Ng Vincent Y, Louie Philip, Punt Stephanie, Conrad Ernest U
Department of Orthopaedics, Greenebaum Cancer Center, University of Maryland Medical Center, 110 S. Paca St., 6 Floor, Suite 300, Baltimore, MD 21201, USA.
Department of Orthopaedics, Rush University Medical Center, Chicago IL, USA.
Open Orthop J. 2017 May 31;11:517-524. doi: 10.2174/1874325001711010517. eCollection 2017.
Synovial chondromatosis (SCh) can undergo malignant transformation. Pathologic diagnosis of secondary synovial chondrosarcoma (SChS) is challenging and misdiagnosis may result in over- or undertreatment.
A systematic review revealed 48 cases of SChS published in 27 reports since 1957. Data was collected to identify findings indicative of SChS and outcomes of treatment.
At median follow-up of 18 months, patients were reported as alive (10%), alive without disease (22%), alive with disease (15%), dead of disease (19%), dead of pulmonary embolism (4%), and unknown (29%). Initial diagnosis of SChS (grade: low/unknown 48%, intermediate/high 52%) was after biopsy in 58%, local resection in 29%, and amputation in 13%. Seventy-four percent of patients underwent 1.8 (mean) resections. Patients treated prior to 1992 were managed with amputation in 79% of cases compared to 48% after 1992. Symptoms were present for 72 mos prior to diagnosis of SChS. Synovial chondrosarcoma demonstrated symptom progression over several months (82%), rapid recurrence after complete resection (30%), and medullary canal invasion (43%). The SChS tumor dimensions were seldom quantified.
Malignant degeneration of synovial chondromatosis is rare but can necessitate morbid surgery or result in death. Pathognomonic signs for SChS including intramedullary infiltration are present in the minority of cases. Progression of symptoms, quick local recurrence, and muscle infiltration are more suggestive of SChS. Periarticular cortical erosion, extra-capsular extension, and metaplastic chondroid features are non-specific. Although poorly documented for SChS, tumor size is a strong indicator of malignancy. Biopsy and partial resection are prone to diagnostic error. Surgical decisions are frequently based on size and clinical appearance and may be in conflict with pathologic diagnosis.
滑膜软骨瘤病（SCh）可发生恶性转化。继发性滑膜软骨肉瘤（SChS）的病理诊断具有挑战性，误诊可能导致过度治疗或治疗不足。
一项系统评价显示，自1957年以来，27篇报告中发表了48例SChS病例。收集数据以确定提示SChS的发现和治疗结果。
中位随访18个月时，患者报告为存活（10%）、无病存活（22%）、带病存活（15%）、死于疾病（19%）、死于肺栓塞（4%）和情况不明（29%）。SChS的初始诊断（分级：低/不明48%，中/高52%）在活检后占58%，局部切除后占29%，截肢后占13%。74%的患者接受了1.8次（平均）切除。1992年之前治疗的患者79%接受了截肢，而1992年之后这一比例为48%。在SChS诊断前症状已存在72个月。滑膜软骨肉瘤表现为症状在数月内进展（82%）、完全切除后快速复发（30%）和髓腔侵犯（43%）。SChS肿瘤大小很少被量化。
滑膜软骨瘤病的恶性退变罕见，但可能需要进行致残性手术或导致死亡。少数病例存在SChS的特征性体征，包括髓内浸润。症状进展、快速局部复发和肌肉浸润更提示为SChS。关节周围皮质侵蚀、囊外扩展和化生软骨样特征不具有特异性。尽管SChS的相关记录较少，但肿瘤大小是恶性程度的有力指标。活检和部分切除容易出现诊断错误。手术决策通常基于大小和临床表现，可能与病理诊断相冲突。
