肺恶性孤立性纤维性肿瘤1例并文献复习

目的：探讨肺恶性孤立性纤维性肿瘤的临床病理特点、鉴别诊断、治疗及预后。方法对1例肺恶性孤立性纤维性肿瘤进行临床病理学分析及免疫组化SP法染色,并结合相关文献复习。结果患者女性,71岁。左肺有一肿物,7．5 cm×7 cm×5 cm大小,呈灰白色,分叶状,质硬,包膜完整。镜下显示在瘤细胞分化较好的区域,细胞多为形态温和的梭形细胞,呈束状或编织状排列,可见纤维瘢痕组织及瘤细胞围绕分支状血管呈血管外皮瘤样结构;在瘤细胞分化较差区域,瘤细胞丰富,异型性明显,可见少数瘤巨细胞,核分裂象多见(>20个/10 HPF),并见多个小坏死灶,局部瘤细胞突破包膜,向周围肺组织浸润性生长。免疫表型：CD34、CD99和vimentin 均呈弥漫强阳性, BCL-2呈弱阳性, Ki-67增殖指数>20%。术后随访16个月无复发,患者一般情况良好。结论肺恶性孤立性纤维性肿瘤极为罕见,诊断和鉴别诊断主要依靠病理形态学和免疫表型,治疗以手术完整切除肿瘤为主,术后应对患者进行长期随访。     

钙化性纤维性肿瘤5例临床病理观察并文献复习

目的探讨钙化性纤维性肿瘤(calcifying fibrous tumor, CFT)的临床特点、组织学特征、诊断、鉴别诊断。方法分析5例CFT的临床表现、影像学特点、病理学形态、免疫表型,并复习相关文献。结果镜下肿瘤边界清楚,由大量致密的、束状排列的胶原纤维组成,其间夹杂少量形态温和的梭形细胞,并见散在的淋巴细胞、浆细胞浸润,可形成淋巴小结或淋巴滤泡。免疫表型:CD31、CD34呈血管内皮阳性,STAT6呈胞质弥漫阳性,浆细胞IgG呈阳性,但IgG4呈阴性。结论 CFT是一种具有典型形态学特点的罕见良性间叶性肿瘤,好发部位是胃,术前诊断困难,需与多种疾病进行鉴别。     

肾恶性孤立性纤维性肿瘤1例报道及文献复习

目的 探讨肾脏恶性孤立性纤维性肿瘤的临床病理特征、鉴别诊断及治疗和预后.方法 报道1例肾恶性孤立性纤维性肿瘤的临床病理资料并复习文献.结果 患者男性,56岁.临床主要表现为乏力、出汗、心慌、气短1年,以低血糖原因待查入院.CT示左肾上方及左肾前下方见3个类圆形软组织密度影,与肾分界不清,大小分别为9 cm×8 cm×7 cm、7 cm×7cm×7 cm和4 cm×4 cm ×4 cm.增强后边缘轻度强化,中心不均匀强化.临床、CT检查考虑为左.肾癌.行左肾癌根治术.眼观:左肾上、下极分别见一肿块,切面灰白色,部分区见有坏死,与肾界限不清.镜检:肿瘤由短梭形或卵圆形细胞组成,排列成束状、旋涡状或不规则状.部分区域血管丰富,细胞密集呈血管外皮瘤样结构;部分区域细胞稀疏伴纤维化、黏液变性.肿瘤超过60%的区域内(尤其是肾下极肿块)瘤细胞丰富密集,有异型性,核分裂象多见(>4个/10 HPF),边缘呈浸润性生长,部分区可见出血坏死.免疫表型:肿瘤细胞vimentin、CD34弥漫阳性,CD99、bcl-2阳性,Ki-67增殖指数>20%.病理诊断:左肾恶性孤立性纤维性肿瘤(低度恶性).术后随访24个月情况良好.结论 肾恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并应与肾的其他梭形细胞肿瘤进行鉴别.治疗以手术完全切除为主,并注意长期随访.     

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。     

肾脏原发性孤立性纤维性肿瘤3例报道并文献复习

目的 探讨肾孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理学特征、诊断、鉴别诊断及预后.方法 对3例罕见的肾SFT进行临床、病理组织学形态观察及免疫组化染色,并复习相关文献.结果 原发于肾脏的SFT临床与影像学检查结果缺乏特征性表现,镜下细胞形态基本为长梭形,部分为卵圆形或上皮样细胞.其主要特点为含丰富的胶原纤维和形成多量瘢痕.免疫组化染色示瘤细胞CD34和CD99均(+),BCL-2(±).结论 发生于肾脏的SFT较为罕见,因缺乏特异性的术前诊断依据而极易误诊,确诊需依赖病理学检查,免疫组化染色有助于诊断及鉴别诊断.     

孤立性纤维性肿瘤临床病理学观察

目的探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床、病理组织学、免疫组化特征,提高对该病的认识。方法对7例SFT病例进行大体观察、病理组织学及免疫组化En V ision二步法标记,观察V im、CD34、CD99、bc l-2 Des、SMA、HMBE-1、CK、S-100蛋白在SFT组织中的表达。结果7例SFT分别发生于胸腔、腹腔、纵隔、眼眶及胸壁软组织,临床表现为肿块压迫所致的症状,肿块1.5 cm×1.5 cm×1 cm~20 cm×20 cm×18 cm,光镜下肿瘤细胞由长梭形细胞构成束状、编织状排列,间质内有多少不一的胶原纤维,无特殊的组织构型及形态的多样性为其组织学特点。免疫表型:CD34、V im、CD99均为弥漫强阳性,bc l-2 5例阳性,Des、SMA、HMBE-1、CK、S-100蛋白阴性。结论SFT是一组形态多样、无特殊组织构型及有多少不一胶原纤维的梭形细胞肿瘤,其特殊的免疫表型,有助于对该病的诊断,但SFT的确诊必须结合组织形态和免疫组化共同完成。SFT的生物学行为取决于其组织学形态和肿块的大小及生长方式,完全切除肿块者预后较好。     

颅内去分化孤立性纤维性肿瘤1例并文献复习

目的探讨颅内去分化孤立性纤维性肿瘤(solitary fibrous tumor, SFT)的临床病理特征、免疫表型及鉴别诊断。方法回顾性分析1例颅内去分化SFT的临床病理特征、影像学表现及免疫表型,并复习相关文献。结果患者男性,54岁,右侧颞叶SFT术后1年,MRI提示原颅内占位术后右侧颞叶占位,考虑复发。镜检:瘤细胞短梭形,异型明显,呈血管外皮瘤样排列,可见出血、坏死,部分区域胞质红染,核偏位,核分裂象10～15个/10 HPF;免疫表型:瘤细胞弥漫表达BCL-2、STAT6,局灶表达CK(AE1/AE3)、EMA、CD34、CD99、desmin、MyoD1、Myogenin、Myoglobin、SMA,Ki-67增殖指数约50%。结论颅内去分化SFT与发生于脑膜部的梭形细胞肿瘤的临床特点及影像学表现相似,需结合病理检查及免疫表型进行鉴别。单一手术难以完整切除,易复发恶变,预后差。     

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。     

甲状腺孤立性纤维性肿瘤1例报道并文献复习

目的探讨甲状腺孤立性纤维性肿瘤的临床病理学特点。方法对1例甲状腺孤立性纤维性肿瘤进行组织学和免疫组织化学观察,并复习相关文献。结果患者女性,于甲状腺右叶触及一肿块。光镜下见肿块境界清楚,梭形及卵圆形的肿瘤细胞排列成条束状、短席纹状,其间含有胶原纤维束,肿瘤细胞间散在少量大小不一的滤泡。肿瘤细胞CD34和bcl-2均呈阳性。结论孤立性纤维性肿瘤发生于甲状腺者较为罕见,并且临床中其生物学行为仍然未能确定,有必要长期随访。     

恶性孤立性纤维性肿瘤2例临床病理分析

目的:探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)的临床病理特征、诊断、鉴别诊断、分子遗传学、治疗及预后.方法:对2例MSFT进行光镜、免疫组织化学研究,并复习相关文献.结果:男性2例,年龄分别为66岁及48岁.发生部位:骶前1例,临床表现为进行性二便障碍3年;后上纵隔1例,临床表现为活动后心慌半个月.肿瘤大小分别为10cm×9 cm×6 cm及12 cm×10 cm×9 cm.镜检:细胞稀少区和细胞丰富区交替分布,两者之间有粗的玻璃样变胶原纤维分隔.密集区肿瘤细胞丰富,呈束状、漩涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形、短梭形,核染色质粗,异型性明显,有肿瘤性坏死、较多量核分裂( ＞4/10 HPF),局部出血.免疫表型:肿瘤细胞CD34,Bcl-2,CD99及Vimentin弥漫阳性;P53部分阳性,上皮细胞膜抗原局灶阳性;ki-67增殖指数20％ ～ 30％;S100,Desmin,CK及胶质纤维酸性蛋白阴性.结论:MSFT是一种少见的间叶肿瘤,确诊主要依靠形态学及免疫组织化学,需与血管外皮细胞瘤、恶性纤维组织细胞瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤、恶性外周神经鞘膜瘤和脑膜瘤等相鉴别.MSFT的预后不良,易复发和转移,根治性切除是主要治疗手段,术后应长期随访.     

Fat-forming solitary fibrous tumor of the kidney: a case report and literature review

Fat-forming solitary fibrous tumor (SFT) is a rare soft tissue tumor. Herein, we reported a 30-year-old woman was found to have a solid mass measuring 60×45 mm in the right kidney on an abdominal computed tomography scan. The tumor was well-circumscribed and composed of cellular nodules with the classic SFT admixed with clusters and lobules of mature adipocytes. Immunohistochemistry staining showed that the tumor cells were diffusely and strongly positive for CD34 and Bcl-2, focally and weakly positive for CD99 and EMA. Mature adipocytes were positive for S-100 protein. Ki-67 expression was found in approximately 2% of tumor cells. However, tumor cells were negative for cytokeratin, S-100 protein, HMB-45, Melan-A, SMA, and CD117. We made the pathological diagnosis of fat-forming SFT of the right kidney. The differential diagnosis includes angiomyolipoma, liposarcoma, spindle cell lipoma, sarcomatoid renal cell carcinoma, synovial sarcoma, and gastrointestinal stromal tumor. The patient was alive and well without evidence of recurrence or metastasis at 19 months after tumor resection.     

Solitary fibrous tumor of the prostate: a report of two cases

We here report two cases of solitary fibrous tumor (SFT) arising in the prostate. Two men, 66 and 69 years old, with urinary tract symptoms were diagnosed with SFT on transrectal needle biopsy and transurethral resection of the prostate, respectively. The tumors were removed by a low anterior resection including tumor, prostate and rectum en bloc and cystoprostatectomy, respectively. Both tumors were well-circumscribed but also showed some infiltration of the prostate glands. They were composed of storiform bundles of bland spindle cells that stained strongly for CD34 and vimentin but negative for muscle markers. Although rare, SFT should be considered as differential diagnosis of spindle cell lesions on prostate biopsies.     

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.     

Solitary fibrous tumor: a study of cytologic features of six cases diagnosed by fine-needle aspiration

Solitary fibrous tumor (SFT) is a spindle-cell neoplasm most often presenting as a pleural-based tumor but increasingly recognized in other locations. Few reports have described the cytologic features of SFTs. Six cases of SFT diagnosed by fine-needle aspiration (3 pleura, 2 retroperitoneum, and 1 orbit) were identified in the Mayo Clinic files. The smears (Papanicolaou-stained) and corresponding histologic specimens were reviewed. Immunohistochemical staining for CD34 was performed in all cases. The cytologic findings were similar in all cases. The tumor cells were oval to polygonal, with cellularity ranging from scant to moderate. The background contained irregular ropy fragments of collagen and a few inflammatory cells. Most cells were dispersed singly, but all cases contained irregular, loose aggregates of cells enmeshed in a collagenous matrix. The nuclei were uniformly bland, with evenly distributed, finely granular chromatin. All cases were immunoreactive for CD34. SFT has distinctive cytologic features that allow diagnosis in cytologic specimens with the help of appropriate immunocytochemical stains on accompanying tissue biopsy specimens. Distinctive cytologic findings predictive of clinical behavior were not identified.     

Solitary fibrous tumor of the lung: a case report with a study of the aspiration biopsy, histopathology, immunohistochemistry, and autopsy findings

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm of ubiquitous location. In addition to its classic presentation as a pleural-based mass, it can be encountered in a variety of other sites. A pleural-based lung lesion can be easily accessed by radiologic guidance for cytologic study. Several reports have described the cytologic findings of SFT at various locations, including the lung. However, diagnostic difficulties can occur due to unusual clinical, radiologic, atypical cytomorphologic, and histologic features. We describe a case of intrapulmonary SFT in which a false-positive malignant diagnosis was rendered on fine-needle aspiration biopsy and concurrent surgical core biopsy prior to radiofrequency ablation. The patient died of procedural complications, and an autopsy was performed. Retrospective study of the case, especially correlation of cytologic, histologic, autopsy findings, and immunohistochemistry results were helpful in correctly diagnosing the case as SFT. We are reporting this case with emphasis on avoiding diagnostic pitfalls by being familiar with the accepted cytohistologic features and appropriate immunohistochemical results.     

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.