Chondroma pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
[[Enchondroma|Enchondromas]] arise from rests of growth plate [[cartilage]] or [[chondrocytes]] that are normally involved in the production and | [[Enchondroma|Enchondromas]] arise from rests of [[growth plate]] [[cartilage]] or [[chondrocytes]] that are normally involved in the production and maintenance of the [[Cartilage|cartilaginous]] [[matrix]], which consists mainly of [[collagen]] and [[proteoglycans]]. [[Genes]] involved in the [[pathogenesis]] of [[Enchondroma|enchondromas]] and [[Periosteal chondroma|periosteal chondromas]] include [[isocitrate dehydrogenase]] 1 (''IDH1'') and [[isocitrate dehydrogenase]] 2 (''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== | ||
*[[Enchondroma|Enchondromas]] arise from rests of [[growth plate]] [[cartilage]] or [[chondrocytes]] that are normally involved in the production and | *[[Enchondroma|Enchondromas]] arise from rests of [[growth plate]] [[cartilage]] or [[chondrocytes]] that are normally involved in the production and maintenance of the cartilaginous [[matrix]], which consists mainly of [[collagen]] and [[proteoglycans]].<ref name="pmid9078305">{{cite journal |vauthors=Dal Cin P, Qi H, Sciot R, Van den Berghe H |title=Involvement of chromosomes 6 and 11 in a soft tissue chondroma |journal=Cancer Genet. Cytogenet. |volume=93 |issue=2 |pages=177–8 |date=February 1997 |pmid=9078305 |doi= |url=}}</ref> | ||
*Chondromas | *Chondromas arise as the result of nests of growth plate [[cartilage]] that have become entrapped in the [[Medullary canal|medullary]] canal of the [[metaphysis]] or in the [[metaphyseal]]-[[Diaphysis|diaphyseal]] junction. These [[hamartomatous]] proliferations persist as islands in the bone and then develop from enchondral [[ossification]].<ref name="pmid24959265">{{cite journal |vauthors=Geng S, Zhang J, Zhang LW, Wu Z, Jia G, Xiao X, Hao S |title=Diagnosis and microsurgical treatment of chondromas and chondrosarcomas of the cranial base |journal=Oncol Lett |volume=8 |issue=1 |pages=301–304 |date=July 2014 |pmid=24959265 |pmc=4063631 |doi=10.3892/ol.2014.2072 |url=}}</ref><ref name="pmid2194723">{{cite journal |vauthors=Lewis MM, Kenan S, Yabut SM, Norman A, Steiner G |title=Periosteal chondroma. A report of ten cases and review of the literature |journal=Clin. Orthop. Relat. Res. |volume= |issue=256 |pages=185–92 |date=July 1990 |pmid=2194723 |doi= |url=}}</ref> | ||
==Genetics== | ==Genetics== | ||
*Genes involved in the [[pathogenesis]] of [[Enchondroma|enchondromas]] and | *[[Genes]] involved in the [[pathogenesis]] of [[Enchondroma|enchondromas]] and [[Periosteal chondroma|periosteal chondromas]] include [[isocitrate dehydrogenase]] 1 (''IDH1'') and [[isocitrate dehydrogenase]] 2 (''IDH2)'' .<ref name="pmid15187889">{{cite journal |vauthors=Sandberg AA |title=Genetics of chondrosarcoma and related tumors |journal=Curr Opin Oncol |volume=16 |issue=4 |pages=342–54 |date=July 2004 |pmid=15187889 |doi= |url=}}</ref> | ||
==Associated conditions== | ==Associated conditions== | ||
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[[Metachondromatosis]] | [[Metachondromatosis]] | ||
| align="center" style="padding: 5px 5px; background: #F5F5F5;" | | | align="center" style="padding: 5px 5px; background: #F5F5F5;" | | ||
:Multiple osteochondromas | :Multiple [[osteochondromas]] | ||
:Multiple enchondromas | :Multiple enchondromas | ||
| align="center" style="padding: 5px 5px; background: #F5F5F5;" | | | align="center" style="padding: 5px 5px; background: #F5F5F5;" | | ||
Genetic (Autosomal dominant) | [[Genetic]] ([[Autosomal dominant]]) | ||
|} | |} | ||
==Gross Pathology== | ==Gross Pathology== | ||
*On gross pathology, small (usually less than 3 cm), [[translucent]], grey blue, [[nodular lesions]] with occasional [[calcification]]s are characteristic findings of chondromas.<ref name="pmid18074822">{{cite journal |vauthors=Semenova LA, Bulycheva IV |title=[Chondromas (enchondroma, periosteal chondroma, enchondromatosis)] |language=Russian |journal=Arkh. Patol. |volume=69 |issue=5 |pages=45–8 |date=2007 |pmid=18074822 |doi= |url=}}</ref><ref name="pmid76505">{{cite journal |vauthors=Chung EB, Enzinger FM |title=Chondroma of soft parts |journal=Cancer |volume=41 |issue=4 |pages=1414–24 |date=April 1978 |pmid=76505 |doi= |url=}}</ref> | *On [[gross pathology]], small (usually less than 3 cm), [[translucent]], grey blue, [[nodular lesions]] with occasional [[calcification]]s are characteristic findings of chondromas.<ref name="pmid18074822">{{cite journal |vauthors=Semenova LA, Bulycheva IV |title=[Chondromas (enchondroma, periosteal chondroma, enchondromatosis)] |language=Russian |journal=Arkh. Patol. |volume=69 |issue=5 |pages=45–8 |date=2007 |pmid=18074822 |doi= |url=}}</ref><ref name="pmid76505">{{cite journal |vauthors=Chung EB, Enzinger FM |title=Chondroma of soft parts |journal=Cancer |volume=41 |issue=4 |pages=1414–24 |date=April 1978 |pmid=76505 |doi= |url=}}</ref> | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
*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 | **Cytologically [[Benign|benign cells]] in 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 [[Bone marrow|marrow]] | ||
{{#ev:youtube|FLqr7Ld5-qc}} | {{#ev:youtube|FLqr7Ld5-qc}} | ||
Latest revision as of 20:24, 24 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] Farima Kahe M.D. [3], Soujanya Thummathati, MBBS [4]
Overview
Enchondromas arise from rests of growth plate cartilage or chondrocytes that are normally involved in the production and maintenance of the cartilaginous matrix, which consists mainly of collagen and proteoglycans. Genes involved in the pathogenesis of enchondromas and periosteal chondromas include isocitrate dehydrogenase 1 (IDH1) and isocitrate dehydrogenase 2 (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 maintenance of the cartilaginous matrix, which consists mainly of collagen and proteoglycans.[1]
- Chondromas arise as 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
- Genes involved in the pathogenesis of enchondromas and periosteal chondromas include isocitrate dehydrogenase 1 (IDH1) and isocitrate dehydrogenase 2 (IDH2) .[4]
Associated conditions
Enchondromas may be single or multiple. Multiple enchondromas are associated with the following:[5][6][7]
| Associated disease | Findings | Occurance |
|---|---|---|
|
Sporadic | |
|
Sporadic | |
|
Gross Pathology
- On gross pathology, small (usually less than 3 cm), translucent, grey blue, nodular lesions with occasional calcifications are characteristic findings of chondromas.[8][9]
Microscopic Pathology
- On microscopic histopathological analysis, characteristic findings of chondroma are as followings:[10][11]
- Cytologically benign cells in spaced nests
- Not extending into surrounding soft tissue
- Avascular, fibrous lobules of hyaline cartilagenous matrix
- Interspersed with chondrocytes and separated by normal marrow
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References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Sandberg AA (July 2004). "Genetics of chondrosarcoma and related tumors". Curr Opin Oncol. 16 (4): 342–54. PMID 15187889.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Semenova LA, Bulycheva IV (2007). "[Chondromas (enchondroma, periosteal chondroma, enchondromatosis)]". Arkh. Patol. (in Russian). 69 (5): 45–8. PMID 18074822.
- ↑ Chung EB, Enzinger FM (April 1978). "Chondroma of soft parts". Cancer. 41 (4): 1414–24. PMID 76505.
- ↑ 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.
- ↑ Blum MR, Danford M, Speight PM (August 1993). "Soft tissue chondroma of the cheek". J. Oral Pathol. Med. 22 (7): 334–6. PMID 8229872.