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Other Rare Benign Lesions

  • Alberto RighiEmail author
Chapter
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Abstract

Dysplasia epiphysealis hemimelica, also called Trevor disease or Fairbank disease, is a rare nonfamilial disease of unknown etiology. It presents in early childhood between the ages of 3 and 15 years with osteochondroma-like swelling close to a joint, a limited range of movement, and occasional “locking” of the involved joint. A cartilaginous lesion that continues to grow and undergo endochondral ossification until skeletal maturity is reached and generally requires surgical treatment causes the swelling. The majority of children have more than one lesion affecting a single limb, usually a lower limb. Radiologically and macroscopically, the features of dysplasia epiphysealis hemimelica overlap with those of osteochondromas. Histologically, dysplasia epiphysealis hemimelica is composed of multiple nodules of cartilage with centers of active endochondral ossification in the epiphysis functioning as accessory secondary ossification centers. By 3–4 years of age, most of the nodules have fused with epiphysis, and the accessory ossification centers have disappeared. The cartilage cap contains chondrocytes arranged in a fibrillar matrix in a somewhat disorganized manner compared with the surrounding normal cartilage. The differential diagnosis includes enchondroma, synovial chondromatosis, and chondrosarcoma. Radiologic correlation is required for diagnosis.

Keywords

Dysplasia epiphysealis hemimelica Osteoma Intraosseous schwannoma Benign fibrous histiocytoma Nora’s disease Subungual exostosis 

Selected Bibliography

  1. Klein MJ, Bonar SF, Freemont T, Vinh TN, Lopez-Ben R, Siegel HJ, Siegal GP. Non-neoplastic diseases of bones and joints. Washington, DC: AFIP-ARP; 2011.Google Scholar
  2. Sciot R, Mandahl N. Subungual exostosis and bizarre parosteal osteochondromatous proliferation. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and Bone. Lyon: IARC Press; 2013. p. 259–60.Google Scholar
  3. Unni KK, Indwards CY, Bridge JA, Kindblom L-G, Wold LE. Tumors of the bones and joints. Washington, DC: AFIP-ARP; 2005.Google Scholar
  4. Unni KK, Indwards CY. Dahlin’s bone tumors. Philadelphia: Lippincott Williams & Wilkins; 2010.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of PathologyIRCCS Istituto Ortopedico RizzoliBolognaItaly

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