CAJ | 학술논문

背景与目的:肾脏原发性滑膜肉瘤少见,容易误诊为其他肾脏肿瘤.本研究探讨该肿瘤的临床病理学特点、免疫组化特征、鉴别诊断及患者预后.方法:选取山东临沂市肿瘤医院病理科诊断的4例肾脏原发性滑膜肉瘤患者,男性2例,女性2例,年龄32～48岁.复习其组织学切片、临床资料并选取典型蜡块进行免疫组化染色,通过逆转录PCR(RT-PCR)方法对其中2例进行SYT-SSX融合基因的分子生物学检测.结果:肿瘤大小为10～15 cm,平均12 cm.大体观察:肿瘤体积较大,边界不清,切面灰白色实性,可见出血坏死.其中3例切面可见散在的小囊腔.显微镜检查:肿瘤由胖梭形细胞构成,分裂相多见,成短不状交错排列;4例均可见细胞稀疏黏液变性区域和血管外皮瘤样区域.部分区域细胞圆形或卵圆形,弥漫排列.肿瘤细胞分裂相平均5～8个/10HPF,出血坏死易见.3例可见散在分布的小囊腔,衬覆扁平、立方或者鞋钉样细胞.免疫组化显示肿瘤细胞Vimentin阳性(4/4),Bcl-2阳性(4/4),CD99阳性(4/4),CD56阳性(3/4);EMA(epithelial membrane antigen)、CK(cytokeratin)局灶阳性(3/4).2例行RT-PCR检测病例均发现SYT-SSX1基因融合片段.4例中1例术后6个月发生肺转移,发现转移后5个月后死亡.1例发生肝转移,术后13个月死亡.另外2例术后8个月和15个月复发,分别于术后18个月、21个月死亡.结论:肾脏原发性滑膜肉瘤少见,预后差,常常伴有SYT-SSX基因融合,需要与其他原发于肾脏的肉瘤鉴别.
배경여목적:신장원발성활막육류소견,용역오진위기타신장종류.본연구탐토해종류적림상병이학특점、면역조화특정、감별진단급환자예후.방법:선취산동림기시종류의원병이과진단적4례신장원발성활막육류환자,남성2례,녀성2례,년령32～48세.복습기조직학절편、림상자료병선취전형사괴진행면역조화염색,통과역전록PCR(RT-PCR)방법대기중2례진행SYT-SSX융합기인적분자생물학검측.결과:종류대소위10～15 cm,평균12 cm.대체관찰:종류체적교대,변계불청,절면회백색실성,가견출혈배사.기중3례절면가견산재적소낭강.현미경검사:종류유반사형세포구성,분렬상다견,성단불상교착배렬;4례균가견세포희소점액변성구역화혈관외피류양구역.부분구역세포원형혹란원형,미만배렬.종류세포분렬상평균5～8개/10HPF,출혈배사역견.3례가견산재분포적소낭강,츤복편평、립방혹자혜정양세포.면역조화현시종류세포Vimentin양성(4/4),Bcl-2양성(4/4),CD99양성(4/4),CD56양성(3/4);EMA(epithelial membrane antigen)、CK(cytokeratin)국조양성(3/4).2례행RT-PCR검측병례균발현SYT-SSX1기인융합편단.4례중1례술후6개월발생폐전이,발현전이후5개월후사망.1례발생간전이,술후13개월사망.령외2례술후8개월화15개월복발,분별우술후18개월、21개월사망.결론:신장원발성활막육류소견,예후차,상상반유SYT-SSX기인융합,수요여기타원발우신장적육류감별.
Background and Objective:Primary renal synovial sarcoma is rare and might be misdiagnosed as another renal tumor.This study demonstrates the clinicopathologic and immunohistochemical features,differential diagnosis,and prognosis of such tumors.Methods:Histologic slides and clinical data were reviewed for 4 patients with primary renal synovial sarcoma and immunohistochemical staining was performed. Molecular analysis was performed on 2 cases to demonstrate the presence of the SYT-SSX gene fusion transcripts by reverse transcriptase polymerase chain reaction(RT- PCR).Results:The patients were 2 women and 2 men aged from 32 to 48 years.The tumors were 10.0-15.0 cm in diameter, grey-white and solid.and hemorrhage or necrosis was observed. Microscopically,the tumors consisted of mitotically active, monomorphic plump spindle cells with indistinct cell borders growing in short, intersecting fascicles.Hypocellular myxoid areas and a prominent hemangiopericytomatous pattern were present in all cases.The average mitotic rate was 5-8 mitoses/10 high.power fields.Hemorrhage and tumor necrosis were easily found. Scanered small cyrsts lined with flat,cuboidal,or hobnailed epithelia were found in 3 cases.Tumor CD56(3/4),and focally for EMA(3/4)and Cytokeratin(3/4).SYT.SSXl gene fusion was detected in the 2 cases in which RT-PCR analysis was performed. One patient had tumor metastasis to the lung 6 months after surgery and died 5 months later.Multiple metastasis to the liver occurred in one patient and the patient died 1 3 months after the initial surgery.The other 2 patients had tumors recur at 8 and 15 months and died at 18 and 21 months,respectively,after the initial operation.Conclusion:Primary renal synovial sarcoma is rare, with poor prognosis, characterized by SYT-SSX gene fusion,and needs to be differentiated from other renal sarcomas.