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Chondrosarcoma in MetachondromatosisA Case Report
Andreas F. Mavrogenis, MD1; Evangelia Skarpidi, MD2; Olympia Papakonstantinou, MD3; Panayiotis J. Papagelopoulos, MD, DSc4
1 First Department of Orthopaedics, Athens University Medical School, 41 Ventouri Street, 15562 Holargos, Athens, Greece. E-mail address: andreasfmavrogenis@yahoo.gr
2 Department of Pathology, Hygeia Medical Center, 4 Erithrou Stavrou and Kifissias Avenue, Maroussi, Athens, Greece. E-mail address: eskarpidi@gmail.com
3 Second Department of Radiology, Athens University Medical School, Rimini 1, 12462 Chaidari, Athens, Greece. E-mail address: olypapak@hotmail.com
4 First Department of Orthopaedics, ATTIKON University Hospital, Athens University Medical School, 4 Christovassili Street, 15451 Neo Psychikon, Athens, Greece. E-mail address: pjp@hol.gr
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Disclosure: The authors did not receive any outside funding or grants in support of their research for or preparation of this work. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity.

Investigation performed at the First Department of Orthopaedics, ATTIKON University Hospital, Athens University Medical School, Athens, Greece

Copyright ©2010 American Society for Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2010 Jun 01;92(6):1507-1513. doi: 10.2106/JBJS.I.00693
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Extract

Metachondromatosis is a rare, inherited disease that was described in 1971 by Maroteaux1. He chose the name metachondromatosis to emphasize the evolving or changing nature of the chondromatous lesions1-5. The complete form of the disease is characterized by multiple metaphyseal juxtaepiphyseal exostoses, metaphyseal enchondromas, periarticular calcifications, and frequent unilateral or bilateral Legg-Calvé-Perthes-like changes in the femoral head, resembling osteonecrosis1-3,6-8. The metachondromatosis-associated exostoses are true osteochondromas with cartilaginous caps and characteristically point toward the adjacent joint, often involve the bones of the hands and feet, and do not produce shortening or growth disturbance of the affected bone8. The periarticular calcifications are due to peripheral enchondral ossification in epiphysis-based exophytic enchondromas. The enchondromatous lesions, unlike those of multiple enchondromatosis, most often affect the iliac crests and the metaphyses of certain long bones, showing similar radiographic irregularities. Metachondromatosis-associated enchondromas may appear as metaphyseal enchondromas6,9, have a periarticular flowery appearance3,6,9, or be similar to lesions seen in lytic metastatic disease3,8. The reported locations of these lesions include the iliac crest, proximal and distal end of the femur, proximal part of the fibula, distal end of the tibia, distal aspect of the radius, proximal part of the humerus, and hands and feet4,6,8,9. The disorder has not been mapped in the human genome. The mode of inheritance is autosomal dominant1-3,6,8.
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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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