Olofson Andrea M, Linos Konstantinos
Arch Pathol Lab Med. 2017 Feb;141(2):301-304. doi: 10.5858/arpa.2016-0101-RS.
Primary intraprostatic synovial sarcoma is a rare presentation of an otherwise well-studied disease, and it is one of the few primary sarcomas to occur in the prostate. Ancillary diagnostic techniques including immunohistochemistry and molecular genetics are useful to establish a definitive diagnosis. Despite its unorthodox location, it shares histologic and molecular genetic characteristics with tumors found elsewhere in the body. Most notably, the chromosomal translocation t(X;18)(p11;q11) encodes a chimeric transcription-activating protein, SS18-SSX, which has been identified as the primary driver mutation. The SS18-SSX fusion gene provides a consistent and dependable means of establishing a definitive diagnosis via reverse transcription-polymerase chain reaction or fluorescence in situ hybridization. Recent studies have continued to provide insight into the oncogenesis of this disease. The goal of this review is to elaborate on the clinicopathologic characteristics and underline those techniques that best facilitate the diagnosis of primary intraprostatic synovial sarcoma.
原发性前列腺滑膜肉瘤是一种罕见的疾病表现,而滑膜肉瘤本身是一种已有充分研究的疾病,原发性前列腺滑膜肉瘤也是少数发生于前列腺的原发性肉瘤之一。包括免疫组织化学和分子遗传学在内的辅助诊断技术有助于明确诊断。尽管其发病部位不常见,但它与身体其他部位发现的肿瘤具有组织学和分子遗传学特征。最值得注意的是,染色体易位t(X;18)(p11;q11)编码一种嵌合转录激活蛋白SS18-SSX,该蛋白已被确定为主要驱动突变。SS18-SSX融合基因提供了一种通过逆转录聚合酶链反应或荧光原位杂交来明确诊断的一致且可靠的方法。最近的研究继续为该疾病的肿瘤发生机制提供了深入见解。本综述的目的是详细阐述原发性前列腺滑膜肉瘤的临床病理特征,并强调那些最有助于诊断原发性前列腺滑膜肉瘤的技术。