甲状腺孤立性纤维性肿瘤的超声诊断学特征并文献复习

目的探讨甲状腺孤立性纤维性肿瘤的超声诊断学特征。 方法回顾性分析2020年7月29日就诊于济宁医学院附属医院甲状腺外科的1例甲状腺孤立性纤维性肿瘤患者的临床资料和影像学、病理学检查结果，并复习相关文献。 结果患者颈前偏左局限性隆起，甲状腺左叶可触及一明显肿物。超声可见甲状腺左叶一不均质回声团块，大小约5.8 cm×4.0 cm×2.7 cm，界清，形态规则，后方回声衰减，内可及较丰富的分支状血流信号。甲状腺CT平扫及增强扫描可见甲状腺左叶内略低密度影，增强后呈不均匀性延迟强化，呈"快进慢出"的特点。术后病理诊断为孤立性纤维性肿瘤。 结论超声对甲状腺孤立性纤维性肿瘤的大小、形态、内部回声、血流情况具有一定特征性，结合其他检查可提高该病的临床诊断率。     

胸腹部孤立性纤维性肿瘤的影像学表现

目的 探讨孤立性纤维性肿瘤的CT及MRI表现.方法 回顾分析10例经手术病理证实的孤立性纤维性肿瘤患者临床及影像资料.所有患者均行CT平扫及双期增强扫描,其中7例行后处理成像.5例同时行MRI平扫及增强.结果 肿瘤位于胸膜5例,肺内2例,腹膜后2例,腹膜1例,肿瘤直径5.5～27 cm.肿瘤边界清晰8例,2例与周边组织分界欠清,肿瘤密度均匀者6例,坏死囊变者4例.肿瘤增强后实质成分呈均质轻到中度强化9例,实质期肿瘤持续强化,明显强化者1例,伴有钙化1例.结论 孤立性纤维性肿瘤的影像学表现有一定的特征性,CT及MRI检查对诊断与鉴别诊断具有重要价值.     

6例恶性孤立性纤维性肿瘤的临床病理特征

回顾性分析6例恶性孤立性纤维性肿瘤(malignant solitar y f ibrous tumor,MSFT)的临床病理特征,其中男5例,女1例;年龄25~69(中位数42.17)岁,分别发生在前额、膝部、大腿、锁骨上及胸腹壁,肿瘤直径1.8~24.5 cm。组织学特点:瘤细胞丰富密集,其间可见绳索样、玻璃样变胶原纤维分隔,瘤细胞呈短梭形、梭形,异型性明显,核分裂易见(≥4/10 HPF),瘤细胞呈束状、漩涡状排列,部分区域可见血管外皮瘤样结构,局灶可见出血或肿瘤性坏死。肿瘤部分区域可见浸润性边缘。免疫组织化学特点:6例均弥漫表达波形蛋白(Vimentin),STAT6,CD34,Bcl-2和CD99,1例的孕激素受体(progesterone receptor,PR)局灶阳性,6例的Ki-67为2%~60%(+)。S100,AE1/AE3,EMA,CD117,SOX10,Desmin,平滑肌肌动蛋白(smooth muscle actin,SMA),雌激素受体(estrogen receptor,ER)均阴性。MSFT的确诊主要依赖组织学的形态特征和免疫组织化学染色。形态学非常温和良善的SFT也可能有侵袭性生物学行为,临床上应尽早完整切除肿物并对孤立性纤维性肿瘤(solitar y fibrous tumor,SFT)长期随访,注意恶变的可能性。手术切除的完整性以及必要的放/化疗、靶向药物治疗等对预后非常重要。     

Malignant solitary fibrous tumor of the greater omentum: A case report and review of literature

BACKGROUNDMalignant solitary fibrous tumors (SFTs) account for 15%-20% of all SFTs, and malignant SFTs arising from the greater omentum are extremely rare. Most malignant SFTs of the greater omentum are diagnosed via pathological examinations after surgery. In this study, we report a case of malignant omental SFT and review the published literature on this rare malignancy.CASE SUMMARYA 64-year-old female presented with an abdominal mass, and underwent exploratory surgery, during which a huge tumor originating from the greater omentum and intraperitoneal implants were identified and resected. The results of the pathological examination, immunohistochemistry staining, and gene sequencing led to the diagnosis of malignant SFT of the greater omentum. The patient died one and a half years later due to tumor recurrence and metastasis.CONCLUSIONThis is the first report of the application of gene sequencing in the diagnosis of malignant SFTs of the greater omentum.     

血管外皮细胞瘤与孤立性纤维瘤的诊断和鉴别诊断

目的血管外皮细胞瘤和孤立性纤维瘤同属纤维母细胞/肌纤维母细胞来源肿瘤的中间性肿瘤,病理和组织学方面重叠表现多,术前误诊率高。本文分析各自影像学特征,探讨其影像学诊断和鉴别诊断的价值。方法回顾性分析经手术病理证实的9例血管外皮细胞瘤和8例孤立性纤维瘤病例,术前经螺旋CT或MR检查,仔细复习CT和MRI扫描结果并和手术病理作回顾性对照分析。结果9例血管外皮细胞瘤病灶中,位于颅内3例,四肢4例,腹膜后1例,腹股沟1例;肿瘤直径4.8-13.5 cm,平均6.7 cm;瘤内见蚯蚓和结节状异常信号6例,不同程度坏死9例;所有肿瘤实质成分T1WI为等或略低信号,T2WI为略高信号;其中,6例行动态增强,动脉期显著强化5例,中等程度强化1例,所有病例门脉期强化程度与血管相仿。8例孤立性纤维瘤中,位于胸部4例,四肢2例,腹腔1例,盆腔1例;肿瘤直径4.0-22.5 cm,平均9.3 cm;密度或信号较均匀5例,不同程度坏死3例,伴点状钙化1例。CT平扫密度与肌肉密度相仿,内可见两种不同密度的实质成分;CT动脉期呈地图样或线样葡行强化,门脉期持续强化,强化趋于均匀;MRSE T1WI呈等信号,T2WI为略高信号,T2 WI脂肪抑制为高信号;MR动脉期明显强化,门脉期持续强化。结论血管外皮细胞瘤和孤立性纤维瘤具有较明显的影像学特征,影像学可以诊断和鉴别多数病例。     

骨原发性恶性纤维组织细胞瘤(附8例报告)

目的讨论骨原发性恶性纤维组织细胞瘤(MFH)的病理、X线表现,提高对该病的认识。方法分析8例骨原发性恶性纤维组织细胞瘤的病理、X线表现。结果病理上由多种细胞成分构成以成纤维细胞和组织细胞为主。X线表现为单发溶骨性破坏为主要表现,肿瘤周围的骨皮质有不同程度膨胀,可形成软组织肿块,骨膜反应或病理性骨折等。结论诊断主要依据于病理检查和X线表现。     

巨大壁层胸膜孤立性纤维性肿瘤一例

孤立性纤维性肿瘤（SFT）是一种来源于间叶组织的梭形细胞性肿瘤。该病早期临床症状隐匿,常因肿瘤增大压迫周围组织而被发现,CT和（或）MRI是诊断SFT的常用手段,确诊需行病理活组织检查及免疫组织化学检查。该文报告l例巨大SFT患者,其以大量胸腔积液为首发表现且伴胸闷、呼吸困难等症状。行胸腔引流术后完善胸部CT检查,结果提示为左侧胸腔顶部肿物,由左锁骨下动脉供血,血供较丰富,考虑为壁层胸膜恶性肿瘤。其后行肿瘤血管栓塞术,待肿瘤缩小后行手术将其完全切除,术后病理活组织检查结果为SFT,患者术后恢复良好。由此可见,手术是治疗SFT的首选治疗方法,由于SFT血供常较丰富,故肿瘤较大时可于术前行肿瘤血管栓塞术,待肿瘤缩小后再行手术切除,以降低手术难度及风险,提高手术成功率。     

Late-onset non-islet cell tumor hypoglycemia: A case report

BACKGROUNDHypoglycemia due to non-insulin-producing tumors is referred to as non-islet cell tumor hypoglycemia (NICTH). As NICTH is a rare lesion, the natural course of NICTH is not well understood. We report a case of NICTH that was observed 30 years before the onset of hypoglycemia. CASE SUMMARYA 50-year-old man was diagnosed with an abnormal right chest shadow during a routine X-ray examination, but no further examination was undertaken because the lesion appeared benign. Thirty years after the tumor discovery, the patient was admitted to the hospital with symptoms of severe hypoglycemia, which was diagnosed as NICTH based on a complete examination. The tumor was resected and found to be a solitary fibrous mass (15.6 cm × 13.7 cm × 10.4 cm); thereafter, the patient’s blood glucose levels normalized and he completely recovered. CONCLUSIONNICTH can have an acute onset, even if the tumor has been present and asymptomatic over a long time period.     

孤立性纤维性肿瘤的超声表现:附10例报告

目的:探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的超声表现并分析超声表现与其临床病理学特征的关系。方法:回顾分析经手术病理证实的10例SFT的超声表现,归纳其声像图特征,包括肿瘤位置、大小、形态、内部回声及血供特点等。结果:SFT的超声表现多为边界清楚、边缘光滑的低回声或中等回声实质性肿块,腹腔是其最常见的原发部位。10例肿瘤均血供丰富。结论:SFT的超声表现为边界清楚、血供丰富的中低回声实质性肿块,但仍需大规模临床试验加以验证。     

Successful laparoscopic extirpation of a large omental lipoblastoma in a child

Omental lipoblastoma is extremely rare among benign tumors. We herein report the case of a child who underwent laparoscopic extirpation of a large omental lipoblastoma. A 4‐year‐old girl was diagnosed with an intra‐abdominal solid tumor. Abdominal imaging revealed a fat density mass that was well encapsulated and measured 18 × 15 × 7.5 cm in size. Considering the MRI findings and movability of the tumor, we strongly suspected that the lesion was an omental lipoblastoma. We initially decided to perform laparoscopic exploration and, if possible, extirpation of the solid tumor sequentially. A total of five trocars were used, and the tumor was found to originate from the omentum. We successfully performed complete resection of the tumor laparoscopically. A histological examination revealed lipoblastoma. For large abdominal tumors in children, the laparoscopic approach is recommended as the first procedure when the tumor is preoperatively considered to be benign and resectable.     

孤立性纤维瘤的多层螺旋CT表现

目的探讨孤立性纤维瘤（SFr）的多层螺旋CT（MSCT）表现,以提高对其的诊断水平。方法回顾性分析21例经手术病理证实的SFT患者的MSCT表现,所有病例均接受MSCT平扫及增强扫描检查。结果SFT的MSCT表现为孤立、边界清楚的单发肿块。瘤体较小时,CT平扫常表现为均匀的低密度肿块,增强扫描多表现为均匀显著性强化;瘤体较大时,CT平扫常因内部囊变、坏死呈不均匀低密度,增强扫描多表现为显著不均匀强化,呈地图样或分层样强化。结论MSCT可清晰显示SFT病灶的形态、大小及其与周围组织的关系,对SFT的诊断和鉴别诊断具有一定的帮助。     

肾孤立性纤维瘤临床病理观察

目的探讨肾孤立性纤维瘤的临床病理特征、诊断和鉴别诊断，提高对该肿瘤的诊断水平。方法对1例肾孤立性纤维瘤进行光镜、免疫组化观察，并结合文献讨论。结果患者女性，47岁。临床、放射学诊断为肾盂癌。肿瘤边界清楚，切面灰白色。镜下梭形细胞呈束状、旋涡状或不规则状排列，部分与胶原纤维混杂，部分呈血管外皮瘤样结构。细胞无明显异型，核分裂象偶见。免疫组化示肿瘤细胞vimentin、CD34和皿199（＋），bel-2部分（＋），desmin、SMA、MAS、HMB45、carponin、S-100蛋白、CD117、CD31、FVⅢ、CK（AE1/AE3）、ENA、Ki-67和p53均（-）。结论肾孤立性纤维瘤是一种非常罕见的肿瘤，诊断主要依靠病理形态学及免疫组化，并应与肾的其他梭形细胞肿瘤鉴别。     

Dumbbell-shaped solitary fibrous tumor in the parapharyngeal space: A case report

BACKGROUNDSolitary fibrous tumors (SFTs) occurring in the parapharyngeal space are rare, and their final diagnosis depends on pathological and immunohistochemical analyses. Once the tumor is diagnosed, complete resection and regular postoperative follow-up are required.CASE SUMMARYA 40-year-old male patient with a right parotid gland mass discovered 8 years ago was admitted to hospital. The mass showed no tenderness or local skin redness. Imaging was carried out as the patient had stable vital signs and showed that the mass was a dumbbell-shaped tumor comprising a superficial tumor approximately 5 cm long and 3 cm wide in size that compressed the right parotid gland and a deep tumor located in the right parapharyngeal space approximately 4.5 cm long and 2.5 cm wide in size. Both tumors were connected in the middle. Prior to surgery, the tumors were considered to be parapharyngeal schwannomas. During surgical dissection, the tumors were found to be smooth and tough, without obvious adhesion to the surrounding tissues. The tumors were revealed to be a SFT following postoperative pathological analysis.CONCLUSIONSFTs in the parapharyngeal space are rarely reported, and complete resection of such tumor is recommended. Adjuvant chemoradiotherapy is used in patients with extensive tumor invasion to lower the recurrence rate. Postoperative long-term follow-up is required.     

Doege–Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor

Doege–Potter syndrome leads to severe and sustained symptomatic hypoglycemia and is associated with the solitary fibrous tumor. It is a rare cause, and its diagnosis requires a clinical suspicion and other markers such as insulin‐like growth factor II. Here, we describe a case of a patient with intrathoracic tumor and hypoglycemia.     

Thoracoscopic resection for mediastinal thymolipoma in a child

Thymolipoma is a rare disease among benign tumors. We herein report the case of a child who underwent thoracoscopic resection of a large thymolipoma. A 3‐year‐old boy was diagnosed with an anterior mediastinal solid tumor. Thoracic imaging revealed a fat density mass that was 8.0 × 5.0 × 3.5 cm in size. Given the MRI findings of the tumor, we suspected that the lesion was a teratoma. We decided to perform thoracoscopic exploration and, if possible, resection of the solid tumor sequentially. We successfully resected the tumor thoracoscopically. A histological examination revealed thymolipoma. For large mediastinal tumors in the intrathoracic space in children, the thoracoscopic approach is recommended when the tumor is preoperatively considered to be benign and resectable.     

以低血糖为首发症状的4例胸膜孤立性纤维性肿瘤的CT表现

目的 探讨以低血糖为首发症状的胸膜孤立性纤维性肿瘤(SFTP)的CT表现。方法 回顾性分析以低血糖为首发症状并经手术病理证实的4例SFTP患者的临床及CT资料。结果 4例CT均表现为胸腔单发类圆形软组织肿物,边界清楚,与胸膜关系密切,肿瘤直径分别为2.0 cm、2.0 cm、3.1 cm、4.3 cm。2例肿瘤密度均匀、呈均匀增强;2例密度不均匀,呈"地图样"强化,1例见少量胸腔积液。结论 SFTP的CT表现具有一定特征性。患者以原因不明的低血糖症状就诊时,如发现边缘光整的胸腔孤立性软组织肿物、伴有"地图样"强化,应仔细观察肿瘤与胸膜的关系,对起源于胸膜者应考虑SFTP可能。     

A case report of solitary fibrous tumor of the pleura: ultrasound diagnostic features

Solitary fibrous tumor of the pleura is a rarely encountered clinical entity. Although the majority of these neoplasms have a benign course, the malignant form has also been reported. We describe the case of a 57-year-old male smoker with diabetes who was incidentally diagnosed on chest X-ray with a large solitary mass of the left hemithorax. The diagnostic tests included computed tomography, ultrasound, and contrast-enhanced ultrasound. Radical surgical resection was performed and histological examination confirmed a malignant solitary fibrous tumor of the pleura. The novelty of the case is the use of contrast-enhanced ultrasound in the diagnostic workup.     

原发长骨良性纤维组织细胞瘤的影像表现及鉴别

目的探讨原发长骨良性纤维组织细胞瘤(BFH)的影像表现及鉴别诊断。方法回顾性分析29例证实的原发长骨良性纤维组织细胞瘤患者的临床及影像学资料。结果本组病例病变均单发,其中股骨14例,胫骨7例,肱骨5例,腓骨2例,锁骨1例,其中29例均行X线检查,21例行CT检查,17例行MRI检查;X及CT示病变呈偏心性或中心性的不同程度膨胀性骨质破坏,边界清楚,均有硬化边,未见软组织肿块。X线中,4例位于骨端关节面下偏心性生长,19例位于干骺端偏心性生长,6例位于骨干呈中心性生长,9例合并病理骨折。CT显示骨质破坏区为与肌肉密度相仿的软组织密度影,4例合并病理骨折,未见骨膜反应;MRI中病灶边缘均见低信号边,5例病灶信号较均匀,12例信号混杂不均合并囊变,T_2WI病灶内见斑片状及条片状低信号影,增强后肿瘤实性部分明显强化。结论原发长骨良性纤维组织细胞瘤影像表现具有一定特征性,综合分析临床资料及影像表现有助于提高术前诊断准确性。     

血管瘤样纤维组织细胞瘤临床病理观察

目的探讨血管瘤样纤维组织细胞瘤(AFH)的临床病理特征。方法对1例发生于左肩的血管瘤样纤维组织细胞瘤的临床表现、组织形态、免疫组化等进行分析,并复习相关文献。结果患者男性,19岁。左肩部缓慢生长的无痛性肿块。镜检:瘤细胞为增生的组织细胞样细胞或肌样细胞,可见含铁血黄素颗粒和假血管样腔隙、较厚的纤维性假包膜和周围淋巴细胞浆细胞浸润。免疫组化:vimentin、CD57和CD99均(+),SMA、CD68和结蛋白部分(+),CD21、CD31、CD34、CD117、CKpan、S-100、calponin和synaptophysin均(-)。结论血管瘤样纤维组织细胞瘤是一种罕见的低度恶性中间型肿瘤,临床上易与血肿、血管瘤混淆。其诊断主要靠病理形态学特征,免疫组化标记有助于诊断与鉴别诊断;肿块局部扩大切除,必要时可局部放疗,术后随访尤其重要。