| Literature DB >> 26622573 |
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