12例孤立性纤维性肿瘤临床病理分析

目的:探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理特点、免疫表型、鉴别诊断、生物学行为及预后。方法:收集12例孤立性纤维性肿瘤的临床病理资料和免疫组化染色,并结合文献进行分析讨论。结果:12例孤立性纤维性肿瘤平均发病年龄为40.2（29-70）岁,多发生于胸腔、腹腔,其中1例为恶性SFT。镜下肿瘤细胞多为梭形细胞,少细胞区和多细胞区相间,两者间隔以瘢痕样透明变性粗胶原纤,可见分枝状的血管外皮瘤样区。免疫表型:梭形细胞Vimentin、CD34和CD99弥漫强阳性,不同程度表达Bcl-2、SMA、EMA、Desmin、S-100。结论:SFT属于中间生物学行为的肿瘤,可复发或转移。少数SFT虽然具有实体富于细胞纤维肉瘤样模式,但其生物学行为仍然相对良性。一些组织学良性的SFT在术后10年也可复发和转移,“去分化SFT”预后差。     

孤立性纤维性肿瘤6例临床病理分析

目的探讨孤立性纤维性肿瘤（SFT）的临床及病理形态特征及免疫组化特点。方法结合文献对6例孤立性纤维性肿瘤的组织学、免疫组化特点进行分析。结果患者年龄37～65岁,平均50.1岁,病程2个月～2年,临床表现主要为局部肿块及压迫症状,肿瘤直径为3.0～5.0cm。免疫组化结果：CD34阳性6例,bcl-2阳性6例,Vim阳性6例,CD99阳性5例,desmin阳性2例,FVIII均阴性。结论SFT的临床诊断应结合临床特点、组织学形态、免疫组化特点综合考虑,其组织学构像并不能准确地判断其预后,患者术后的定期随访是必要的。     

腹腔孤立性纤维性肿瘤

孤立性纤维性肿瘤(solitary fibrous tumor,SFT)是一种罕见肿瘤,因其组织学的复杂性、多样性,临床上易误诊.本研究就我院1例SFT结合文献对其组织起源、诊断、手术及其预后等进行讨论.     

孤立性纤维性肿瘤3例临床病理分析

孤立性纤维性肿瘤(Solitary fibrous tumor,SFT),为不常见的独特性梭形细胞肿瘤。     

腹膜后恶性孤立性纤维性肿瘤的临床病理分析

目的探讨腹膜后恶性孤立性纤维性肿瘤(MSFT)的临床病理特征、病理诊断、治疗和预后。方法收集2002年1月至2018年2月确诊的7例MSFT患者的临床病理资料,对其临床表现、组织病理学特征、免疫表型、治疗及预后进行分析。结果 7例患者中男性4例,女性3例;中位年龄51岁(范围:22~73岁)。所有患者均接受根治性手术切除。2例患者术后接受异环磷酰胺联合表柔比星方案化疗6个周期,1例接受顺铂联合丝裂霉素术中腹腔热灌注化疗。随访截止于2019年1月,1例无瘤生存,2例死亡,4例带瘤生存;4例复发2次,1例复发4次。MSFT病理特征为肿瘤界限清楚,可有包膜或者假包膜,肿瘤边缘浸润性生长,可见"鹿角状"血管,有坏死和出血,肿瘤细胞丰富,细胞呈中度至重度异型性,核分裂象多见(≥2个/10HPF)。CD34、Bcl-2、STAT6、CD99和Vimentin蛋白均呈阳性表达。结论腹膜后MSFT确诊主要依赖病理形态学和免疫表型。MSFT术后易复发,需长期随访。     

115例颅外孤立性纤维性肿瘤的临床病理学分析

目的 探讨孤立性纤维性肿瘤(SFT)的临床病理学特征、分子遗传学特征及预后。方法对南京医科大学第一附属医院病理学部2010年1月至2020年12月诊断的115例颅外SFT进行回顾性研究,进行组织学观察、免疫组化检测、分子遗传学检测及随访。结果 其中,男性45例,女性70例,年龄19～82岁,中位年龄54岁,发生部位最常见于肺/胸膜,肿瘤最大径为1～25 cm,中位数6 cm,组织学形态多数表现为经典型SFT,由梭形或卵圆形肿瘤细胞及背景胶原和“鹿角形”血管构成;此外,还有2例脂肪瘤型、8例黏液型、2例巨细胞型、4例去分化型。免疫组化显示为特征性的STAT6、CD34弥漫阳性,少数病例表现出Desmin、CK-pan、PAX2、PAX8等抗体的反常表达。分子遗传学上经NGS检测的病例均具有NAB2::STAT6融合基因。12例伴有TERT启动子,4例出现TP53基因突变。术后随访时间为0～136个月(中位随访时间45个月),有10例复发,5例转移,4例出现肿瘤相关性死亡。结论 依据典型的组织学形态和特异的免疫组化表型可以对SFT进行诊断,但对于特殊形态或特殊免疫表型的SFT诊断需注意诊断...     

膀胱孤立性纤维性肿瘤1例

患者,男性,52岁,2004年7月因尿频、尿急入院,影像学发现下腹部盆腔实质性占位,可能来源于腹膜后.术中发现肿物起源于膀胱,与膀胱颈部紧密粘连,向外呈膨胀性生长,压迫乙状结肠、前列腺、双侧输尿管.完整切除肿物后送病理检查.     

原发性子宫孤立性纤维性肿瘤1 例

患者女性,41岁,因“月经量增多1年”于2014年12月21日就诊绍兴市中心医院。妇科检查发现子宫前位增大如孕2个月,活动无压痛。双侧附件区未扪及包块及压痛。超声检查发现子宫中部可见一个低回声团,大小为5.1 cm×3.3 cm,位于内膜下方,双附件未见明显异常回声,考虑黏膜下肌瘤。临床诊断为子宫肌瘤,于2014年12月22日在腹腔镜下行子宫肌瘤剔除术,术中可见肿瘤位于子宫后壁黏膜下,表面光滑,大小为5 cm×4 cm,肿物边界清楚。镜下可见肿瘤由梭形细胞、胶原基质和丰富的血管构成,细胞丰富区和细胞疏松区交替分布,核无异型性,核染色质疏松,未见核分裂及坏死,部分血管呈不规则鹿角状,可见血管外皮瘤样结构（图1）。免疫组织化学法显示CD34、CD99、Bcl-2弥漫阳性表达,Ki-67增殖指数约10%（+）,Desmin灶性阳性,CD10、S-100、SMA、CD117、CK、EMA、DOG-1均阴性表达。术后病理诊断为子宫孤立性纤维性肿瘤。随访15个月未见肿瘤复发,患者情况良好。     

胸腔恶性孤立性纤维性肿瘤1例

患者女性,29岁,主因间断腹部、后背隐痛不适3月余于2010年11月入院.查体:双肺呼吸音清,右上腹轻压痛,未触及明显包块.     

A malignant solitary fibrous tumor in the retroperitoneum

Solitary fibrous tumor in the retroperitoneum is rare. We report a case of a malignant solitary fibrous tumor in the retroperitoneum, with a lymph node metastasis, in a 48-year-old-woman. Computed tomography and magnetic resonance imaging indicated a mass in the retroperitoneal space beside the left kidney, with a swollen paraaortic lymph node. Surgical findings revealed a circumscribed tumor in the retroperitoneum. A pathohistological examination revealed two separate components: a benign hypocellular area rich in collagen fibers and a malignant hypercellular area with nuclear atypia. The histological finding for the lymph node was the same as that in the hypercellular area. Because of these findings, the mass was diagnosed as malignant solitary fibrous tumor. Only surgical treatment was performed, and the patient is alive without recurrence 2.5 years after the surgery. Immunohistochemical staining for p53 was positive in the malignant component but negative in the benign area, indicating that the expression of p53 may play a critical role in the malignant transformation of solitary fibrous tumor.     

Clinical outcomes of extra-thoracic solitary fibrous tumours

Background

Extra-thoracic solitary fibrous tumours (ESFTs) have traditionally been regarded as indolent neoplasms similar to their intra-thoracic counterparts. However there has been some evidence that a subset of more aggressively malignant tumours exist. We examined our experience with these rare tumours in an effort to clarify their clinico-pathological behaviour and relate this to their histopathological findings.

Patients/Methods

All patients with a histopathological diagnosis of solitary fibrous tumour (SFT) who presented to the Royal Marsden Hospital between 1998 and 2006 were reviewed. Clinico-pathological data were recorded for all cases and subset analysis performed to compare rates of locoregional recurrence, distant metastases and death.

Results

There were 33 cases included in the study. 18 cases had malignant features on histological examination. Locoregional recurrent disease was more common in those with malignant histopathological findings compared to those with benign histopathology (6/18 vs 0/15 p 0.021). Distant metastatic disease was more common in those with malignant histopathological findings (7/18 vs 1/15 p 0.046) and these patients were at increased risk of death (10/18 vs 0/15 p < 0.01). The presence of malignant histopathology was the only factor to affect survival with no benign cases dying of disease and malignant cases having a median survival of 59 months (p 0.003).

Conclusion

In our experience ESFTs have a higher rate of malignant behaviour than that classically described. Those tumours with atypical or malignant features on histological examination have poor prognosis and should be managed and followed up in the same manner as other high-grade soft tissue tumours.     

The therapeutic challenge of a nonresectable solitary fibrous tumor in a hypoglycemic patient

We report a patient with a nonresectable histologically benign solitary fibrous tumor who suffered from paraneoplastic non-islet cell tumor hypoglycemia (NICTH). Diagnostic workup revealed malignant characteristics in which the tumor showed up as, presumably, false-negative on fluorodeoxyglucose-positron emission tomography (FDG-PET), while being positive on tyrosine-PET. Neoadjuvant treatment, which consisted of combined chemo-radiation and consecutive selective embolization of the tumor feeding vessels, caused such a therapeutic effect, on both NICTH and reduction in tumor volume, that a secondary resection, with the patient in a normoglycemic status, was possible. Our report highlights several important issues in the management of the patient with a nonresectable solitary fibrous tumor with severe episodes of hypoglycemia due to NICTH.     

A case of solitary fibrous tumor of the meninges

We report a rare case of solitary fibrous tumor (SFT) of the meninges of the posterior fossa presenting as an intracerebellar hemorrhage. A 29-year-old woman was admitted with sudden-onset severe headache, nausea, and vomiting. A computed tomographic (CT) scan of the brain revealed an intracerebellar hemorrhage 3.5 cm in diameter. Gadolinium-enhanced magnetic resonance imaging (MRI) showed a heterogeneous enhancement mass. A posterior craniotomy found a firm, highly vascular tumor attached to the meninges. Histologically, the tumor showed mostly sclerotic tissues with spindle cells. In few areas, the tumor had a more compact arrangement of spindle-shaped cells with vascular spaces and highly cellular components. Immunohistochemical study revealed strong CD-34 immunopositivity in many tumor cells. The tumor was diagnosed as SFT of the meninges. We report the clinical and histological features of this newly described tumor with a heterogeneous component.     

The demographic features,clinicopathologic characteristics,treatment outcome and disease-specific prognostic factors of solitary fibrous tumor: a population-based analysis

Background

Solitary fibrous tumor''s (SFT) demographic features, clinicopathologic characteristics, treatment outcome and disease-specific prognostic factors were unexplored comprehensively.

Methods

SEER program was used to identify patients diagnosed with SFT from 1973 to 2012. Overall collected data were analyzed by using the SPSS 18.0.

Results

In total, 804 cases were found including 613 cases with SFT-specific mortality and 801 patients were analyzed for overall survival (OS). The 3-year disease specific survival (DSS), 5-year DSS and 10-year DSS were 73.3%, 65.7% and 53.3%. The 3-year OS, 5-year OS and 10-year OS were 71.9%, 63.3% and 47.3%. In the multivariate survival analysis, the age > 51 years (hazard ratio [HR] = 1.851 for DSS, P = 0.024 and HR = 1.652 for OS, P = 0.033; Reference [Ref] ≤ 51 years for DSS and ≤ 53 years for OS), SEER stage metastasized tumor (HR = 4.269 for DSS, P = 0.000 and HR = 2.905 for OS, P = 0.028, Ref - localized + regional tumor), pathologic grade III + IV (HR = 2.734 for DSS, P = 0.001 and HR = 2.585 for OS, P = 0.000, Ref - grade I + II) were adversely associated with DSS and OS. In addition, surgery was favorably associated with DSS (HR = 0.217, P = 0.045, Ref - surgery + radiotherapy).

Conclusions

The surgery was an independent prognostic factor for DSS. The patient''s age, SEER stage and pathologic grade were SFT-specific independent prognostic indicators for DSS and OS.     

Recurrent intracranial solitary fibrous tumor initially diagnosed as hemangiopericytoma

We describe a case of an intracranial solitary fibrous tumor that recurred three times consecutively in an 11-year period. A 72-year-old man presented with a headache and gait disturbance. Magnetic resonance imaging (MRI) revealed a dumbbell tumor at the left tentorium. The tumor was removed but recurred. The first diagnosis was hemangiopericytoma, but all specimens showed a “patternless pattern” and few reticulin fibers, which features were not compatible with hemangiopericytoma. All tumors showed immunoreactivity for CD34 and bcl-2. These results point to a solitary fibrous tumor (SFT) and not to hemangiopericytoma. We present here a hypercellular spindle-cell tumor that was very similar to hemangiopericytoma but is better diagnosed as SFT.     

Solitary fibrous tumor of the liver: retrospective study of reported cases

A solitary fibrous liver tumor is a rare disease that is difficult to diagnose. Radiological findings are not specific and cannot confirm benignity or malignancy. Immunohistologically, CD 34, Vimentin, and Desmin should be used as markers to precisely diagnose solitary fibrous tumors. In most cases, there is low cellularity with no cellular atypia or necrosis, making this a benign lesion. Occasionally, a large size, high mitotic rate, cellular pleomorphism and atypia, and necrosis are interpreted as features suggestive of an increase malignant potential. The outcome of solitary fibrous tumor mostly is related to resectability, although correlated with neither pathological grade nor tumor size. Thus, total surgical resection of the neoplasm is most commonly proposed. Physicians should be alerted that solitary fibrous tumor of the liver can be malignant and can metastasize.     

孤立性纤维瘤的影像学表现与临床病理特征

目的 探讨孤立性纤维瘤(SFT)的影像学特点、临床表现和病理学特征.方法 回顾性分析27例经病理确诊的SFT患者的影像学表现、临床特征和病理学表现.结果 27例SFT中,发生于胸部18例,腹部4例,腰骶部1例,盆腔3例,左肩后部1例.恶性8例,良性17例,交界性2例.CT扫描23例,均见包膜,其中16例良性SFT为边界清楚的类圆形肿块,大部分密度均匀,与周围组织分界清楚;6例恶性SFT边界不清,与周围组织粘连或侵犯周围组织,密度不均匀;1例交界性SFT边界清楚,与周围组织分界清楚,可见蛋壳样钙化.MRI扫描4例,肿瘤边界清楚,信号欠均匀,T1WI序列呈等或略高于肌肉组织信号,T2WI序列呈稍高信号,其中恶性SFT中可见不规则坏死区.27例SFT在增强扫描时均呈不均匀强化,强化程度不一,呈中至重度强化.免疫组化检查结果显示,CD34(+)22例,占81.5%;Vimentin(+)27例,占100.0%;CD99(+)27例,占100.0%;bcl-2(+)26例,占96.3%;CK(-)27例,占100.0%;S-100(-)26例,占96.3%.结论 SFT的影像学表现具有一定的特征性,病理形态学及免疫组织化学检查可以明确诊断.CT和MRI可以对SFT进行明确的定位,确定肿瘤与周围组织的关系,为手术切除提供帮助.