] of teenagers or young adults.
'''Chondromyxoid fibroma''' is a rare benign [[Chondro-osseous tumours|chondro-osseous tumour]] typically found in the [[metaphysis]] of teenagers or young adults.


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*Metaphyseal lesion - classic location.<ref name=pmid18312923>{{Cite journal  | last1 = Budny | first1 = AM. | last2 = Ismail | first2 = A. | last3 = Osher | first3 = L. | title = Chondromyxoid fibroma. | journal = J Foot Ankle Surg | volume = 47 | issue = 2 | pages = 153-9 | month =  | year =  | doi = 10.1053/j.jfas.2007.08.013 | PMID = 18312923 }}</ref>
*Metaphyseal lesion - classic location.<ref name=pmid18312923>{{Cite journal  | last1 = Budny | first1 = AM. | last2 = Ismail | first2 = A. | last3 = Osher | first3 = L. | title = Chondromyxoid fibroma. | journal = J Foot Ankle Surg | volume = 47 | issue = 2 | pages = 153-9 | month =  | year =  | doi = 10.1053/j.jfas.2007.08.013 | PMID = 18312923 }}</ref>
*Well-circumscribed.
*Well-circumscribed.
*Fragments of white-grey rubbery tissue
*Fragments of white-grey rubbery tissue.


==Microscopic==
==Microscopic==
Line 14: Line 45:
*Spindle cells or stellate cells in a myxoid or chondroid stroma.
*Spindle cells or stellate cells in a myxoid or chondroid stroma.
*Lobules with hypocellular centers and hypercellular peripheries.   
*Lobules with hypocellular centers and hypercellular peripheries.   
*Tumor lacks a true hyaline cartilage matrix
*[[Giant cells]] in the hypercellular periphery.
*Lack of mitotic activity
*Scattered calcifications.
*No true hyaline cartilage formation.
*No mitotic activity.
 
==IHC==
SOX9 positive


DDx:
DDx:
*[[Chondroblastoma]].
**Likewise has immature cartilage but (1) [[epiphysis|epiphyseal]] location, (2) chickenwire-like calcifications.
*[[Chondrosarcoma]].
**Different age group.
**Mature hyaline [[cartilage]] formation.
**Tumour permeation of the surrounding bone.
**Mitotic activity.
*[[Phosphaturic mesenchymal tumour]] - case report.<ref>{{Cite journal  | last1 = Suryawanshi | first1 = P. | last2 = Agarwal | first2 = M. | last3 = Dhake | first3 = R. | last4 = Desai | first4 = S. | last5 = Rekhi | first5 = B. | last6 = Reddy | first6 = KB. | last7 = Jambhekar | first7 = NA. | title = Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance. | journal = Skeletal Radiol | volume = 40 | issue = 11 | pages = 1481-5 | month = Nov | year = 2011 | doi = 10.1007/s00256-011-1159-6 | PMID = 21533894 }}</ref>
*[[Phosphaturic mesenchymal tumour]] - case report.<ref>{{Cite journal  | last1 = Suryawanshi | first1 = P. | last2 = Agarwal | first2 = M. | last3 = Dhake | first3 = R. | last4 = Desai | first4 = S. | last5 = Rekhi | first5 = B. | last6 = Reddy | first6 = KB. | last7 = Jambhekar | first7 = NA. | title = Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance. | journal = Skeletal Radiol | volume = 40 | issue = 11 | pages = 1481-5 | month = Nov | year = 2011 | doi = 10.1007/s00256-011-1159-6 | PMID = 21533894 }}</ref>
*Chondroblastoma
 
**Also produces immature cartilage but
===Images===
**Epiphyseal location
<gallery>
**Chicken-wire calcifications
Image:Bone ChondromyxoidFibroma HP2 PA (2).jpg|Stellate cells in a myxoid stroma. (SKB)
*Chondrosarcoma
Image:Bone ChondromyxoidFibroma LP CTR.jpg|Lobule with calcification. (SKB)
**Mature hyaline cartilage formation
Image:Bone ChondromyxoidFibroma MP CTR.jpg|Stellate cells in a myxohyaline stroma. (SKB)
**Tumor permeation of the surrounding bone
Image:Bone ChondromyxoidFibroma MP2 CTR.jpg|Stellate cells in a myxoid stroma. (SKB)
**Mitotic activity
Image:Bone ChondromyxoidFibroma MP2 PA.jpg|Lobule with a darker (hypercellular) periphery and lighter (hypocellular) center. (SKB)
Images:
Image:Bone ChondromyxoidFibroma LP2 CTR.jpg|Stellate cells in a myxohyaline stroma. (SKB)
Image:Bone ChondromyxoidFibroma MP4 CTR.jpg|Stellate cells in a myxohyaline stroma. (SKB)
Image:Bone ChondromyxoidFibroma MP3 CTR.jpg|Stellate cells in a myxoid stroma. (SKB)
Image:Bone ChondromyxoidFibroma HP2 PA.JPG|Stellate cells in a myxoid stroma. (SKB)
Image:Bone ChondromyxoidFibroma LP PA.jpg|Low power view displaying the lobular nature of these proliferations. (SKB)
Image:Bone ChondromyxoidFibroma Calcium MP PA.JPG|Focus of amorphous calcification. (SKB)
Image:Bone ChondromyxoidFibroma MP3 PA.JPG|Foci of amorphous calcification. (SKB)
</gallery>
www:
*[http://www.webpathology.com/image.asp?n=7&Case=331 Chondromyxoid fibroma - low mag. (webpathology.com)].
*[http://www.webpathology.com/image.asp?n=7&Case=331 Chondromyxoid fibroma - low mag. (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=331&n=8 Chondromyxoid fibroma - high mag. (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=331&n=8 Chondromyxoid fibroma - high mag. (webpathology.com)].
*Tumor Library [http://www.tumorlibrary.com/case/images/1490.jpg].
*Tumor Library [http://www.tumorlibrary.com/case/images/1491.jpg].
*Tumor Library [http://www.tumorlibrary.com/case/images/3618.jpg].
*Tumor Library [http://www.tumorlibrary.com/case/images/3619.jpg].
*Tumor Library [http://www.tumorlibrary.com/case/images/3621.jpg].
*Tumor Library [http://www.tumorlibrary.com/case/images/3623.jpg].
*Diagnostic Pathology [http://www.diagnosticpathology.org/content/figures/1746-1596-2-44-4.jpg].


==Molecular==
==Molecular==
*Activating rearrangements of GRM1 (metabotropic glutamate receptor 1)<ref>{{Cite journal  | last1 = Nord | first1 = KH. | last2 = Lilljebjörn | first2 = H. | last3 = Vezzi | first3 = F. | last4 = Nilsson | first4 = J. | last5 = Magnusson | first5 = L. | last6 = Tayebwa | first6 = J. | last7 = de Jong | first7 = D. | last8 = Bovée | first8 = JV. | last9 = Hogendoorn | first9 = PC. | title = GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma. | journal = Nat Genet | volume = 46 | issue = 5 | pages = 474-7 | month = May | year = 2014 | doi = 10.1038/ng.2927 | PMID = 24658000 }}<ref>
*Activating rearrangements of GRM1 (metabotropic glutamate receptor 1).<ref name=pmid24658000>{{Cite journal  | last1 = Nord | first1 = KH. | last2 = Lilljebjörn | first2 = H. | last3 = Vezzi | first3 = F. | last4 = Nilsson | first4 = J. | last5 = Magnusson | first5 = L. | last6 = Tayebwa | first6 = J. | last7 = de Jong | first7 = D. | last8 = Bovée | first8 = JV. | last9 = Hogendoorn | first9 = PC. | title = GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma. | journal = Nat Genet | volume = 46 | issue = 5 | pages = 474-7 | month = May | year = 2014 | doi = 10.1038/ng.2927 | PMID = 24658000 }}</ref>
 
==See also==
==See also==
*[[Chondro-osseous tumours]].
*[[Chondro-osseous tumours]].
Line 38: Line 97:
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
==External links==
*[http://njms2.umdnj.edu/tutorweb/case4.htm UMDNJ Tutor Web - Case (umdnj.edu)].
*[http://www.pathologyoutlines.com/topic/bonechondromyxoidfibroma.html Chondromyxoid fibroma (pathologyoutlines.com)].


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Chondro-osseous tumours]]
[[Category:Chondro-osseous tumours]]

Latest revision as of 07:51, 20 November 2014

Chondromyxoid fibroma
Diagnosis in short

Chondromyxoid fibroma. H&E stain.
LM spindle cells or stellate cells in a myxoid or chondroid stroma, lobules with hypocellular centers and hypercellular peripheries, +/-giant cells in the hypercellular periphery, scattered calcifications, no true hyaline cartilage formation, no mitotic activity
LM DDx chondroblastoma, chondrosarcoma, phosphaturic mesenchymal tumour (case report)
Site bone (metaphysis) - see bone tumours
Clinical history teenager/young adult
Prevalence uncommon
Prognosis benign

Chondromyxoid fibroma is a rare benign chondro-osseous tumour typically found in the metaphysis of teenagers or young adults.

General

  • Uncommon and benign.[1]
  • Teenagers or young adults.

Gross

  • Metaphyseal lesion - classic location.[2]
  • Well-circumscribed.
  • Fragments of white-grey rubbery tissue.

Microscopic

Features:[3]

  • Spindle cells or stellate cells in a myxoid or chondroid stroma.
  • Lobules with hypocellular centers and hypercellular peripheries.
  • Giant cells in the hypercellular periphery.
  • Scattered calcifications.
  • No true hyaline cartilage formation.
  • No mitotic activity.

IHC

SOX9 positive

DDx:

Images

  • Stellate cells in a myxoid stroma. (SKB)

  • Lobule with calcification. (SKB)

  • Stellate cells in a myxohyaline stroma. (SKB)

  • Stellate cells in a myxoid stroma. (SKB)

  • Lobule with a darker (hypercellular) periphery and lighter (hypocellular) center. (SKB)

  • Stellate cells in a myxohyaline stroma. (SKB)

  • Stellate cells in a myxohyaline stroma. (SKB)

  • Stellate cells in a myxoid stroma. (SKB)

  • Stellate cells in a myxoid stroma. (SKB)

  • Low power view displaying the lobular nature of these proliferations. (SKB)

  • Focus of amorphous calcification. (SKB)

  • Foci of amorphous calcification. (SKB)

www:

Molecular

  • Activating rearrangements of GRM1 (metabotropic glutamate receptor 1).[5]

See also

References

  1. ↑ Bhamra, JS.; Al-Khateeb, H.; Dhinsa, BS.; Gikas, PD.; Tirabosco, R.; Pollock, RC.; Skinner, JA.; Aston, WJ. et al. (2014). "Chondromyxoid fibroma management: a single institution experience of 22 cases.". World J Surg Oncol 12: 283. doi:10.1186/1477-7819-12-283. PMID 25217119.
  2. ↑ Budny, AM.; Ismail, A.; Osher, L.. "Chondromyxoid fibroma.". J Foot Ankle Surg 47 (2): 153-9. doi:10.1053/j.jfas.2007.08.013. PMID 18312923.
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 642. ISBN 978-0781765275.
  4. ↑ Suryawanshi, P.; Agarwal, M.; Dhake, R.; Desai, S.; Rekhi, B.; Reddy, KB.; Jambhekar, NA. (Nov 2011). "Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance.". Skeletal Radiol 40 (11): 1481-5. doi:10.1007/s00256-011-1159-6. PMID 21533894.
  5. ↑ Nord, KH.; Lilljebjörn, H.; Vezzi, F.; Nilsson, J.; Magnusson, L.; Tayebwa, J.; de Jong, D.; Bovée, JV. et al. (May 2014). "GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma.". Nat Genet 46 (5): 474-7. doi:10.1038/ng.2927. PMID 24658000.

External links

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