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Contributor: Maria Flynn - National Capital Consortium
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More Like This ? Enchondromatosis, Ollier's disease, Chondrosarcoma
Factoid 3472 - Created: 2001-12-05 18:01:41-05 - Modified: 2006-11-10 16:29:32-05
ACR Codes: 4.3
Enchondromas are benign cartilaginous lesion. It is the most common benign cystic-appearing lesion in the phalanges. The hands (phalanges & metacarpals) are the most common site of visible enchondromas, although they are also common in long tubular bones. Usually enchondromas are metaphyseal or diaphyseal and may be central, eccentric, expansile or nonexpansile. In lesion other than the hands, they may contain calcified chondroid matrix. If it is extracortical in location (periosteal) then it is termed a chondroma (periosteal or juxtacortical) rather than an enchondroma.

Clinically there is no sex predilection and the usually age range is 20 – 40 although it may be seen through out life. Enchondromas are commonly asymptomatic however patients may have knobby swelling of digits or fracture. Plain film is usually diagnostic.


Ollier’s disease is a nonhereditary condition of multiple enchondromas which is distinguished from Maffucci syndrome by the lack of soft tissue hemangiomas (radiographically identified by soft tissue calcifications). The enchondromas are generally metaphyseal or diaphyseal. The are often extensive and unilateral in Ollier’s disease. Clinical manifestations of enchondromatosis include deformities of the bones including knobby swelling of digits. There can be gross disparity in the lengths of the forearms or legs secondary to interference of the growth plate by the lesion. They can also present as pathologic fracture.

The most worrisome complication of Ollier’s disease is malignant transformation into chondrosarcoma. In contrast to solitary enchondromas, any enchondroma – even lesions in the short tubular bones - may undergo sarcomatous change in Ollier’s disease and Maffucci syndrome.

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Regarding the potential for malignant transformation:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Display&dopt=pubmed_pubmed&from_uid=15723211

http://bonetumor.org/tumors/pages/page39.html

http://www.emedicine.com/radio/topic168.htm

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Search&db=PubMed&term=enchondromatosis+and+chondrosarcoma&tool=QuerySuggestion

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=473035

http://health.enotes.com/genetic-disorders-encyclopedia/chondrosarcoma

http://www.patient.co.uk/showdoc/40001121/
Reference(s):
Brant, W. and C. Helms. Fundamentals of Diagnostic Radiology. 2nd edition. Williams & Wilkins.Baltimore. 1999. P. 962, 964-966, 979.

Greenspan, A. Orthopedic Radiology, 2nd edition. Lippincott-Raven. Philadelphia. 1996. P. 16.12-16.18.
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Location:
MSK - Musculoskeletal
Sublocation:
Cartilage
Category:
Neoplasm, benign
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Prepared by: Maria Flynn
Affiliation: National Capital Consortium - || - Author Profile
Approved by: Philip A Dinauer
Affiliation: Civilian Medical Center - || - Editor Profile
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