Osteochondroma: ignore or investigate?☆

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Abstract

Osteochondromas are bone protuberances surrounded by a cartilage layer. They generally affect the extremities of the long bones in an immature skeleton and deform them. They usually occur singly, but a multiple form of presentation may be found. They have a very characteristic appearance and are easily diagnosed. However, an atypical site (in the axial skeleton) and/or malignant transformation of the lesion may sometimes make it difficult to identify osteochondromas immediately by means of radiographic examination. In these cases, imaging examinations that are more refined are necessary. Although osteochondromas do not directly affect these patients’ life expectancy, certain complications may occur, with varying degrees of severity.

Resumo

Osteocondromas são protuberâncias ósseas envolvidas por uma camada de cartilagem. Atingem, habitualmente, as extremidades dos ossos longos no esqueleto imaturo e os deformam. Em geral são únicos, mas a forma de apresentação múltipla pode ser encontrada. De aspecto bastante característico, são de fácil diagnóstico. Contudo, por vezes, a localização atípica (esqueleto axial) e/ou a malignização da lesão podem dificultar a sua pronta identificação por exames radiográficos. Nesses casos, exames de imagem mais apurados são necessários. Apesar de não afetarem diretamente a expectativa de vida do portador, algumas complicações, com variados graus de gravidade, podem ocorrer.

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Most cited references 70

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Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation.

M Murphey, J. Choi, M J Kransdorf … (2015)

Osteochondroma represents the most common bone tumor and is a developmental lesion rather than a true neoplasm. It constitutes 20%-50% of all benign bone tumors and 10%-15% of all bone tumors. Its radiologic features are often pathognomonic and identically reflect its pathologic appearance. Osteochondromas are composed of cortical and medullary bone with an overlying hyaline cartilage cap and must demonstrate continuity with the underlying parent bone cortex and medullary canal. Osteochondromas may be solitary or multiple, the latter being associated with the autosomal dominant syndrome, hereditary multiple exostoses (HME). Complications associated with osteochondromas are more frequent with HME and include deformity (cosmetic and osseous), fracture, vascular compromise, neurologic sequelae, overlying bursa formation, and malignant transformation. Malignant transformation is seen in 1% of solitary osteochondromas and in 3%-5% of patients with HME. Continued lesion growth and a hyaline cartilage cap greater than 1.5 cm in thickness, after skeletal maturity, suggest malignant transformation. Variants of osteochondroma include subungual exostosis, dysplasia epiphysealis hemimelica, turret and traction exostoses, bizarre parosteal osteochondromatous proliferation, and florid reactive periostitis. Recognition of the radiologic spectrum of appearances of osteochondroma and its variants usually allows prospective diagnosis and differentiation of the numerous potential complications, thus helping guide therapy and improving patient management.

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The natural history of hereditary multiple exostoses.

Chelsie Conrad, W Raskind, David G. Schmale (1994)

We established a database of hereditary multiple exostoses for the state of Washington, on the basis of a retrospective review of the medical records and a clinical evaluation of family members, to determine the prevalence, clinical range of expression, and rate of malignant degeneration. The database comprised forty-six kindreds with 113 affected members; all kindreds had at least one member living in the state of Washington. The over-all prevalence was at least one in 50,000. Approximately 10 per cent of the subjects had no family history of multiple exostoses. With the use of twenty-three pedigrees that demonstrated an adequate multigenerational history for determination of penetrance of the gene, we identified one unaffected individual among twenty-six obligate heterozygotes, a rate of penetrance of 96 per cent. There was no evidence for a substantial reduction of penetrance in female subjects. The median age at the time of the diagnosis in the 113 affected individuals was three years (range, birth to twelve years). In a cohort of eighty-four subjects for whom we had complete information, the clinical range of expression was wide: thirty-three (39 per cent) had an obvious deformity of the forearm, eight (10 per cent) had an inequality in the lengths of the limbs, seven (8 per cent) had an angular deformity of the knee, and two (2 per cent) had a deformity of the ankle. The average number of operations for the patients for whom the operative history was known was two.(ABSTRACT TRUNCATED AT 250 WORDS)

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Multiple osteochondromas

Judith VMG Bovée (2008)

Multiple osteochondromas (MO) is characterised by development of two or more cartilage capped bony outgrowths (osteochondromas) of the long bones. The prevalence is estimated at 1:50,000, and it seems to be higher in males (male-to-female ratio 1.5:1). Osteochondromas develop and increase in size in the first decade of life, ceasing to grow when the growth plates close at puberty. They are pedunculated or sessile (broad base) and can vary widely in size. The number of osteochondromas may vary significantly within and between families, the mean number of locations is 15–18. The majority are asymptomatic and located in bones that develop from cartilage, especially the long bones of the extremities, predominantly around the knee. The facial bones are not affected. Osteochondromas may cause pain, functional problems and deformities, especially of the forearm, that may be reason for surgical removal. The most important complication is malignant transformation of osteochondroma towards secondary peripheral chondrosarcoma, which is estimated to occur in 0.5–5%. MO is an autosomal dominant disorder and is genetically heterogeneous. In almost 90% of MO patients germline mutations in the tumour suppressor genes EXT1 or EXT2 are found. The EXT genes encode glycosyltransferases, catalyzing heparan sulphate polymerization. The diagnosis is based on radiological and clinical documentation, supplemented with, if available, histological evaluation of osteochondromas. If the exact mutation is known antenatal diagnosis is technically possible. MO should be distinguished from metachondromatosis, dysplasia epiphysealis hemimelica and Ollier disease. Osteochondromas are benign lesions and do not affect life expectancy. Management includes removal of osteochondromas when they give complaints. Removed osteochondromas should be examined for malignant transformation towards secondary peripheral chondrosarcoma. Patients should be well instructed and regular follow-up for early detection of malignancy seems justified. For secondary peripheral chondrosarcoma, en-bloc resection of the lesion and its pseudocapsule with tumour-free margins, preferably in a bone tumour referral centre, should be performed.

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Author and article information

Contributors

Rosalvo Zósimo Bispo Júnior

Journal

Journal ID (nlm-ta): Rev Bras Ortop

Journal ID (iso-abbrev): Rev Bras Ortop

Title: Revista Brasileira de Ortopedia

Publisher: Elsevier

ISSN (Electronic): 2255-4971

Publication date PMC-release: 27 October 2014

Publication date Collection: Nov-Dec 2014

Publication date (Electronic): 27 October 2014

Volume: 49

Issue: 6

Pages: 555-564

Affiliations

[a ]School of Medicine, Federal University of Pernambuco (UFPE), Recife, PE, Brazil

[b ]School of Medicine, Federal University of Paraíba (UFPB), João Pessoa, PB, Brazil

[c ]University Center of João Pessoa (UNIPÊ), João Pessoa, PB, Brazil

Author notes

[* ] Corresponding author. zosimobispo@ 123456gmail.com

Article

Publisher ID: S2255-4971(14)00161-X

DOI: 10.1016/j.rboe.2013.10.002

PMC ID: 4487501

PubMed ID: 26229862

SO-VID: 23feec1a-6db9-49cf-a41f-058d9e9c5db6

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 23 August 2013

Date accepted : 31 October 2013

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