Literature DB >> 26622573

Identification of a rare case of intra-articular osteochondroma manifesting as three loose bodies in a patient with hereditary multiple osteochondromas: A case report.

Mingxiang Kong1, L I Cao1, Qiong Zhang2, Yong Fang1, Chen Zhao1, Haifeng Gu1, Shuijun Zhang1, Y U Chen1, Jinhua Wu1, Qing Bi1.   

Abstract

Hereditary multiple osteochondromas (HMO) is an autosomal dominant bone disorder characterised by the presence of multiple benign cartilage-capped tumours. Exostosin-1 (EXT1) and EXT2 are the major morbigenous genes associated with HMO, mutations in which are responsible for 90% of all HMO cases. In patients with HMO, osteochondromas arise adjacent to the metaphysis and typically remain in the metaphyseal region of the long bones. Therefore, it is rare for osteochondromas to be identified intra-articularly, although they may manifest as loose bodies. The present study describes a rare case of HMO manifesting as limited flexing range in the right knee joint of a 27-year-old male patient. Computed tomography and magnetic resonance imaging (MRI) revealed three intra-articular osteochondromas located in the intercondylar fossa of the patient's right knee. The intra-articular osteochondromas and protuberant extra-articular osteochondromas around the right knee were resected, resulting in improved right knee function and no postoperative recurrence. Pathological analysis revealed that the intra-articular osteochondromas had a thinner cartilage cap layer than the extra-articular osteochondromas. In addition, genetic analysis of the patient and the patient's mother was conducted. From this, it was determined that a nonsense mutation, c.115G>T (p.E39X) in exon 1 of the EXT1 gene, was the cause of HMO in this case. Thus, it is proposed that osteochondromas with a pedicle within the knee, may tear and become loose intra-articular bodies, resulting in limited joint function and thereby contributing to the progression of HMO.

Entities:  

Keywords:  exostosin-1 gene; hereditary multiple osteochondromas; intra-articular; loose body; nonsense mutation

Year:  2015        PMID: 26622573      PMCID: PMC4509409          DOI: 10.3892/ol.2015.3284

Source DB:  PubMed          Journal:  Oncol Lett        ISSN: 1792-1074            Impact factor:   2.967


  22 in total

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Journal:  Arthroscopy       Date:  2001 Nov-Dec       Impact factor: 4.772

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Journal:  Arthroscopy       Date:  2004-07       Impact factor: 4.772

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Journal:  Skeletal Radiol       Date:  1994-02       Impact factor: 2.199

7.  The variable manifestations of extraarticular synovial chondromatosis.

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Journal:  AJR Am J Roentgenol       Date:  1981-10       Impact factor: 3.959

8.  A gene for hereditary multiple exostoses maps to chromosome 19p.

Authors:  M Le Merrer; L Legeai-Mallet; P M Jeannin; B Horsthemke; A Schinzel; H Plauchu; A Toutain; F Achard; A Munnich; P Maroteaux
Journal:  Hum Mol Genet       Date:  1994-05       Impact factor: 6.150

9.  Para-articular osteochondroma of the knee.

Authors:  Giacomo Rizzello; Francesco Franceschi; Maria Chiara Meloni; Emanuela Cristi; Simona Angela Barnaba; Carla Rabitti; Vincenzo Denaro
Journal:  Arthroscopy       Date:  2006-11-27       Impact factor: 4.772

10.  Hereditary multiple exostoses of the hip.

Authors:  Tarek A M El-Fiky; Wang Chow; Yun Hoi Li; Michael To
Journal:  J Orthop Surg (Hong Kong)       Date:  2009-08       Impact factor: 1.118

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  1 in total

1.  A Case Report of Trevor's Disease in a Pediatric Patient with Hereditary Multiple Exostoses Disease.

Authors:  Timothy W Torrez; Elizabeth Marks; Shane Strom; John Scott Doyle
Journal:  J Orthop Case Rep       Date:  2021-12
  1 in total

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