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Synovial Chondrosarcoma Arising in Synovial Chondromatosis

机译:滑膜软骨瘤病引起的滑膜软骨肉瘤

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Primary synovial chondromatosis (SC) is a rare proliferative disorder that causes pain, swelling, and restriction of movement to the joints it affects. The disease frequently runs a protracted course, often requiring multiple surgical procedures to obtain some control. Few reports exist detailing the natural history of SC, although malignant transformation to synovial chondrosarcoma (CHS) is recognized to be a rare event. The aim of our study was to review a large orthopaedic oncology database in order to evaluate the incidence of CHS arising from SC. We identified 78 patients who have presented to our centre with primary synovial chondromatosis (SC). Of those patients, 5 went on to develop malignant change. This represents a 6.4% incidence of developing synovial chondrosarcoma (CHS) within preexisting primary synovial chondromatosis. The patients had a mean age of 28 years at first diagnosis with synovial chondromatosis with the median time from original diagnosis to malignant transformation being 20 years (range 2.7–39 yrs).
机译:原发性滑膜软骨瘤病(SC)是一种罕见的增生性疾病,可引起疼痛,肿胀和所影响的关节活动受限。该病经常经历一个漫长的过程,经常需要多次外科手术才能获得一定的控制。很少有报道详细报道SC的自然史,尽管公认滑膜软骨肉瘤(CHS)恶变是罕见的事件。我们的研究目的是回顾一个大型的骨科肿瘤学数据库,以评估由SC引起的CHS的发生率。我们确定了78例原发于滑膜软骨病(SC)的患者。在这些患者中,有5位继续发生恶变。这表示在既存的原发性滑膜软骨瘤病中发展为滑膜软骨肉瘤(CHS)的发生率为6.4%。初诊滑膜软骨病的患者平均年龄为28岁,从最初诊断到恶性转化的中位时间为20年(2.7-39岁)。

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