ten Heuvel Suzan E, Hoekstra Harald J, Bastiaannet Esther, Suurmeijer Albert J H
Department of Surgical Oncology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Appl Immunohistochem Mol Morphol. 2009 May;17(3):189-95. doi: 10.1097/PAI.0b013e31818a6f5c.
The aim of this study was to investigate prognostic factors influencing the survival of synovial sarcoma, including the debated role of SYT-SSX fusion type and the newly suggested immunohistochemical marker ezrin.
From 1984 to 2005, 45 patients-25 men (56%) and 20 women (44%) with a median age of 31 (range: 2 to 81) years-were diagnosed with a synovial sarcoma. Age at diagnosis, tumor site, tumor size, tumor histology (biphasic vs. monophasic), mitotic count, necrosis, histologic grade, SYT-SSX fusion type, and ezrin immunostaining were analyzed for influence on survival by univariate and multivariate methods.
The median follow-up for all patients was 55 (range: 2 to 238) months. Five patients had metastatic disease at the time of presentation. Five-year disease-specific survivals (DSS) were 67% overall and 72% for the 40 patients presenting with localized disease at diagnosis. Nineteen patients (48%) developed metastases during follow-up. Five-year metastasis-free survival (MFS) for the 40 patients with localized disease at diagnosis was 60% and the 10-year MFS was 52%. Disease stage at presentation, tumor size >5 cm, and histologic grade 3 were univariate significant factors associated with a worse DSS. Age >or=30 years, tumor size >5 cm, necrosis, and histologic grade were univariate significant factors associated with a worse MFS. In multivariate analysis, tumor size and tumor grade remained significant prognostic factors for DSS and MFS. A role of SYT-SSX fusion type could not be confirmed in our patient group. Ezrin showed high expression in glandular and nonglandular epithelioid components in biphasic synovial sarcoma. Variable expression was found in the mesenchymal component of monophasic and biphasic synovial sarcoma. Low versus high ezrin expression levels in monophasic and/or biphasic synovial sarcoma did not correlate with patient outcome.
Disease stage at presentation, tumor size, and tumor grade were significant predictors of survival in synovial sarcoma. SYT-SSX fusion type was not correlated with survival in our series. Ezrin expression levels were not discriminative in predicting outcome.
本研究旨在探讨影响滑膜肉瘤患者生存的预后因素，包括存在争议的SYT-SSX融合类型的作用以及新提出的免疫组化标志物埃兹蛋白（ezrin）。
1984年至2005年期间，45例患者（25例男性，占56%；20例女性，占44%）被诊断为滑膜肉瘤，中位年龄为31岁（范围：2至81岁）。通过单因素和多因素方法分析诊断时的年龄、肿瘤部位、肿瘤大小、肿瘤组织学类型（双相型与单相型）、有丝分裂计数、坏死情况、组织学分级、SYT-SSX融合类型以及埃兹蛋白免疫染色对生存的影响。
所有患者的中位随访时间为55个月（范围：2至238个月）。5例患者在就诊时已有转移性疾病。总体5年疾病特异性生存率（DSS）为67%，诊断时表现为局限性疾病的40例患者的5年DSS为72%。19例患者（48%）在随访期间发生转移。诊断时表现为局限性疾病的40例患者的5年无转移生存率（MFS）为60%，10年MFS为52%。就诊时的疾病分期、肿瘤大小>5 cm以及组织学分级为3级是与较差DSS相关的单因素显著因素。年龄≥30岁、肿瘤大小>5 cm、坏死情况以及组织学分级是与较差MFS相关的单因素显著因素。在多因素分析中，肿瘤大小和肿瘤分级仍然是DSS和MFS的显著预后因素。在我们的患者组中无法证实SYT-SSX融合类型的作用。埃兹蛋白在双相型滑膜肉瘤的腺上皮和非腺上皮样成分中呈高表达。在单相型和双相型滑膜肉瘤的间叶成分中发现表达情况各异。单相型和/或双相型滑膜肉瘤中埃兹蛋白低表达与高表达水平与患者预后无关。
就诊时的疾病分期、肿瘤大小和肿瘤分级是滑膜肉瘤生存的显著预测因素。在我们的系列研究中，SYT-SSX融合类型与生存无关。埃兹蛋白表达水平在预测预后方面并无判别作用。
