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==Overview==
==Overview==
Enchondromas arise from rests of growth plate [[cartilage]] or [[chondrocytes]] that are normally involved in the production and maintainence of the cartilaginous matrix, which consists mainly of [[collagen]] and [[proteoglycans]]. Genes involved in the pathogenesis of enchondromas and periostal chondromas include [[isocitrate dehydrogenase]] 1 (''IDH1'') and ''IDH2'' . On gross pathology, small (usually less than 3 cm), [[translucent]], grey blue, nodular lesions with occasional [[calcification]]s are characteristic findings of chondromas.
[[Enchondroma|Enchondromas]] arise from rests of growth plate [[cartilage]] or [[chondrocytes]] that are normally involved in the production and maintainence of the cartilaginous [[matrix]], which consists mainly of [[collagen]] and [[proteoglycans]]. Genes involved in the pathogenesis of enchondromas and periostal chondromas include [[isocitrate dehydrogenase]] 1 (''IDH1'') and ''IDH2'' . On gross pathology, small (usually less than 3 cm), [[translucent]], grey blue, [[nodular]] lesions with occasional [[calcification]]s are characteristic findings of chondromas. Enchondromas may be single or multiple. Multiple enchondromas are associated Maffucci syndrome, Ollier disease and metachondromatosis.


==Pathogenesis==
==Pathogenesis==
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*On microscopic histopathological analysis, characteristic findings of chondroma are as followings:<ref name="pmid27233920">{{cite journal |vauthors=Uhl M, Herget G, Kurz P |title=[Cartilage tumors : Pathology and radiomorphology] |language=German |journal=Radiologe |volume=56 |issue=6 |pages=476–88 |date=June 2016 |pmid=27233920 |doi=10.1007/s00117-016-0112-z |url=}}</ref><ref name="pmid8229872">{{cite journal |vauthors=Blum MR, Danford M, Speight PM |title=Soft tissue chondroma of the cheek |journal=J. Oral Pathol. Med. |volume=22 |issue=7 |pages=334–6 |date=August 1993 |pmid=8229872 |doi= |url=}}</ref>
*On microscopic histopathological analysis, characteristic findings of chondroma are as followings:<ref name="pmid27233920">{{cite journal |vauthors=Uhl M, Herget G, Kurz P |title=[Cartilage tumors : Pathology and radiomorphology] |language=German |journal=Radiologe |volume=56 |issue=6 |pages=476–88 |date=June 2016 |pmid=27233920 |doi=10.1007/s00117-016-0112-z |url=}}</ref><ref name="pmid8229872">{{cite journal |vauthors=Blum MR, Danford M, Speight PM |title=Soft tissue chondroma of the cheek |journal=J. Oral Pathol. Med. |volume=22 |issue=7 |pages=334–6 |date=August 1993 |pmid=8229872 |doi= |url=}}</ref>
**Cytologically benign cells is spaced nests
**Cytologically benign cells is spaced nests
**Not extending into surrounding soft tissue
**Not extending into surrounding [[soft tissue]]
**[[Avascular]], [[fibrous]] lobules of [[hyaline cartilage]]nous matrix
**[[Avascular]], [[fibrous]] lobules of [[hyaline cartilage]]nous matrix
**Interspersed with [[chondrocyte]]s and separated by normal marrow
**Interspersed with [[chondrocyte]]s and separated by normal marrow
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Maffucci syndrome  
Maffucci syndrome  
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| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
:Multiple enchondromas
:Multiple enchondromas
:Multiple hemangiomas
:Multiple hemangiomas
:Lymphangiomas
:Lymphangiomas
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| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
Sporadic
Sporadic
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|-
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |
Ollier disease
Ollier disease
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| style="padding: 5px 5px; background: #F5F5F5;" align="center" |
:Multiple enchondromas
:Multiple enchondromas
:Asymmetric dwarfism
:Asymmetric dwarfism
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Sporadic
Sporadic
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|-
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Metachondromatosis
Metachondromatosis
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:Multiple osteochondromas  
:Multiple osteochondromas  
:Multiple enchondromas
:Multiple enchondromas
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Genetic (Autosomal dominant)
Genetic (Autosomal dominant)
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Revision as of 15:33, 26 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Farima Kahe M.D. [2] Soujanya Thummathati, MBBS [3]

Overview

Enchondromas arise from rests of growth plate cartilage or chondrocytes that are normally involved in the production and maintainence of the cartilaginous matrix, which consists mainly of collagen and proteoglycans. Genes involved in the pathogenesis of enchondromas and periostal chondromas include isocitrate dehydrogenase 1 (IDH1) and IDH2 . On gross pathology, small (usually less than 3 cm), translucent, grey blue, nodular lesions with occasional calcifications are characteristic findings of chondromas. Enchondromas may be single or multiple. Multiple enchondromas are associated Maffucci syndrome, Ollier disease and metachondromatosis.

Pathogenesis

  • Enchondromas arise from rests of growth plate cartilage or chondrocytes that are normally involved in the production and maintainence of the cartilaginous matrix, which consists mainly of collagen and proteoglycans.[1]
  • Chondromas is the result of nests of growth-plate cartilage that have become entrapped in the medullary canal of the metaphysis or in the metaphyseal-diaphyseal junction. These hamartomatous proliferations persist as islands in the bone and then develop from enchondral ossification.[2][3]

Genetics

Gross Pathology

  • On gross pathology, small (usually less than 3 cm), translucent, grey blue, nodular lesions with occasional calcifications are characteristic findings of chondromas.[5][6]

Microscopic Pathology

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Associated conditions

Enchondromas may be single or multiple. Multiple enchondromas are associated with the following:[9][10][11]

Associated disease Findings Occurance

Maffucci syndrome

Multiple enchondromas
Multiple hemangiomas
Lymphangiomas

Sporadic

Ollier disease

Multiple enchondromas
Asymmetric dwarfism

Sporadic

Metachondromatosis

Multiple osteochondromas
Multiple enchondromas

Genetic (Autosomal dominant)

References

  1. Dal Cin P, Qi H, Sciot R, Van den Berghe H (February 1997). "Involvement of chromosomes 6 and 11 in a soft tissue chondroma". Cancer Genet. Cytogenet. 93 (2): 177–8. PMID 9078305.
  2. Geng S, Zhang J, Zhang LW, Wu Z, Jia G, Xiao X, Hao S (July 2014). "Diagnosis and microsurgical treatment of chondromas and chondrosarcomas of the cranial base". Oncol Lett. 8 (1): 301–304. doi:10.3892/ol.2014.2072. PMC 4063631. PMID 24959265.
  3. Lewis MM, Kenan S, Yabut SM, Norman A, Steiner G (July 1990). "Periosteal chondroma. A report of ten cases and review of the literature". Clin. Orthop. Relat. Res. (256): 185–92. PMID 2194723.
  4. Sandberg AA (July 2004). "Genetics of chondrosarcoma and related tumors". Curr Opin Oncol. 16 (4): 342–54. PMID 15187889.
  5. Semenova LA, Bulycheva IV (2007). "[Chondromas (enchondroma, periosteal chondroma, enchondromatosis)]". Arkh. Patol. (in Russian). 69 (5): 45–8. PMID 18074822.
  6. Chung EB, Enzinger FM (April 1978). "Chondroma of soft parts". Cancer. 41 (4): 1414–24. PMID 76505.
  7. Uhl M, Herget G, Kurz P (June 2016). "[Cartilage tumors : Pathology and radiomorphology]". Radiologe (in German). 56 (6): 476–88. doi:10.1007/s00117-016-0112-z. PMID 27233920.
  8. Blum MR, Danford M, Speight PM (August 1993). "Soft tissue chondroma of the cheek". J. Oral Pathol. Med. 22 (7): 334–6. PMID 8229872.
  9. Prokopchuk O, Andres S, Becker K, Holzapfel K, Hartmann D, Friess H (February 2016). "Maffucci syndrome and neoplasms: a case report and review of the literature". BMC Res Notes. 9: 126. doi:10.1186/s13104-016-1913-x. PMC 4769492. PMID 26920730.
  10. Mavrogenis AF, Skarpidi E, Papakonstantinou O, Papagelopoulos PJ (June 2010). "Chondrosarcoma in metachondromatosis: a case report". J Bone Joint Surg Am. 92 (6): 1507–13. doi:10.2106/JBJS.I.00693. PMID 20516327.
  11. Watanabe F, Saiki T, Ochochi Y (2012). "Extraskeletal chondroma of the preauricular region: a case report and literature review". Case Rep Med. 2012: 121743. doi:10.1155/2012/121743. PMC 3400396. PMID 22844293.


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