Osteochondroma

Osteochondroma
Other namesOsteocartilaginous exostoses
Lateral radiograph of the knee demonstrating ossification in the peritendinous tissues in a patient with osteochondroma.
SpecialtyOrthopedics

Osteochondromas are the most common benign tumors of the bones.[1][2] The tumors take the form of cartilage-capped bony projections or outgrowth on the surface of bones (exostoses).[3][4] It is characterized as a type of overgrowth that can occur in any bone where cartilage forms bone. Tumors most commonly affect long bones about the knee and in the forearm.[1][3] Additionally, flat bones such as the pelvis and scapula (shoulder blade) may be affected.[5]

Hereditary multiple exostoses usually present during childhood. Yet, the vast majority of affected individuals become clinically manifest by the time they reach adolescence.[3][6] Osteochondromas occur in 3% of the general population and represent 35% of all benign tumors and 8% of all bone tumors. The majority of these tumors are solitary non-hereditary lesions and approximately 15% of osteochondromas occur as hereditary multiple exostoses preferably known as hereditary multiple osteochondromas (HMOs).[4][7]

Osteochondromas do not result from injury and the exact cause remains unknown. Recent research has indicated that multiple osteochondromas is an autosomal dominant inherited disease. Germline mutations in EXT1 and EXT2 genes located on chromosomes 8 and 11 have been associated with the cause of the disease.[8]

The treatment choice for osteochondroma is surgical removal of solitary lesion or partial excision of the outgrowth, when symptoms cause motion limitations or nerve and blood vessel impingements.[4][7] In hereditary multiple exostoses the indications of surgery are based upon multiple factors that are taken collectively, namely: patient's age, tumor location and number, accompanying symptomatology, esthetic concerns, family history and underlying gene mutation.[1][3] A variety of surgical procedures have been employed to remedy hereditary multiple exostoses such as osteochondroma excision, bone lengthening, corrective osteotomy and hemiepiphysiodesis. Sometimes a combination of the previous procedures is used.[3] The indicators of surgical success in regard to disease and patient characteristics are greatly disputable.[3] Because most studies of hereditary multiple exostoses are retrospective and of limited sample size with missing data, the best evidence for each of the currently practiced surgical procedures is lacking.[3]

  1. ^ a b c Wuyts, W; Schmale, GA; Chansky, HA; et al. (21 November 2013). "Hereditary Multiple Osteochondromas". GeneReviews. Retrieved 30 March 2018.
  2. ^ Sekharappa, V; Amritanand R; Krishnan V; David KS (February 2014). "Symptomatic solitary osteochondroma of the subaxial cervical spine in a 52-year-old patient". Asian Spine J. 8 (1): 84–88. doi:10.4184/asj.2014.8.1.84. PMC 3939376. PMID 24596611.
  3. ^ a b c d e f g EL-Sobky, TA; Samir, S; Atiyya, AN; Mahmoud, S; Aly, AS; Soliman, R (21 March 2018). "Current paediatric orthopaedic practice in hereditary multiple osteochondromas of the forearm: a systematic review". Sicot-J. 4: 10. doi:10.1051/sicotj/2018002. PMC 5863686. PMID 29565244.
  4. ^ a b c Panagiotis, Kitsoulis; Vassiliki Galani; Kalliopi Stefanaki; Georgios Paraskevas; Georgios Karatzias; Niki John Agnantis; Maria Bai (October 2008). "Osteochondromas: Review of the Clinical, Radiological and Pathological Features". In Vivo. 22 (5): 633–646. PMID 18853760. Retrieved 22 March 2014.
  5. ^ "Osteochondroma". University of Rochester Medical Center. Retrieved 15 March 2014.
  6. ^ de Andrea, CE; Reijnders CM; Kroon HM; De Jong D; Hogendoorn PC; Szuhai K; Bovée JV (1 March 2012). "Secondary peripheral chondrosarcoma evolving from osteochondroma as a result of outgrowth of cells with functional EXT". Oncogene. 31 (9): 1095–1104. doi:10.1038/onc.2011.311. hdl:1887/100035. PMID 21804604. S2CID 11418240.
  7. ^ a b Reijnders, Christianne; Liesbeth Hameetman; Judith VMG Bovée (September 2008). "Bone: Osteochondroma". Atlas of Genetics and Cytogenetics in Oncology and Haematology. Retrieved 15 March 2014.
  8. ^ Krahe, Ralf; Zhang, Feng; Liang, Jinlong; Guo, Xiong; Zhang, Yingang; Wen, Yan; Li, Qiang; Zhang, Zengtie; Ma, Weijuan; Dai, Lanlan; Liu, Xuanzhu; Yang, Ling; Wang, Jun (2013). "Exome Sequencing and Functional Analysis Identifies a Novel Mutation in EXT1 Gene That Causes Multiple Osteochondromas". PLOS ONE. 8 (8): e72316. doi:10.1371/journal.pone.0072316. ISSN 1932-6203. PMC 3757002. PMID 24009674.