恶性孤立性纤维性肿瘤4例临床病理分析

目的探讨恶性孤立性纤维性肿瘤(malignant solitary fibroustumor,MSFT)的临床病理特征、组织发生、诊断、鉴别诊断、治疗及预后。方法报道4例罕见的MSFT,并结合文献进行复习。结果女性2例,男性2例,年龄22岁～63岁,中位年龄42.5岁。发生部位:中枢神经系统2例,临床表现为头痛、头晕和恶心等;胸腔2例,无明显症状,体检发现。肿块直径3～14cm,平均8.5cm。镜检:低倍镜下肿瘤细胞分布疏密不均。密集区肿瘤细胞丰富,呈束状、漩涡状或不规则状排列,可见血管外皮瘤样结构;瘤细胞梭形、短梭形,核染色质粗且不均匀,异型性明显,核分裂象多见(4个/10HPF),局灶出血坏死。疏松区细胞稀少,纤维化伴黏液变性。免疫表型:肿瘤细胞Bcl-2、CD99、vimentin弥漫阳性;CD34、MSA、α-SMA、h-caldesmon局灶阳性,Ki-67增殖指数25%～50%;EMA、CD117、GFAP和S-100阴性。结论 MSFT非常罕见,确诊主要依赖病理形态学和免疫表型,需与滑膜肉瘤、脑膜瘤、恶性外周神经鞘膜瘤、恶性纤维组织细胞瘤和纤维肉瘤等相鉴别。MSFT的预后不佳,易复发和转移,手术完整切除后,应注意随访。     

恶性孤立性纤维性肿瘤4例临床病理分析并文献复习

目的：探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)临床病理特点、诊断和鉴别诊断及治疗预后。方法：对4例MSFT回顾并分析其临床病理特点,并复习相关文献。结果：男性2例,女性2例,发病年龄43~64岁。肿瘤部位分别为胸腔内、乙状结肠系膜、右肩部、左足。镜下肿瘤呈细胞丰富区与稀疏区相间的特点,局灶形态类似血管外皮瘤。瘤细胞梭形或短梭形,异型性明显,核分裂像易见,局灶可见坏死。免疫组化：瘤细胞表达Vim、CD34、CD99、Bcl-2,不表达CK、S-100。结论：MFST少见,确诊依靠形态学和免疫组织化学,需与血管外皮瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤及恶性外周神经鞘瘤相鉴别。MFST易复发和转移,手术完整切除是其主要治疗手段,需长期随访。     

恶性孤立性纤维性肿瘤临床病理及分子遗传学分析

目的探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor, MSFT)的临床病理、免疫表型及分子遗传学特征。方法收集2018年7月至2020年12月郑州大学第一附属医院诊断的MSFT病例7例, 并行免疫组织化学及RNA-based和DNA-based二代测序检测。结果 7例患者中, 男性5例, 女性2例;中位年龄53岁(37～69岁);肿瘤原发于颅底2例, 原发于小脑幕、顶枕叶、枕部、胸腔、臀部各1例;肿瘤最大径2.5～20.0 cm。镜下可见典型血管外皮瘤样结构, 细胞密度丰富, 呈梭形或卵圆形, 异型性大, 可见坏死和核分裂象(>4个/10 HPF), 2例可见经典孤立性纤维性肿瘤形态与去分化区域并存。免疫组织化学结果显示CD34(6/7)、STAT6(7/7)、bcl-2(7/7)阳性表达, S-100蛋白(7/7)阴性表达, 广谱细胞角蛋白或上皮细胞膜抗原均有不同程度的阳性表达, p53表现为突变型(3/7), Ki-67阳性指数均大于10%;7例均检测到NAB2-STAT6基因融合, 其中4例还分别检测到ZNF415-FGFR1、C...     

软组织恶性孤立性纤维性肿瘤2例

例1,男性,65岁,因腹膜后巨大肿瘤4个月余入院.查体:各项体检无明显阳性特征,无畏寒、发热、恶心、呕吐,精神睡眠可,大小便正常,体重下降5 kg.CT示:腹膜后可见一大小15 cm×15 cm×10 cm肿物,肿物密度不均匀,呈不均匀强化,中央低密度区未见强化;周围肠管组织被推挤,肿物周围见较多扭曲粗大血管组织.患者于2个月前在中山大学附属第一医院行手术治疗,手术所见:患者无腹水,左下腹部至盆腔腹膜后可及一巨大实性肿物,大小18 cm×15 cm×15 cm,边界尚清,肿物将膀胱推向右下方,肿物部分侵及左侧输尿管,造成左肾积水.因术中出血量较多,止血后行肿瘤部分切除.     

中枢神经系统孤立性纤维性肿瘤4例临床病理分析

目的探讨中枢神经系统孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理特征及鉴别诊断。方法回顾性分析4例中枢神经系统SFT的临床特点,行HE和免疫组化染色,镜下观察其病理组织学特征。结果 4例均为中年男性,镜下见肿瘤细胞呈梭型或胖梭形,呈席纹状排列,免疫组化标记vimentin、BCL-2、CD34阳性率为100%。结论中枢神经系统SFT需与纤维型脑膜瘤相鉴别,免疫组化标记有助于鉴别,避免误诊造成过度治疗。     

13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（SFT）的临床病理形态学和免疫组织化学特点及诊断要点。方法采用光镜及免疫组化对13例SFT进行研究。结果本组病例，男性9例，女性4例，发病年龄30-76岁，平均46．76岁，良性8例，恶性5例，发生于9个部位，其中胸腔2例，肺2例，腹膜后3例，左大腿肌间1例，髂骨1例，肩背部皮下1例，腋窝1例，鼻腔1例，膀胱1例。临床主要表现为局部肿块及其引起的压迫症状。主要病理学改变：肿唐直径2-16cm，平均5．6cm，大部份边界清楚。镜下：瘤组织由梭形、短梭形、圆形细胞构成，细胞稀疏区和密集区交替分布，薄壁血管较多，细胞间富有粗细不等的胶原纤维。免疫组化结果：Vimentin阳性率100％（13／13），CD99阳性率92.3％（12／13），Bcl-2阳性率92．3％（12／13），CD34阳性率69.2％（9／13），S10014．3％（1／7），EMA阳性率28．6％（2／7），Ki67阳性率2-40％。结论SFT大部份为良性，诊断主要靠形态学及免疫组织化学，可见于全身多个部位，根治性切除是主要治疗手段。     

骨原发性孤立性纤维性肿瘤2例临床病理分析

目的探讨骨原发性孤立性纤维性肿瘤(solitary fibrous tumor, SFT)的临床病理学特点、诊断及鉴别诊断。方法回顾性分析2例骨原发性SFT的临床病理学特征、免疫表型、诊断及鉴别诊断等,并复习相关文献。结果 2例影像学均表现为骨质破坏;镜下可见瘤细胞呈卵圆形、短梭形,瘤细胞间可见玻璃样变的胶原纤维分割,局部区域可见丰富的形状不规则血管。例1细胞有异型,核分裂象4个/10 HPF;例2肿瘤细胞形态温和,核分裂象罕见。免疫表型:肿瘤细胞STAT6、CD34、CD99和vimentin均阳性,CKpan、S-100、desmin、SMA、EMA、ERG和STAB2均阴性。结论骨原发SFT非常罕见,病理诊断主要依靠影像学、病理学形态和免疫组化标记,需与骨的其他原发肿瘤及转移性肿瘤鉴别。     

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 (SFT)的临床病理学特征、组织来源及鉴别诊断。方法　对 3例SFT患者进行临床病理分析 ,并用免疫组化两步法检测CD34、Des、actin(HHF35 )、CD6 8、S 10 0蛋白的表达。结果　肿瘤光镜下表现为大量梭形类似成纤维细胞的细胞呈束状、编织状、席纹状排列 ,有丰富的胶原纤维和多量瘢痕形成。免疫表型 :CD34( )、actin(+)、CD6 8(+)、S 10 0蛋白 (± )、Des(- )。随访 3～ 12个月 ,均未复发。结论　本组孤立性纤维性肿瘤均为良性 ,应注意与神经鞘瘤或神经纤维瘤等鉴别     

孤立性纤维性肿瘤

一、材料与方法1.材料 :10例孤立性纤维性肿瘤 (ＳＦＴ)病例系我院1990～ 2 0 0 0年间手术标本 ,男性 5例 ,女性 5例 ;年龄 37～ 74岁 ,平均年龄 5 5 .5岁。病程数天至数十年不等。肿瘤位于肺内 4例 ,眼眶 2例 ,下胸腔、纵隔、外耳道及肺叶间裂各 1例。首发症状为肿块所在部位引起的局部表现 ,如眼球突出、面瘫、胸闷、气急、咳嗽、下肢浮肿等 ,有者则是扪及肿块或体检发现肿块。2 .方法 :所有标本均经常规固定 ,石蜡包埋 ,4μｍ连续切片 ,除常规ＨＥ染色外均同时采用ＳＰ法免疫组织化学方法染色标记。使用抗体ＣＤ34、波形蛋白、ＨＨＦ35、…     

孤立性纤维性肿瘤

孤立性纤维性肿瘤 (ｓｏｌｉｔａｒｙｆｉｂｒｏｕｓｔｕｍｏｒ ,ＳＦＴ)是一种罕见肿瘤 ,又因组织学的复杂性、多样性 ,较易误诊。笔者就我院 2例ＳＦＴ结合文献对其组织学、免疫组化和电镜诊断及鉴别诊断等进行讨论。1　临床资料例 1,患者女性 ,6 1岁。体检发现左下肺阴影 2天 ,无明显不适。ＣＴ示左下肺后基底段球形阴影 ,边缘较规整 ;术前诊断错构瘤或隔离肺。术中见肿瘤位于左下肺基底段紧靠肺膜 ,与周围组织分界清 ,极易剥离 ,遂行单纯切除。术后已5年 ,无复发或转移。例 2 ,患者男性 ,45岁。体检发现右腹股沟肿物 2周。查体 :右腹股…     

Solitary fibrous tumor with malignant potential arising in sublingual gland

A rare case is described of a solitary fibrous tumor (SFT) with malignant potential arising in the sublingual gland. A 59-year-old man presented with a 4-month history of a slowly enlarging painless mass in the center of the floor of the mouth. The tumor was a well-demarcated, firm mass with a multicystic lesion. The tumor exhibited highly cellular areas of spindle cells with patternless architecture alternating with hypocellular areas. The tumor cells were positive for CD34 and bcl-2 as well as vimentin, and negative for epithelial, myogenic, neurogenic and histiocytic markers. The tumor cells formed multiple satellite nodules around dilated ducts in the multicystic lesion, indicating infiltrative growth. In addition, areas exhibiting higher cellularity with increased mitoses were noticed in the satellite nodules, although cellular atypia was not obvious. These findings led to a final diagnosis of SFT with malignant potential. There has been no recurrence or metastasis for 27 months after the surgery. Solitary fibrous tumor of the salivary gland must be differentiated from various spindle cell neoplasms including myogenic, peripheral nerve sheath, fibroblastic and fibro-histiocytic spindle cell neoplasms, hemangiopericytoma and myoepithelioma. In addition to characteristic morphological features, an immunohistochemical positivity for CD34 and bcl-2 may aid in the diagnosis of SFT.     

Disseminated malignant solitary fibrous tumor of the pleura

Solitary fibrous tumor (SFT) of the pleura typically forms a localized pleura-based mass, and most are benign. A rare case of disseminated malignant SFT of the pleura is reported. The patient was a 71-year-old man who presented with complaints of shortness of breath to his primary care physician. A diagnosis of malignant mesothelioma was suspected, based on clinical, radiological and needle biopsy findings. He was referred to our institution for surgery. An extrapleural pneumonectomy, encompassing all pleural masses, was performed. Gross examination of the resected specimen was remarkable for numerous masses, ranging in size from 0.2 to 13.5 cm, covering the majority of the visceral pleura. Histologically, the tumor was composed of short spindle cells admixed with variable proportions of collagenous stroma. There were great intra- and intertumoral heterogeneity in tumor growth pattern, cellularity, pleomorphism and mitoses. Histologically malignant areas were present in all of the masses examined. The neoplastic cells were diffusely and intensely positive for bcl-2. Most tumor cells were also strongly stained for CD34 and CD99. Staining for cytokeratin was negative. The tumor also revealed p53 over-expression. Thus, the histological and immunohistochemical features of the tumor were consistent with a disseminated malignant SFT. This report shows that SFT rarely presents with disseminated pleural involvement, and a panel with CD34, bcl-2 and cytokeratin are valuable for differentiating SFT from malignant mesothelioma and other malignant spindle cell neoplasms of the pleura.     

Pelvic cavity malignant solitary fibrous tumor with dedifferentiation and multifocal cytokeratin expression

Pelvic cavity small-sized malignant solitary fibrous tumors are rare. Surgeons easily misjudge these tumors as benign lesions, and they are usually resected by laparoscopic surgery. When accompanied by dedifferentiation, malignant solitary fibrous tumors are difficult to diagnose by pathologists. Here, we describe a challenging case. A 47-year-old man was accidentally found to have a pelvic mass after three months. Surgeons assumed a diagnosis of a benign tumor according to the Computed Tomography and Ultrasonography reports. The patient underwent laparoscopic surgery to resect the tumor. After the operation, the patient was diagnosed as having a malignant solitary fibrous tumor with dedifferentiation. The patient had a recurrence one year later. This pelvic cavity malignant solitary fibrous tumor was a rare case because of its dedifferentiation and cytokeratin expression. The expression of cytokeratin and the absence of vimentin and CD34 were pitfalls to diagnosis. In this case, there was still a high amount of malignancy despite the small size of the tumor. Clinical image and pathologic multidisciplinary analysis and core needle biopsies before surgery had a great effect on diagnosis and therapy of this disease. The case is a cautionary tale not only for pathologists but also for surgeons.     

Clinicopathological features of solitary fibrous tumor of the meninges: An immunohistochemical reappraisal of cases previously diagnosed to be fibrous meningioma or hemangiopericytoma

Cases of solitary fibrous tumor (SFT) of the meninges are increasingly being reported. However, the real incidence of SFT among meningeal tumors has yet to be determined. We therefore clinicopathologically re-examined 64 meningeal tumors originally diagnosed to be either fibrous meningioma (FM group, n = 46) or hemangiopericytoma (HPC group, n = 18) while paying special attention to SFT. We thus reclassified one case from the FM group (2%) and one case from the HPC group (6%) to be SFT, both of which showed diffuse CD34-immunoreactivity and dense intercellular reticulin fibers but neither epithelial membrane antigen nor S-100 protein expression. The MIB-1 staining index of these cases were 6. 2% and 3.9%, respectively. The former recurred 15 years after the initial surgery and the patient underwent a second removal of the tumor. The patient has been alive with no evidence of recurrence for 7 years after the second surgery. The latter patient has been alive with no evidence of recurrence for 3 years postoperatively. The results confirmed that the incidence of SFT among meningeal tumors is relatively low, however, because of its clinically indolent nature, a careful histochemical examination is necessary to differentiate SFT from other neoplasms with a more aggressive nature. Our findings emphasize the need to clinically recognize this lesion as a distinct entity.     

Malignant solitary fibrous tumor of the thyroid gland: report of a case and review of the literature

Solitary fibrous tumors of the thyroid gland (T-SFT) are rarely described, with only 21 cases being reported in the English literatures, all showing benign clinical characteristics. We herein present a 76-year-old woman presenting with a 3-month history of rapidly enlarging neck masses and the CT showed masses with partial calcification in the right thyroid lobe. We performed right hemithyroidectomy and isthmectomy with negative margin under general anesthesia. Histologically, the masses consisted of pleomorphic spindle cells with high mitoses and collagen bands. Immunohistochemically, the tumor cells showed positive reactions for CD34, vimentin and bcl-2, then a diagnosis of malignant solitary fibrous tumor of the right thyroid was made. Six months postoperatively, the CT showed the recurrence of the thyroid tumor and the presence of many nodules of varying sizes throughout bilateral pulmonary lobes. To our knowledge, this is the first case of malignant solitary fibrous tumor of the thyroid gland (T-SFT) with local recurrence and pulmonary metastasis and T-SFT must be considered in the differential diagnosis of spindle cells lesions in the thyroid gland. Correct diagnosis of the malignant T-SFT plays an important role in choosing appropriate therapeutic strategies and long-term follow-up is also extremely essential for these patients.     

Primary myxoid solitary fibrous tumor involving the seminal vesicle

Although focal myxoid change is a well-recognized feature of solitary fibrous tumor (SFT), predominantly myxoid SFT are exceedingly rare. Reported herein is the case of a 65-year-old man with SFT containing abundant myxoid matrix, arising from the capsule of the right seminal vesicle. To the authors' knowledge this is the first myxoid SFT involving the male genito-urinary system. Recognition of the myxoid variant of SFT is crucial, given possible confusion with a variety of myxoid spindle cell neoplasms with different biological potential in the pelvic cavity.     

Hypoglycemia in a patient with a huge malignant solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura (SFTP) is rare. A 73-year-old man, without past history of diabetes mellitus, was admitted to hospital with profound hypoglycemia, loss of consciousness and respiratory failure. CT indicated a huge heterogenous mass lesion (12 x 12 x 17 cm) at the right thoracic cavity with total atelectasis of the right lower lung. He underwent a standard thoracotomy with complete excision of the tumor. Pathology indicated malignant SFTP with insulin-like growth factor-binding protein-2 production. There were no further incidences of hypoglycemia or respiratory failure after excision of the tumor. Hypoglycemia resulting from a solitary fibrous tumor is uncommon. Standard thoracotomy and complete resection of this giant tumor provided a good result for the present patient.     

D2-40-positive solitary fibrous tumors of the pleura: diagnostic pitfall of biopsy specimen

Solitary fibrous tumor of the pleura (SFTP) is an uncommon spindle-cell neoplasm and is sometimes confused with other spindle-cell tumors, such as sarcomatoid mesotheliomas. D2-40 is a new monoclonal antibody recognizing podoplanin and several studies have confirmed the reliability of D2-40 in the diagnosis of pleural mesothelioma. The authors encountered a case of SFTP that was immunoreactive to D2-40. A 56-year-old woman was admitted to hospital because of an abnormal shadow on a chest X-ray. Percutaneous needle biopsy indicated a spindle-cell tumor showing positive immunoreactivity for CD34, supporting the diagnosis of SFTP. However, the tumor was also immunoreactive to D2-40 and thus malignant mesothelioma could not be ruled out. The tumor was surgically resected to make a definitive pathological diagnosis. The tumor had a patternless architecture and immunohistochemistry was positive for CD34 and D2-40 but was negative for calretinin and cytokeratin 5/6. Therefore a pathological diagnosis of SFTP was made. An additional six tumors diagnosed as SFTP were also tested. Focal immunoreactivity to D2-40 was positive in three out of seven cases, including the first case. Care is required in diagnosing biopsy specimens of D2-40-positive pleural spindle-cell tumors, especially in making the differential diagnosis between SFTP and malignant mesothelioma.