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。     

55例滑膜肉瘤的治疗分析

我院自1962年12月至1984年12月收治滑膜肉瘤55例,失访1例。最小年龄10岁,最大年龄65岁,平均35岁。男36例,女19例,男:女=1.9:1。通过治疗效果探讨影响疗效的因素,广泛性切除和术后放疗的疗效无明显差异,但上皮或纤维型滑膜肉瘤对放疗有明显效果;病程,复发时间的长短,肿瘤的部位与疗效都无明显差异。但年龄越大,肿瘤越小其疗效就越好。临床Ⅰ～Ⅱ期比Ⅲ～Ⅳ期的病人疗效要好。     

Prognostic Factors in Synovial Sarcoma: A Clinicopathologic Study of 18 Cases

The prognostic factors for synovial sarcoma were analyzed retrospectivelyin 18 patients. Variables evaluated for their use in predictingsurvival included the location of the primary tumor, tumor size,local recurrence, completeness of surgical resection, and histologicsubtype. Chemotherapy was not evaluated because the patientsreceived various regimens and/or inadequate doses. Tumor sizewas the most significant prognostic factor, and a diameter ofmore than 10 cm was associated with a significantly worse prognosis(risk ratio: 5.38) (P=0.012).We conclude that complete surgicalresection of tumors less than 5 cm in maximum diameter may becurative and that adjuvant chemotherapy may not be necessaryfor such patients, because the value of chemotherapy for synovialsarcoma is still controversial.     

DNA含量与滑膜肉瘤预后关系的研究

目的：研究ＤＮＡ含量与滑膜肉瘤预后的关系。方法：采用流式细胞术对５２例滑膜肉瘤中的ＤＮＡ含量进行分析。结果：５２例滑膜肉瘤中４４例为异倍体,异倍体检出率为８４．６％。异倍体的检出率与滑膜肉瘤患者术后３年自下而上率呈明显负相关（Ｐ〈０．０５）,其与淋巴转移、骨侵袭及 肺转移呈明显正相关（Ｐ〈０．０５）。结论：ＤＮＡ含量可作为判定滑膜肉瘤口才预后的一个理想指标。     

滑膜肉瘤患者预后的多因素分析

目的:探讨影响滑膜肉瘤患者的预后因素.方法:采用免疫组织化学方法检测72例滑膜肉瘤中上皮钙粘素(E-cad)、基质金属蛋白酶-2(MMP-2)和Ki-67的表达,并对可能影响预后的14项临床病理因素进行单因素和多因素分析.结果:1)E-cad(-)、( )、( )和( )分别为11例(15.28%)、22例(30.56%)、22例(30.56%)和17例(23.60%),MMP-2(-)、( )、( )和( )分别为11例(15.28%)、18例(25.00%)、17例(23.60%)和26例(36.12%),Ki-67标记指数(LI)为2.00%～73.00%(28.30%±16.61%);2)全组术后1、3、5、7和10年生存率分别为86.44%、54.24%、28.81%、16.95%和8.47%;3)单因素分析显示,手术切除范围、转移、组织学分级、临床分期、Ki-67LI、及E-cad和MMP-2表达情况对患者预后有影响(P＜0.05);4)多因素分析表明临床分期、E-cad和MMP-2表达情况影响滑膜肉瘤的预后(X2=21.00,v=3,P=0.000).结论:临床分期、E-cad和MMP-2表达情况是影响滑膜肉瘤预后的重要因素.     

滑膜肉瘤相关发病机制的研究进展

滑膜肉瘤（synovial sarcoma, SS）是源于关节、滑膜及腱鞘滑膜的软组织恶性肿瘤,因其在关节周围的经典表现而得名。滑膜肉瘤可发生在身体任何部位,但以四肢近关节处多见。95%的SS以t（X;18）（p11.2-q11.2）染色体易位为特点,形成融合基因SYT-SSX,从而通过Wnt/β-catenin、PcG和ERK等信号通路促进SS细胞发生。另外,TGF-β1、Smad、Snail和Slug通过EMT途径也参与SS的发生发展。除此之外SS的发病还涉及有许多因子改变,如Twist1、Bmi1等。近年来研究表明上述因素均和SS的发病机制有关。本文将对这些因素在SS发生发展方面的作用作一概述。     

Salvage Treatment Experience in Advanced Synovial Sarcoma: a Multicenter Retrospective Analysis of the Anatolian Society of Medical Oncology

Background: We aimed to evaluate prognostic factors and response rates to various treatment approaches topatients with synovial sarcoma in an advanced setting. Materials and Methods: We retrospectively reviewed themedical records of 55 patients (18 pts; 32.7% women) diagnosed with synovial sarcomas. Twenty had metastaticdisease at the time of diagnosis while the remainder of the study group consisted of patients who developedmetastatic or inoperable locally advanced disease during follow up. Results: The median follow up time was 15months (range: 1-53). Regarding outcomes for the 55 patients, 3 and 5 year overall survival rates were 26% and14%, respectively. In univariate analyses among demographic factors female gender was associated with a betteroutcome (p=0.030). Patients with early progressing disease (<2 years) had a worse prognosis when comparedto patient group with late relapse, but this difference did not reach statistical significance (p=0.056). Accordingto multivariate Cox regression analysis patients who had undergone metastasectomy had a significant survivaladvantage (p=0.044). The overall response rate to different salvage chemotherapy regimens given as second linetreatment was around 42.9-53.9% for all regimes. There were no statistically significant differences betweenchemotherapy regimens given in either second or third line settings in terms of overall survival. Conclusions: Weobserved no major differences in terms of response rate and survival between different salvage chemotherapyregimens. Although metastatic disease still carries a poor prognosis, metastasectomy was found to be associatedwith improved survival     

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。     

Synovial sarcoma:a rare presentation of parapharyngeal mass

Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space.The diagnosis of synovial sarcoma can be very challenging to the pathologists.We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II.The fine needle aspiration cytology of the mass was proved inconclusive.Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma ...     

Synovial sarcoma of parapharyngeal and retropharyngeal space: A case report

Synovial sarcoma is a rare tumour of head and neck. It was first described by Pack and Ariel in 1950. Synovial sarcoma is said to be the commonest sarcoma of hands and feet and they are usually found adjacent to the articular surfaces. They rarely occur in extra-articular sites originating from bursa or tendon sheaths. Parapharyngeal space appears to be the site of predilection in most of the cases occurring in the head and neck region. One such case is being reported here along with its management and review of literature.     

软组织多形性细胞肉瘤标记特点研究

作者采用免疫组化技术对43例软组织多形性细胞肉瘤进行了多种抗体（α－AT、α－ACT、VM、DM、MG、S-100、SMA）标记，结果提示，除DM、MG及SMA对肌源性肿瘤有较大的特异性外，大多数抗体在不同组织类型的多形性细胞肉瘤中存在着交叉反应。作者认为，由于软组织肿瘤的多分化潜能，其组织类型的确定仍应主要依靠形态学特征，而免疫络化标记只能作为辅助的诊断依据。     

Synovial sarcoma in childhood: Clinical and radiological findings

Objective: To study the clinical characteristics andradiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods: The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma. Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.     

Synovial sarcoma in children. A single centre experience

Synovial sarcoma (SS) is a rare disease in the paediatric population, predominating in adolescents. Surgery remains the best treatment strategy for resectable cases. Adjuvant chemotherapy or radiotherapy can be used but have not proven efficacy. Metastatic disease and unsuccessful surgery are major prognostic factors. We describe four cases in children aged 7–13 years old. All of them were affected by SS of upper and lower limbs, and had satisfactory resections with different outcomes in spite of receiving diverse chemotherapy regimens. One of them is at the moment under treatment for relapse, two are disease-free and another died after metastatic disease.     

头颈部滑膜肉瘤的治疗及预后分析

目的：探讨头颈部滑膜肉瘤的临床诊断和治疗方法,分析头颈部滑膜肉瘤的预后不良因素,为治疗该肿瘤提供借鉴。方法：回顾性分析1984年5月～2006年12月本院收治的8例头颈部滑膜肉瘤病例,单纯手术治疗5例,单纯放射治疗1例,手术加放射治疗1例,未治疗1例。复习国内外相同疾病文献36例。通过对所有患者病历资料的研究,得出头颈部滑膜肉瘤的诊断、治疗经验,并从中分析出影响头颈部滑膜肉瘤预后的因素。结果：头颈部滑膜肉瘤临床表现无特异性,鉴别诊断困难。头颈部滑膜肉瘤组织来源不清,明确诊断需病理学检查。肿瘤呈浸润性生长,手术切除安全范围及放射治疗野皆难以确定,治疗后经常复发,复发后的肿瘤恶性程度增加,并且远处转移机会增大,导致治疗失败。头颈部神经血管密集,造成广泛切除的局限性是影响预后的主要因素。头颈部滑膜肉瘤5年生存率小于20%,目前综合治疗是提高生存率的有效方法。结论：头颈部滑膜肉瘤目前无特殊的诊断方法,诊断需有经验的病理科医师配合免疫组织化学。单纯手术易复发,单纯放射治疗效果差,目前仍无有效的化疗方案。该病死亡率高,传统的扩大切除术配合放射治疗是目前唯一可行的治疗方法。     

Multiple Recurrence of Primary Orbital Synovial Sarcoma: Report of Two Cases and Literature Review

Synovial sarcoma (SS) is typically an aggressive malignant soft tissue tumor that mostly affects adolescents and young adults. It is extremely rare in orbit and carries a high risk of recurrence and metastasis, posing a challenge to ophthalmologists in diagnosing and managing. We present two primary orbital synovial sarcoma cases with unilateral exophthalmos and limited motility. Both male patients underwent reoperation in our hospital since tumor recurrence; the pathologic diagnoses were biphasic type and occult type, respectively. Both cases were positive for EMA and CK, and SOX-9 and INI-1 were newly discovered immune markers. Fluorescence in situ hybridization analysis (FISH) revealed a translocation of t (X; 18) (p11.2; q11.2) that was detected in case 1 but not in case 2. Both patients initially refused adjuvant therapy, developed multiple recurrences and metastasis, and eventually died of distant metastasis. We provided clinical features, imaging findings, histopathology, treatments, outcomes of these very rare cases, and a literature review, underlining the timely diagnosis and management.     

Synovial sarcoma of children and adolescents: the prognostic role of axial sites

BackgroundThe outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours.MethodsThe present study analysed a series of 115 consecutive SS patients treated in Italian paediatric protocols (period 1979–2005), mainly focusing on the 30 cases arising from ‘axial’ sites (16 head–neck, 8 trunk, 4 lung-pleura and 2 retroperitoneum).ResultsInitial gross resection was achieved in 40% of axial cases and in 80% of limb SS (p < 0.0001). Five-year EFS and overall survival (OS) were, respectively, 43.3% and 55.1% for axial SS, and 69.6% (p = 0.0068) and 84.0% (p = 0.0004) for extremity SS. Local progression/recurrence was the cause of treatment failure in 75% of relapsing patients axial disease.ConclusionsOur findings emphasise that children and adolescents with SS originating at non-extremity locations have a worse prognosis than those with limb SS. Tumour site should be considered when defining a risk-adapted treatment strategy for SS.     

Sarcoma of the buttock: a trend toward limb-saving resection

We review a series of 100 consecutive cases of malignancy treated for ascites with abdominal paracentesis. The results of treatment for ascites with intracavitary Thiotepa are reported for 23 patients. The temporary partial response rate was 8/23 (35%) patients. There were symptoms of intestinal obstruction in five patients (22%) treated with Thiotepa, and one of these patients died of intestinal obstruction. In 60% of autopsied patients there were abundant intestinal adhesions in the peritoneal cavity after Thiotepa. We concluded that Thiotepa is contraindicated for instillation into the peritoneal cavity. The median survival time for patients with carcinoma of the mammary gland was 47 days, carcinoma of the ovary 121 days, and carcinoma of the large intestine 54 days after the first abdominal paracentesis. We found that patients without clinical liver metastasis at the time of first abdominal paracentesis had almost three times longer median survival time than that of patients with metastatic liver. In four cases the immediate cause of death was a complication of an abdominal paracentesis.