Abstract
Multiple osteochondromatosis is a familial disease characterized by multiple osteochondromas, defect in metaphyseal remodeling, and asymmetric longitudinal growth retardation. There is a greater incidence in males than females (7:3). It was often first discovered at a younger age than solitary form. There is a predilection for the metaphyseal regions around the knee, hip, and shoulder joints. Radiologically individual lesions are similar to those of solitary form.
The lesions have the same gross and microscopic appearances as seen in solitary osteochondroma. The development of secondary malignancy varies from 5 % to 25 %. The treatment should be considered to correct deformities or functional disturbances.
Keywords
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsRecommended Reading
Jaffe HL. Tumors and tumorous conditions of the bones and joints. Philadelphia: Lea & Febiger; 1958. p. 150–62.
Jamsheer A, Socha M, Sowińska-Seidler A, Telega K, Trzeciak T, Latos-Bieleńska A. Mutational screening of EXT1 and EXT2 genes in Polish patients with hereditary multiple exostoses. J Appl Genet. 2014;55:183–8.
Jennes I, Pedrini E, Zuntini M, Mordenti M, Balkassmi S, Asteggiano CG, et al. Multiple osteochondromas: mutation update and description of the multiple osteochondromas mutation database (MOdb). Hum Mutat. 2009;30:1620–7.
Johnston II CE, Sklar F. Multiple hereditary exostoses with spinal cord compression. Orthopedics. 1988;11:1213–6.
Matsuno T, Ichioka Y, Yagi T, Ishii S. Spindle-cell sarcoma in patients who have osteochondromatosis. A report of two cases. J Bone Joint Surg Am. 1988;70:137–41.
Tian C, Yan R, Wen S, et al. A splice mutation and mRNA decay of EXT2 provoke hereditary multiple exostoses. PLoS One. 2014;9:e94848.
Wuyts W, Van Hul W. Molecular basis of multiple exostoses: mutations in the EXT1 and EXT2 genes. Hum Mutat. 2000;15:220–7.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag London
About this chapter
Cite this chapter
Park, YK. (2015). Multiple Osteochondromatosis. In: Santini-Araujo, E., Kalil, R., Bertoni, F., Park, YK. (eds) Tumors and Tumor-Like Lesions of Bone. Springer, London. https://doi.org/10.1007/978-1-4471-6578-1_20
Download citation
DOI: https://doi.org/10.1007/978-1-4471-6578-1_20
Publisher Name: Springer, London
Print ISBN: 978-1-4471-6577-4
Online ISBN: 978-1-4471-6578-1
eBook Packages: MedicineMedicine (R0)