滑膜肉瘤临床病理分析

目的：探讨滑膜肉瘤的临床病理诊断和鉴别诊断。方法：收集滑膜肉瘤石蜡包埋标本41例,采用组织化学染色,免疫组织化学标记和逆转录-聚合酶链式反应（RT-PCR）等方法对肿瘤进行临床病理分析,为肿瘤的诊断和鉴别诊断提供科学依据。结果：12例（29%）双相分化型,22例（54%）单相纤维型,7例（17%）低分化型。36例（88%）上皮性标记[CK和（或）EMA]和间叶性标记（Vim）均阳性,5例（12%）仅间叶性标记阳性。RT-PCR检测20例标本,其中18例（90%）有融合基因SYT-SSX表达。结论：通过常规染色组织形态观察,结合免疫组织化学标记及特异性融合基因SYT-SSX检测,能够对滑膜肉瘤做出临床病理诊断。     

腹壁滑膜肉瘤一例

腹壁滑膜肉瘤是指原发于前腹壁、腹股沟和腹膜后软组织的滑膜肉瘤，约占滑膜肉瘤２．６％［１］。由于发病部位特殊，病理形态特异，容易误诊。现报告一例并进行简单讨论。患者女，２４岁，右腹股沟疼痛１年。体检发现右腹股沟上方触及巨大肿块，活动度低，质实，无压痛，...     

罕见右肾滑膜肉瘤1例

滑膜肉瘤多见于青少年，85％～95％发生于四肢。原发于肾脏的滑膜肉瘤极其罕见。我院最近收治一例。肾脏滑膜肉瘤患者．报道如下。     

喉咽部滑膜肉瘤的临床特征

目的　探讨喉咽部滑膜肉瘤的临床特征。方法　通过复习文献并对 1例喉咽部滑膜肉瘤患者的临床资料进行分析。结果　该病例治疗结束后随访 6个月无复发。结论　喉咽部滑膜肉瘤是头颈部罕见的软组织恶性肿瘤。其诊断除根据临床表现外 ,主要依靠病理学检查。治疗采用以手术为主的综合治疗。预后受多种因素影响。     

腹膜后滑膜肉瘤6例临床病理及分子病理学分析

目的:探讨腹膜后滑膜肉瘤的临床病理特征、免疫表型及分子遗传学改变。方法:收集北京大学国际医院病理科2014年12月至2021年07月期间手术切除腹膜后标本病理诊断为滑膜肉瘤6例,进行光镜观察、免疫表型及分子病理学分析,并复习相关文献。结果:男性2例,女性4例,年龄12~56岁（平均年龄29岁,中位年龄26岁）;肿瘤大小8~20 cm,平均15.2 cm,界限较清,梭形细胞型5例,双相型1例;CKpan、EMA、Bcl-2、CD99、TLE1、Vimentin、CD34、Calponin阳性比例分别为5/6、6/6、6/6、3/6、6/6、5/6、0/6、4/6,INI1呈特殊模式表达6/6,Ki-67高表达;FISH结果显示6例均有SS18-SSX基因融合;3例为SS18-SSX2基因融合,并有伴随基因突变。结论:腹膜后滑膜肉瘤是少见的发病部位,明确诊断常需结合组织病理学形态、免疫组织化学表达及分子遗传学特征进行综合分析。     

染色体易位与滑膜肉瘤的关系研究进展

滑膜肉瘤是一种高度恶性的软组织肿瘤，其诊断和鉴别诊断有一定困难。大多数滑膜肉瘤中存在特异性的X染色体与18号染色体的相互易位。本文综述染色体易位与滑膜肉瘤诊断、组织学分型及预后的关系。     

四肢软组织脂肪肉瘤MRI表现与组织分化的关系

探讨四肢软组织脂肪肉瘤ＭＲＩ表现与组织分化程度的关系,方法回顾性分析６例脂肪肉瘤ＭＲＩ术前图像,与术后病理组织分化程度相对照评价ＭＲＩ在肿瘤的大小,形态、边界,信号特点和邻近组织结构侵犯的情况,以找出它们间的关系。     

细胞增殖微血管密度及临床病理学参数对滑膜肉瘤复发的影响*

目的：探讨肿瘤细胞增殖、凋亡、微血管密度及临床病理学参数对滑膜肉瘤患者复发的预后意义。方法：选择2006年1 月至2009年1 月期间本院临床及随访资料完整并在随访期间未见转移的滑膜肉瘤56例,采用免疫组织化学染色检测Ki-67和CD31的表达,应用TUNEL染色检测肿瘤细胞凋亡情况,比较上述因素及临床病理学参数与复发的关系,并分析它们对患者无复发生存的影响。结果：1）在随访期间41例（73.2%）患者出现复发,中位无复发生存时间为19.5 个月,患者术后1、2、3、4、5 年的无复发生存率分别为45.0% 、41.0% 、34.0% 、28.0% 和28.0% 。2）56例滑膜肉瘤中,Ki-67标记指数为19.98%±11.64% ,微血管密度为51.83± 21.92个/高倍视野（× 400）,凋亡指数为0～9.0% 。3）组织学类型（P=0.000）和微血管密度分级（P=0.045）在复发与无复发组之间差异有统计学意义。4）单因素分析结果显示,Ki-67表达（P=0.009）、组织学类型（P=0.012）、放疗（P=0.014）与滑膜肉瘤患者的无复发生存有关。5）多因素分析结果表明,组织学类型（RR= 0.207,P=0.031）、Ki-67表达（RR= 1.944,P=0.045）和放疗（RR=0.482,P=0.04）是滑膜肉瘤复发的独立危险因素。结论：组织学类型和微血管密度可能影响滑膜肉瘤的复发,并且组织学类型和肿瘤细胞增殖情况可能影响滑膜肉瘤患者的无复发生存。     

肺滑膜肉瘤1例

1临床资料患者,男,27岁,因＂左侧血气胸＂入院。患者2010年6月因＂感冒＂出现咳嗽、咳少量白色稀痰,无畏寒、发热等不适,未引起重视;半个月后,出现左胸顶部疼痛,伴呼吸困难,遂到当地日喀则地区人民医院住院治疗,入院后行胸部CT检查提示左侧大量胸腔积液,给予抽液、抗感染、止血和对症治疗,并行胸腔闭式引流术,每天引流出血性液体约100ml,     

滑膜肉瘤107例分析

本文报道107例滑膜肉瘤,均经手术、病理证实.除17例术前明确诊断外,余90例被误诊为其它肿瘤,甚至关节炎及关节结核等,误诊率为84.1%.为提高早期诊断符合率,根据本组资料,重点分折了误诊原因,并提出有助于早期诊断的临床和X线依据.     

Early detection of a deep-seated synovial sarcoma by magnetic resonance imaging

An 18-year-old man who complained of left thigh pain was referred to our institute. No abnormal findings were detected on physical examination and plain roentgenogram or gallium scintigram. On magnetic resonance imaging (MRI), a high-signal of 1-cm diameter was seen in the left vastus medialis muscle on T1- and T2-weighted images. The lesion was initially considered to be: (1) angiolipoma, which is usually associated with fatty tissue, based on the MR findings or (2) a sport-induced inflammation based on the patient's history of baseball playing. Because of the persistent thigh pain and similar findings on the second MRI, an excisional biopsy was performed; the lesion was diagnosed histologically as synovial sarcoma. Additional wide resection was carried out later, without adjuvant therapy. He has been disease-free for 3 years postoperatively. Many soft tissue sarcomas are discovered with extensive tumor formation and are accompanied by obvious abnormal findings on various imaging studies. It is extremely rare that deep-seated sarcomas are discovered without obvious abnormalities. To the best of our knowledge, this is one of the smallest detected deep-seated soft tissue sarcomas. MRI was the most sensitive and useful procedure for detecting the lesion. Physicians should recognize that painful lesions may possibly be synovial sarcoma, even if they are demonstrated as a subtle lesion of 1-cm diameter on MRI.     

The place of radiotherapy in the treatment of synovial sarcoma

This paper reviews 36 patients with synovial sarcoma; 18 were referred within three months of surgery. None had undergone “en bloc” excision and all were treated with post-operative radiotherapy. Local control and survival were analyzed with respect to Tumor Node Metastasis Classification, histology, site of primary and surgical procedure. Eight patients with TI-2NOMO tumors were alive and well (minimum two year follow-up) following excision and radiotherapy; 7 had a normally functional extremity. In contrast, only one of 8 patients with T3NOMO tumors is alive and well. Seven of 8 patients with well or moderately differentiated histology were alive and well whereas no patient with poorly differentiated histologies survived. Six of 7 patients were alive and well if their honor was distal to the elbow or knee whereas none of those who had a primary thigh synovlal sarcama survived. Eighteen patients were referred with recurrent disease and 2 were salvaged. A management policy is proposed for synovial sarcomas with the integrated use of surgery, radiation and chenotherapy; it emphasizes optimal cure rata and a functional extremity reserving amputation for salvage.     

mTOR信号通路与滑膜肉瘤研究进展

哺乳动物雷帕霉素靶蛋白(Mammalian target of rapamycin,mTOR)是一种丝/苏氨酸蛋白激酶,属于磷脂酰肌醇三激酶-相关激酶家族,通过影响Akt、4EBP1和S6K1等关键因子在肿瘤的发生发展中扮演重要角色.滑膜肉瘤(Synovial sarcoma,SS)是一种恶性程度极高的软组织肉瘤,早期外科手术及化疗是目前主流的治疗方式,但晚期生存率仍不理想,通过对信号通路转导的研究为滑膜肉瘤的治疗提供了新的研究方向.本文对近年来mTOR与滑膜肉瘤的相关研究进行综述.     

Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy

PURPOSE: To evaluate prognostic factors and treatment outcomes in patients with localized synovial sarcoma treated with conservation surgery and radiotherapy (RT). METHODS AND MATERIALS: Between 1960 and 2003, 150 patients with nonmetastatic synovial sarcoma were treated with conservation surgery and RT. The majority of patients (81%) were aged >20 years. Sixty-eight percent received postoperative RT, and 32% received preoperative RT. Forty-eight percent received adjuvant chemotherapy. RESULTS: Median follow-up was 13.2 years. Overall survival (OS) rates at 5, 10, and 15 years were 76%, 57%, and 51%, respectively. Corresponding disease-free survival (DFS) rates were 59%, 52%, and 52%, respectively. Tumor size >5 cm predicted worse OS, DFS, disease-specific survival (DSS), and higher rate of distant metastases (DM). Age >20 years predicted worse DFS and DSS but not OS. Local control (LC) was 82% at 10 years. Positive or unknown resection margins predicted inferior LC rates. Forty-four percent developed DM by 10 years. Only 1% developed nodal metastases. Analysis of outcomes by treatment decade showed no significant differences with respect to LC and DM rates. CONCLUSIONS: Synovial sarcoma is adequately controlled at the primary site by conservation surgery and RT. Elective nodal irradiation is not indicated. Rates of development of DM and subsequent death from disease remain high, with no significant improvement in outcomes for this disease in the past four decades.     

Sentinel node biopsy in synovial sarcoma

Aims

To examine the relevance of sentinel node biopsy in patients with synovial sarcoma.

Methods

Between July 2004 and February 2007 11 consecutive patients with synovial sarcoma treated in our clinic underwent sentinel node biopsy after a preoperative lymphoscintigraphy. A handheld γ-probe was used during the procedure to identify the sentinel nodes, which were then resected and submitted for histopathologic evaluation.

Results

At least one sentinel node was identified in every patient. Of a total of 15 sentinels, one was positive and 14 negative. The patient with the positive sentinel underwent a regional lymph node dissection and remains disease-free 17 months later. One patient developed regional nodal metastases despite negative sentinel node biopsy and died 12 months after the procedure. No biopsy-associated complications were observed.

Conclusions

Sentinel node biopsy can be successfully and safely applied to patients with synovial sarcoma. Further prospective studies are required to determine the optimal treatment approach, the false negative rate and the prognostic significance of a positive sentinel node biopsy.     

Intracranial synovial sarcoma: A clinical,radiological and pathological study of 16 cases

IntroductionSynovial sarcoma (SS) is a tumor of unknown origin and is extremely rare in the central nervous system. Most studies on intracranial SS included only one or two cases. To better understand the disease, we review a series of primary intracranial SS.Method and materials16 primary intracranial SS in Tiantan Hospital during 2008–2017 were included. The clinical characteristics, including radiological and histological examination, operative records, and prognoses were reviewed.ResultThe case series included nine male and seven female patients with an average age of 23.8 years. Radiological results showed that the supratentorial region (81.25%) was the most common site of the brain involved. All patients were misdiagnosed as non-SS tumors. Gross total resection (GTR) was achieved in 12 cases (75.0%), and subtotal resection (STR) was achieved in 4 cases. All cases showed the characteristic SYT-SSX fusion gene, as detected by RT-PCR. The mean progression-free survival time (PFS) was 10.0 months and the mean overall survival time (OS) was 15.5 months. Multivariate analysis revealed that GTR and postoperative adjuvant radiotherapy were independent factors for PFS (HR = 6.143, 95% CI = 1.491–25.312; P = 0.012, HR = 6.143, 95% CI = 1.491–25.312; P = 0.012 respectively) and OS (HR = 9.000, 95% CI = 1.627–49.773; P = 0.012, HR = 0.017, 95% CI = 0.001–0.213; P = 0.002 respectively).ConclusionIntracranial SS were more frequently observed in the supratentorial region and in young patients without sex predilection. We recommend adjuvant radiation regardless of the extent of resection. More patients and longer follow-up periods were needed to further elucidate the biological features of intracranial SS.     

心包原发性滑膜肉瘤1例报道

Primary pericardial sarcomas are extremely rare. We report a case of 19 year old male who presented with cough, dyspnoea, and orthopnea. Investigations and exploratory thoracotomy revealed a large pericardial mass. Surgical debulking of the tumor was performed and the histopathological examination was compatible with synovial sarcoma. The tumor was unresectable due to its invasion and adhesion to mediastinal structures. Hence patient was started on palliative chemotherapy (adriamycin and ifosfamide based). ...     

Trabectedin is a promising antitumour agent for synovial sarcoma

Synovial sarcoma (SS) is an aggressive soft tissue tumour with poor prognosis. Using five human SS cell lines, we examined the cytotoxic effects of trabectedin (ET-743; Yondelis^®), a novel marine natural product, which was approved in Europe for the treatment of soft tissue sarcomas (STS). The significant growth inhibitory effects were observed in all SS cell lines below nanomolar concentration of trabectedin. Furthermore, trabectedin significantly suppressed the tumour growth in xenograft models. Flow cytometer analysis in vitro and immunohistochemical analysis in vivo revealed its effect of cell cycle inhibition and apoptosis induction. We also examined the expression of ERCC1, 5 and BRCA1 in SS cell lines and clinical samples, and majority of them showed highly trabectedin-sensitive pattern as previously reported in other cancers. Our preclinical data indicated that trabectedin could be a promising therapeutic option for patients with SS.     

Neutrophil/lymphocyte ratio is associated with survival in synovial sarcoma

Background

Synovial sarcoma is a rare malignant soft tissue tumor, more common in adolescents and young adults and entails a poor prognosis. Several good prognostic factors have been well established such as age less than 25, size less than 5?cm and absence of a poorly differentiated component. Inflammation has a well-established role in tumor proliferation and survival. The aim of this study was to investigate the prognostic significance of the neutrophil/lymphocyte ratio (NLR) in a large cohort of synovial sarcoma patients.