Anract P, Katabi M, Forest M, Benoit J, Witvoët J, Tomeno B
Service de Chirurgie Orthopédique, Hôpital Cochin, Paris.
Rev Chir Orthop Reparatrice Appar Mot. 1996;82(3):216-24.
This retrospective study concerns six patients in whom chondrosarcoma was suspected to develop in synovial chondromatosis. We discuss for these cases different diagnosis. The authors expose the clinical signs and radiological aspects which suggest malignant transformation. We report the indispensable criteria for established diagnosis of malignant transformation of synovial chondromatosis and appropriated forms of therapeutic management were suggested.
Six patients, 3 males and 3 females from 36 to 58 years of age were included in this study. Three patients presented 6 months, 3 years and 25 years history of synovial chondromatosis of the knee joint. When the malignant transformation appeared, a surgical biopsy was performed and the pathologist diagnosed a chondrosarcoma in all cases. For the other three patients, the chondrosarcoma and synovial chondromatosis were diagnosed at the same time. The localization was shoulder, hip and knee. A surgical biopsy was performed and the pathologist diagnosed chondrosarcoma. Synovial chondromatosis was diagnosed by histologic examination of the resection or amputation specimen.
Four patients had thigh amputation, one patient had "en bloc resection" of the hip-joint and the last patient had resection of the shoulder joint. In all cases, the histologic examination diagnosed chondrosarcoma and synovial chondromatosis. All patients were free of disease.
The malignant transformation of synovial chondromatosis is rare but this diagnosis must be established to perform appropriate treatment. Other possible diagnosis are: low grade synovial chondrosarcoma initially diagnosed as a synovial chondromatosis. Bertoni believes that all cases of malignant transformation of synovial chondromatosis are initially low grade chondrosarcoma. We believe that his criteria are too strict for diagnosed chondrosarcoma. coexistence of synovial chondromatosis and synovial chondrosarcoma. These 2 diagnosis are extremely rare and their coexistence are unlikely. secondary synovial chondromatosis developed into chondrosarcoma. We don't have histologic criteria to confirm this diagnosis in all our cases. The symptoms that should suggest a malignant transformation of synovial chondromatosis were: rapid late deterioration of clinical conditions, bone invasion diagnosed by X-ray films and medullar invasion discovered by MRI. According to us, the indispensable criteria to diagnose malignant transformation were: 1.) histologic diagnosis of synovial chondromatosis established before diagnosis of chondrosarcoma, 2.) histologic diagnosis of chondrosarcoma on the same anatomic site as the synovial chondromatosis, 3.) diagnosis of chondrosarcoma and synovial chondromatosis on the same resection specimen. Only the three first cases were in accordance with these criteria. The treatment must be a "en bloc resection" of the joint or an amputation.
Malignant transformation is rare, but this diagnosis should be established to perform adequate treatment. This diagnosis should be suspected when a rapid deterioration of the clinical status appeared and when bone involvement was detected by MRI. However, the danger still lies in the misinterpretation of the synovial chondromatosis as chondrosarcoma. This diagnosis can be made with clinical, radiological and pathological criteria. The treatment must be a wide resection or an amputation.
本回顾性研究涉及6例疑似滑膜软骨瘤病发展为软骨肉瘤的患者。我们讨论了这些病例的不同诊断。作者阐述了提示恶性转化的临床体征和影像学表现。我们报告了滑膜软骨瘤病恶性转化确诊的必要标准，并提出了适当的治疗管理方式。
本研究纳入6例患者，3男3女，年龄在36至58岁之间。3例患者有膝关节滑膜软骨瘤病6个月、3年和25年的病史。当出现恶性转化时，进行了手术活检，病理学家在所有病例中均诊断为软骨肉瘤。另外3例患者，软骨肉瘤和滑膜软骨瘤病同时被诊断出来。病变部位分别为肩部、髋部和膝部。进行了手术活检，病理学家诊断为软骨肉瘤。滑膜软骨瘤病通过对切除或截肢标本的组织学检查来诊断。
4例患者进行了大腿截肢，1例患者进行了髋关节“整块切除”，最后1例患者进行了肩关节切除。在所有病例中，组织学检查均诊断为软骨肉瘤和滑膜软骨瘤病。所有患者均无疾病。
滑膜软骨瘤病的恶性转化很少见，但必须确立这一诊断以进行适当治疗。其他可能的诊断包括：最初被诊断为滑膜软骨瘤病的低级别滑膜软骨肉瘤。贝托尼认为滑膜软骨瘤病的所有恶性转化病例最初都是低级别软骨肉瘤。我们认为他的标准对于诊断软骨肉瘤来说过于严格。滑膜软骨瘤病与滑膜软骨肉瘤并存。这两种诊断极其罕见，它们并存的可能性不大。继发性滑膜软骨瘤病发展为软骨肉瘤。我们没有组织学标准来在所有病例中证实这一诊断。提示滑膜软骨瘤病恶性转化的症状包括：临床状况迅速晚期恶化、X线片诊断的骨侵犯以及MRI发现的骨髓侵犯。据我们所知，诊断恶性转化的必要标准为：1.）在诊断软骨肉瘤之前确立滑膜软骨瘤病的组织学诊断；2.）软骨肉瘤的组织学诊断与滑膜软骨瘤病在同一解剖部位；3.）在同一切除标本上诊断软骨肉瘤和滑膜软骨瘤病。只有前3例符合这些标准。治疗必须是关节的“整块切除”或截肢。
恶性转化很少见，但应确立这一诊断以进行充分治疗。当临床状况迅速恶化且MRI检测到骨受累时，应怀疑这一诊断。然而，危险仍然在于将滑膜软骨瘤病误诊为软骨肉瘤。这一诊断可依据临床、影像学和病理学标准做出。治疗必须是广泛切除或截肢。
