Gille Justus, Krueger Stefan, Aberle Jens, Boehm Stephanie, Ince Akif, Loehr Joachim F
Endo-Klinik, Hamburg, Germany.
Acta Orthop Belg. 2004 Apr;70(2):182-8.
Primary synovial chondromatosis (PSC) is a rare, usually monoarticular disorder of synovial joints. PSC is characterised by the formation of osteocartilaginous nodules in the synovial connective tissue. We report the case of a 32-year-old male with PSC of the left hip. At clinical examination abduction of the left hip was limited and rotation was painful. Ultrasound examination of the hip revealed joint effusion and multiple hyperechogenic foci due to distal acoustic shadowing. Plain radiographs showed a slight soft tissue swelling around the femoral neck and multiple round or ovoid calcifications of a uniform size. MRI revealed a large joint effusion with multiple small filling defects. Open total synovectomy was performed after dislocation of the femoral head. The diagnosis of PSC was confirmed by histological examination of the excised material. The majority of cells failed to exhibit any staining for cerb B-2 and ki-67. None of the sections showed more than 5% labelling for DNA-fragmentation proven by terminal deoxytransferase-mediated dUTD nick-end labeling (TUNEL), and all were completely non-reactive for p53 as well. In conclusion, immunohistochemical analysis suggests that in this case PSC originated from metaplasia and not from a proliferative process. After two years, the patient was free of symptoms and radiological control did not show evidence of recurrence or femoral head necrosis. Physical findings, diagnosis, histological features and management of PSC are discussed.
原发性滑膜软骨瘤病(PSC)是一种罕见的、通常累及单关节的滑膜关节疾病。PSC的特征是在滑膜结缔组织中形成骨软骨结节。我们报告一例32岁男性左髋关节PSC病例。临床检查发现左髋关节外展受限且旋转时疼痛。髋关节超声检查显示关节积液以及因后方声影导致的多个高回声灶。X线平片显示股骨颈周围轻度软组织肿胀以及多个大小均匀的圆形或椭圆形钙化灶。MRI显示大量关节积液伴多个小充盈缺损。在股骨头脱位后进行了开放性全滑膜切除术。切除组织的组织学检查证实了PSC的诊断。大多数细胞对cerb B-2和ki-67均无染色。通过末端脱氧核苷酸转移酶介导的dUTD缺口末端标记法(TUNEL)证实,所有切片中DNA片段化标记超过5%的情况均未出现,并且所有切片对p53也完全无反应。总之,免疫组化分析表明,在该病例中PSC起源于化生而非增殖过程。两年后,患者无症状,影像学检查未显示复发或股骨头坏死迹象。本文讨论了PSC的体格检查结果、诊断、组织学特征及治疗。