滑膜软骨瘤病与软骨肉瘤：诊断难题
Synovial chondromatosis and chondrosarcoma: a diagnostic dilemma.
作者信息
Sperling Brita L, Angel Steven, Stoneham Grant, Chow Vance, McFadden Andrew, Chibbar Rajni
机构信息
Department of Pathology Royal University Hospital Saskatchewan Saskatoon S7N OW8 Canada.
出版信息
Sarcoma. 2003;7(2):69-73. doi: 10.1080/13577140310001607293.
PURPOSE
The progression of synovial chondromatosis to chondrosarcoma is very rare. Distinction between these two entities may be difficult on histology alone, and should be based on clinical, radiographic and microscopic evidence. Immunohistochemical markers that would facilitate differentiation between synovial chondromatosis and chondrosarcoma are currently being investigated.
PATIENTS
We describe the cases of two patients who presented with synovial chondromatosis and progression to synovial chondrosarcoma during periods of 7 and 11 years. Several biopsies and resected specimens demonstrated synovial chondromatosis before a diagnosis of chondrosarcoma was made.
METHOD
We have examined five markers (Bcl2, Ki67, p27, p16, and p53) in all specimens from these cases, as well as known cases of chondromatosis and chondrosarcoma for control purposes.
RESULTS
We found increased expression of Bcl2 in benign chondromatosis compared to synovial or central chondrosarcomas.
DISCUSSION
Distinction between chondromatosis and its progression to low grade chondrosarcoma is difficult at histological level, and must involve incorporation of clinical and radiographical data. Although preliminary, our study suggests that reduced or absent expression of Bcl2 is associated withmalignant transformation of chondromatosis.
目的
滑膜软骨瘤病进展为软骨肉瘤极为罕见。仅依靠组织学鉴别这两种病变可能存在困难，而应依据临床、影像学和显微镜检查证据。目前正在研究有助于区分滑膜软骨瘤病和软骨肉瘤的免疫组织化学标志物。
患者
我们描述了2例滑膜软骨瘤病患者的病例，这2例患者分别在7年和11年期间进展为滑膜软骨肉瘤。在诊断为软骨肉瘤之前，多次活检及切除标本均显示为滑膜软骨瘤病。
方法
我们检测了这些病例所有标本以及软骨瘤病和软骨肉瘤已知病例标本中的5种标志物（Bcl2、Ki67、p27、p16和p53），以作对照。
结果
我们发现，与滑膜或中央型软骨肉瘤相比，良性软骨瘤病中Bcl2表达增加。
讨论
在组织学层面，区分软骨瘤病及其进展为低级别软骨肉瘤存在困难，必须结合临床和影像学数据。尽管我们的研究尚属初步，但提示Bcl2表达降低或缺失与软骨瘤病的恶性转化有关。
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