近端型上皮样肉瘤临床病理研究

目的 探讨近端型上皮样肉瘤(PES)的临床病理特点、诊断及鉴别诊断依据.方法 收集5例PES患者资料,免疫组织化学EnVision法染色.第一抗体选用细胞角蛋白(CK)、波形蛋白、上皮细胞膜抗原(EMA)、CD34、β-catenin、S-100蛋白、平滑肌肌动蛋白(SMA)、肌调节蛋白(MyoD1)、结蛋白、HMB45、CK7及CK20,观察和分析其临床病理学形态及免疫表型特征.结果 5例PES中女1例,男4例;发病年龄19-46岁,发生部位分别为:会阴部2例,下腹部、髂前上棘和臀部各1例,均表现为进行性增大的无痛性单发肿块.光镜下肿瘤细胞呈结节状排列,浸润性生长,瘤细胞大部分由相对独特的上皮样细胞组成,胞质丰富,嗜酸性;核卵圆形,肿瘤中心常见坏死.免疫组织化学染色示5例瘤细胞均表达波形蛋白,4例表达CK、EMA,3例表达β-catenin、CD34,1例表达S-100蛋白;而SMA、MyoD1、结蛋白、HMB-45、CK7及CK20均阴性.结论 根据PES组织学和病理学特征,结合免疫组织化学染色结果可以做出明确诊断.     

树突状纤维黏液脂肪瘤的临床病理学研究

目的探讨树突状纤维黏液脂肪瘤的临床病理特征,并讨论与其他易于混淆的软组织肿瘤的鉴别诊断。方法收集8例树突状纤维黏液脂肪瘤,观察和分析其临床和病理组织学特征,并通过免疫组织化学标记链霉素卵白素生物素(LSAB)法,分析其CD34、bcl-2、波形蛋白、细胞角蛋白(CK)、上皮膜抗原(EMA)、S-100、HHF35和平滑肌肌动蛋白(SMA)等的表达。结果该肿瘤主要见于中老年男性皮下软组织或肌筋膜;主要分布在颈、背、肩及小腿,也可见于面部和足部。大体上边界清楚或有薄包膜,切面部分呈黏液样或胶冻状。该肿瘤在组织形态学上以成熟脂肪组织、增生的梭形细胞或星状细胞、黏液样及纤维化间质等为主要成分,在不同区域以不同的比例混杂构成。梭形细胞或星状细胞伸出细长分枝状胞质突起是其形态特征之一。无细胞异型性和核分裂象。此外,该肿瘤常有较丰富的丛状小血管和毛细血管,间质有肥大细胞、小淋巴细胞及浆细胞浸润。免疫组织化学染色梭形细胞显示CD34、bcl-2和波形蛋白强阳性,而HHF35、SMA和S-100阴性,肿瘤中所有成分对CK和EMA呈阴性反应。结论树突状纤维黏液脂肪瘤在临床表现、病理组织学改变以及免疫组织化学反应等方面有其独自的特点。该肿瘤似乎是介于梭形细胞脂肪瘤和软组织孤立性纤维性肿瘤的中间类型。需注意与黏液性脂肪肉瘤和黏液性恶性纤维组织细胞瘤鉴别诊断。该肿瘤生物学行为为良性,只需局部手术切除。     

肝原发血管肉瘤临床及病理学特点分析

目的 探讨肝原发性血管肉瘤的临床病理特点并复习相关文献.方法 搜集并分析解放军总医院病理科1988至2012年诊断的20例肝原发性血管肉瘤患者的临床表现、肿瘤病理形态特征及免疫表型特点(CD34、CD31、第八因子相关抗原、CK、GPC-3、Hepatocyte、波形蛋白、PTEN、结蛋白、CD117、Ki-67)等并进行随访.结果 20例患者中,男性11例,女性9例,平均年龄53.2岁(7～86岁),肿瘤发生部位:肝右叶11例,肝左叶6例,左右叶弥漫生长3例.大体观察:肿瘤组织呈暗红色,蜂窝状,伴出血坏死.镜下观察:形态多样,有海绵状血管瘤样、上皮样血管内皮细胞瘤样改变,移植肝复发肿瘤与肝原发肿瘤形态有差别,但均能找到较特异的血管肉瘤诊断特征.免疫组织化学染色显示肿瘤组织至少表达CD31、CD34、第八因子相关抗原中的1项,波形蛋白表达均为弥漫阳性(20/20),10例PTEN低表达(10/20),Ki-67阳性指数均大于10％,最高达60％.1例CK表达阳性(1/20),结蛋白、CD117、Hepatocyte及GPC-3均为阴性.结论 原发性肝血管肉瘤是一种罕见的预后很差的恶性肿瘤,结合组织学形态并联合应用多项血管内皮标志物可作出明确诊断.     

头皮低分化皮肤血管肉瘤的临床病理分析

目的 探讨头皮低分化皮肤血管肉瘤的临床免疫病理特征.方法 分析8例头皮低分化皮肤血管肉瘤患者的临床特点,观察其组织病理形态和CD31、CD34、第八因子相关抗原、波形蛋白、细胞角蛋白(AEl/AE3和CAM5.2)、上皮细胞膜抗原(EMA)、癌胚抗原(CEA)和S-100蛋白免疫组织化学染色.结果 患者平均发病年龄为69.0岁,男:女为5:3,临床早期常在头面部出现暗红斑,后期发生浸润性暗红斑块,均伴结节,偶发溃疡.组织病理检查示真皮广泛梭形细胞局部或广泛实性浸润,常见细胞异形,未见明确血管腔样结构.梭形细胞强阳性表达CD31、第八因子相关抗原和波形蛋白,弱阳性表达CD34、AEl/AE3和CAM5.2,而EMA、CEA和S-100蛋白阴性.结论 头面部出现淤斑样浸润性斑块和结节的老年患者,应及时做组织病理检查,CD31、CD34、第八因子相关抗原和波形蛋白免疫组织化学标记有助于明确诊断.     

血管肌纤维母细胞瘤3例并文献复习

目的 探讨血管肌纤维母细胞瘤(angiomyfibroblastoma,AMF)的临床病理特征、诊断和鉴别诊断.方法 对3例AMF进行组织学观察和免疫组化标记,抗体为vimentin、SMA、MSA、ER、PR、CD34、desmin、S-100蛋白和CK.结果 3例均表现为外阴部肿块.眼观:肿瘤境界均清楚;镜检:肿瘤均由相互交替分布的细胞密集区和细胞稀疏区组成,其间血管丰富,多为毛细血管至中等大薄壁血管.瘤细胞呈巢状或束状围绕血管排列.免疫表型:瘤细胞vimentin、SMA、ER和PR均阳性,CD34在血管壁呈阳性、瘤细胞阴性,MSA、desmin、S-100蛋白和CK均阴性.结论 AMF是一种少见的好发于外阴的良性软组织肿瘤,需与侵袭性血管黏液瘤、富细胞性血管纤维瘤等鉴别.     

骨上皮样血管内皮瘤临床病理学观察

目的 探讨骨上皮样血管内皮瘤的临床病理特征和诊断。方法 对3例骨上皮样血管内皮瘤进行临床资料分析、光镜观察和免疫组织化学检测，并结合文献进行讨论。结果 3例病变部位均为下肢。最常见的临床症状是局部疼痛。X线表现为溶骨性骨破坏，1例伴有病理性骨折。组织形态学特征是上皮样瘤细胞形成较原始的小血管腔，呈巢状、索状、不规则形分布于有黏液样变或透明变性的间质中，肿瘤组织内或边缘散布成熟的骨小梁组织。3例肿瘤均表达vimentin、FⅧRAg和CD34。随访结果2例未见肿瘤复发，1例失访。结论 原发于骨的上皮样血管内皮瘤是较少见中间型血管源性肿瘤，其组织形态学要与骨上皮样血管瘤、上皮样血管肉瘤和转移性癌等鉴别。     

微结节性胸腺瘤伴淋巴样间质3例临床病理观察

目的:探讨微结节性胸腺瘤伴淋巴样间质(micronodular thymoma with lymphoid stroma,MNT)的临床病理特征.方法:通过组织学和免疫组织化学方法观察3例MNT,研究其临床病理特征,并复习文献.结果:肿瘤有纤维性假包膜,肿块内见多发性散在或局部融合的上皮性结节,由丰富淋巴细胞间质分隔,其中可见淋巴滤泡形成.上皮性结节由温和的细长形或卵圆形细胞组成,核仁不明显,结节内淋巴细胞稀少.免疫组织化学:上皮性结节内上皮细胞CKpan,CK5/6,CK19,CK8/18均阳性,Ki67约2%阳性,CD20,EMA阴性;间隔内淋巴细胞CD20,CD3,CD5,CD99,TdT均灶区阳性,p53,CD1α均散在阳性;淋巴细胞背景内CK5/6,C8/18,EMA均阴性.结论:MNT是一种罕见的胸腺肿瘤,目前WHO归于交界性,有特殊的发病部位和形态学表现,组织学及免疫组织化学有助于该肿瘤的诊断和鉴别诊断.     

严重急性呼吸综合征肺部病变发展过程的形态观察

目的探讨严重急性呼吸综合征(SARS)肺部病变发展过程中组织形态和免疫表型的变化.方法对4例SARS的肺标本进行病理形态和1例超微结构观察,并作VG、Masson、网状纤维、地依红、PAS和天狼猩红组织化学染色及采用链霉素抗生物素蛋白-过氧化物酶(SP)法作结蛋白、波形蛋白、细胞角蛋白(CK)、上皮膜抗原(EMA)、平滑肌肌动蛋白(SMA)、HHF-35、CD34、CD68、bel-2、第八因子相关抗原(FⅧRAg)、AE1、Ⅰ型胶原、Ⅲ型胶原免疫组织化学标记.结果依病程及病变损害程度分为3期.急性渗出期2例病程＜20 d.以急性渗出性肺泡炎、肺泡上皮细胞增生,坏变和肺透明膜形成、脱屑性肺炎为主要病变,局部有纤维母细胞增生;肺泡内渗出蛋白PAS阳性,网状纤维增多并断裂,纤维母细胞明显增多,结蛋白、波形蛋白阳性.纤维增殖期1例病程25 d.以增殖性间质肺炎为主,早期纤维化表现为机化性肺炎和血管周围机化性改变.网状纤维呈肾小球样增生,间叶细胞向肌纤维母细胞分化,结蛋白、HHF-35、SMA、波形蛋白呈强阳性;向平滑肌细胞分化结蛋白、SMA呈阳性,波形蛋白阴性;向纤维母细胞分化仅波形蛋白阳性,并见弹力纤维增多,断裂,胶原纤维增生.纤维化期1例75 d.以弥漫性纤维化,以纤维增生为主的伴蜂窝肺形成.天狼猩红染色胶原纤维显红色,波形蛋白和SMA弱阳性,Ⅰ、Ⅳ胶原纤维明显增多.各期巨噬细胞均增多,CD68阳性.结论SARS肺部病变可分为3期,随病程延长,肺纤维化进行性加重;肺泡壁原始间叶细胞,肺泡上皮细胞增生、损伤和增生的巨噬细胞在肺纤维化中起着重要作用.     

特殊染色和免疫组织化学染色在肾上皮性肿瘤诊断中的应用

目的 探讨肾上皮性肿瘤的诊断及鉴别诊断。方法 对91例具有较完整病理资料的肾上皮性肿瘤进行了常规组织学观察,Mowy胶状铁染色及CD10、细胞角蛋白(CK)7、波形蛋白免疫组织化学染色。结果 91例肾上皮性肿瘤中肾透明细胞癌78例,占86％;肾乳头状癌8例,占9％;肾嫌色细胞癌4例,占4％;肾嗜酸细胞腺瘤1例,占1％。78例肾透明细胞癌中CD10、波形蛋白阳性分别为63例(81％)和69例(88％),主要表现为细胞膜阳性。74／78肾透明细胞癌CK7呈阴性,17／17 Mowy胶状铁染色阴性或呈灶状粗颗粒。肾嫌色细胞癌4例,CK7均呈胞膜阳性;Mowy胶状铁染色表现为胞质内蓝色细网状;4例CD10肿瘤细胞膜均阴性,波形蛋白阴性。肾嗜酸细胞腺癌1例CD10,CK7,波形蛋白,Mowy胶状铁染色均为阴性或不特异。结论 在常规诊断肾上皮性肿瘤有困难时免疫组织化学CD10,CK7,波形蛋白染色以及Mowy胶状铁染色对鉴别诊断有帮助。     

肾细胞癌的临床病理与免疫表型研究

目的 研究肾细胞癌的临床病理特征、预后及免疫表型特点.方法 复习114例肾细胞癌的临床病理资料、HE切片,按2004年WHO肾肿瘤分类标准重新分类、随访并进行免疫组织化学染色.结果 114例.肾细胞癌包括5个类型,肾透明细胞癌77例(67.5%)、乳头状肾癌11例(9.6%)、肾嫌色细胞癌14例(12.3%)、Xp11.2易位_/TFE3基因融合相关性肾癌10例(8.8%)、未能分类肾肿瘤2例(1.8%).免疫组织化学结果,肾透明细胞癌主要表达CK(93.5%,72/77)、CD10(93.5%,72/77)、波形蛋白(75.3%,58/77),乳头状肾癌主要表达α-甲酰基辅酶A消旋酶(AMACR,11/11),肾嫌色细胞癌主要表达CD117(11/14),Xp11.2易位/TFE3基因融合相关性肾癌TFE3、AMACR、CD10和CK的阳性率分别为10/10、10/10、9/10和7/10.结论 肾癌是一组形态学上各有特征的异质性肿瘤,在形态学基础上,CD10、波形蛋白、CD117、AMACR、CK7、TFE3有助于亚型的诊断.     

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5–44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis.     

小细胞性骨肉瘤14例临床病理分析

目的探讨小细胞性骨肉瘤的临床特征、影像学表现、病理诊断及鉴别诊断。方法收集14例小细胞性骨肉瘤的临床病理资料,按2002年WHO骨肿瘤分类标准重新复片,进行临床、影像学、病理学特征分析并采用EnVision法行免疫组化染色。结果 14例小细胞性骨肉瘤男女比1∶1。年龄12～73岁(平均34岁,中位29岁)。肿瘤多位于长管状骨干骺端(7/14,50%)。影像学表现以溶骨性骨质破坏为主伴钙化,并见软组织肿块。组织学表现为小～中等的圆形或短梭形肿瘤细胞弥漫性分布,伴有灶性纤细的花边状肿瘤性成骨。免疫表型:vimentin(10/10,100%)、CD99(7/10,70%)、BCL-2(4/10,40%)均呈阳性;S-1OO(2/10,20%)和Syn(2/10,20%)均呈弱阳性。结论小细胞性骨肉瘤为少见、特殊类型的骨肉瘤。小细胞性骨肉瘤的诊断需密切结合临床、影像学及病理特征,需与发生于骨的多种其他类型的小细胞恶性肿瘤鉴别。     

Primary peritoneal epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor of intermediate grade that occurs in a variety of organs and soft tissues. Diagnosis of epithelioid hemangioendothelioma is often complicated by the rarity of the tumor, and because the tumor shares many morphologic features with other peritoneal neoplasms. This report presents 3 cases of epithelioid hemangioendotheliomas arising as primary tumors of the peritoneum and reviews 7 previously reported cases to establish the demographic, clinical, morphologic, immunohistochemical, and ultrastructural features of this neoplasm. These cases demonstrate that the light microscopic features are very similar to epithelioid hemangioendothelioma arising at more conventional sites, such as the liver, that immunohistochemical analysis provides a reliable approach for confirming or establishing the diagnosis, and that at least one endothelial marker (either CD31, CD34, or factor VIII) should be positive for a definitive diagnosis. This series identifies the characteristics of peritoneal epithelioid hemangioendothelioma that can be reliably used for diagnosis.     

Vascular lesions of bone in children,adolescents, and young adults. A clinicopathologic reappraisal and application of the ISSVA classification

Vascular lesions of bone are rare and their terminology is not standardized. Herein, we report 77 patients with such lesions in order to characterize their morphologic spectrum and the applicability of the International Society for the Study of Vascular Anomalies (ISSVA) classification. In this system, malformations are structural anomalies distinguishable from tumors, which are proliferative. The radiologic images/reports and pathologic materials from all patients were reviewed. All lesions were either restricted to bone or had minimal contiguous soft tissue involvement with the exception of some multifocal lymphatic lesions that extensively affected soft tissue and/or viscera. We found that certain lesions of bone often regarded as tumors should be classified as malformations. Malformations (n = 46) were more common than tumors (n = 31); lymphatic and venous malformations were equally frequent. In the tumor category, hemangioendothelioma and epithelioid hemangioma were the most common. We also describe new vascular entities that arise in or involve bone. Utilizing the ISSVA approach, the diverse and often contradictory terminology of vascular lesions of bone can be largely eliminated. Standardized nomenclature is critical for scientific communication and patient management, and we hereby recommend the ISSVA classification be applied to vascular lesions of bone, just as for skin, soft tissue, and viscera.     

Multicentric epithelioid hemangioendothelioma of bone. Report of a case with radiologic-pathologic correlation

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of uncertain biologic behavior. Most cases come out as a single lesion of the soft tissue but also may appear in the lung, liver, and other locations. Epithelioid hemangioendothelioma of bone is an extremely rare tumor and more prevalent in the second and third decades of life; its behavior is uncertain, it most commonly is unifocal, and it affects preferentially lower extremities. In this work, we present the clinical, radiologic, and pathologic findings of a 19-year-old man with a multicentric EHE of bone that involved 3 vertebrae and developed lung metastasis.     

Primary vascular tumors of bone: a spectrum of entities?

Vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification systems. None of the classification schemes have been accepted due to lack of consistent terminology, accepted histologic criteria, and limited correlation with clinical outcome. It is acknowledged that vascular tumors of bone originate from endothelial cells, resulting in variable expression of endothelial markers. None of these markers are useful to discriminate between benign and malignant lesions. Although radiologic appearance is not specific, radiologic multifocality should trigger to include a vascular neoplasm in the differential diagnosis. This review gives an overview of current literature by describing all different histologic subtypes in correspondence with clinical, radiologic and genetic data. We propose the classification of vascular tumors of bone according to the three-tiered World Health Organization classification scheme for soft tissue tumors dividing entities into a benign, intermediate and malignant category. Hemangioma is the most often and commonly recognized benign lesion. Epithelioid hemangioma has been better defined over the past few years. Based on its locally aggressive behavior and occurrence of lymph node metastases, classification within the intermediate category could be considered. Angiosarcoma is the only accepted term for high-grade malignant vascular tumor of bone and so far, epithelioid hemangioendothelioma is the only accepted low-grade malignant vascular tumor of bone. It is still unclear whether other low-grade malignant vascular tumors of bone (e.g. hemangioendothelioma) truly exist. Unfortunately, molecular / genetic studies of vascular tumors of bone which might support the proposed classification are very sparse.     

老年原发性骨肉瘤12例临床及病理分析

目的 探讨老年原发性骨肉瘤的临床、影像学、病理学特征及鉴别诊断要点.方法 回顾分析北京积水潭医院1985-2010年间手术治疗的12例年龄>60岁的老年原发性骨肉瘤患者的临床、影像学及病理学资料,并对患者进行随访.结果 发病部位位于四肢长骨7例,髂骨1例,颅面骨2例及软组织2例.影像学显示,10例骨肿瘤患者为混合性骨破坏(既有成骨性又有溶骨性破坏),其中8例有骨膜反应和软组织包块.2例软组织骨肉瘤患者可见软组织内有高密度影.组织学除经典普通型骨肉瘤外,2例以恶性梭形细胞为主要成分,2例以软骨为主要成分,1例为髓内高分化骨肉瘤.选择的免疫组织化学(EnVision法)染色没有特异性的改变.10例患者接受瘤段切除或截肢手术,其中1例术前依据个人情况接受正规辅助化疗.除3例失随访外,对9例进行了跟踪随访,随访时间3个月到6年,平均2.8年,其中3例死于肺转移,1例因心脑血管疾病死亡,5例仍存活.结论 老年人原发性骨肉瘤极罕见,可能漏诊或误诊.确诊需结合临床和影像学的相关证据支持.

Abstract：

Objective To study the clinical manifestations, radiologic findings, pathologic diagnosis and differential diagnosis of primary osteosarcoma in elderly patients. Methods Twelve cases of primary osteosarcoma occurring in patients older than 60 years were encountered during the period from 1985 to 2010. The clinical manifestations, radiologic features and pathologic findings were studied and the follow-up data were analyzed. Results The sites of involvement included long bones (number=7), ilium (number=1), craniofacial bones (number=2) and soft tissue (number=2). Radiologic examination showed a mixture of osteosclerotic and osteolytic lesions in 10 patients, soft tissue lesions with high-density areas in 2 patients and soft tissue lesions with periosteal reaction in 8 patients. Histologically, most cases showed features of conventional osteosarcoma. There were 2 cases of malignant fibrous histiocytoma-like osteosarcoma, 2 cases of chondroblastic osteosarcoma and 1 case of well-differentiated intraosseous osteosarcoma. Immunohistochemical study played little role in pathologic diagnosis. Ten patients had undergone amputation, including one patient who had received adjuvant chemotherapy beforehand. Nine patients had follow-up information available. Three of them died of lung metastasis and 1 died of cardiovascular disease. Conclusions Primary osteosarcoma rarely occurs in elderly patients and can easily be missed. Correlation with clinical, radiologic and histologic features is important for arriving at a correct diagnosis.     

Intraosseous lipoma. A clinical, radiologic, and pathologic study of 5 cases

Intraosseous lipoma is an uncommon tumor of bone with indistinct radiologic features that makes it diagnostically challenging to radiologists and pathologists. There is a need to familiarize these physicians with the radiographic and pathologic features of this lesion for the correct diagnosis. We described the radiologic and pathologic features of intraosseous lipoma in 5 women. In 4 patients, the tumors occurred in long bones, whereas in the fifth patient, the skull was involved. Patients' age ranged from 50 to 63 years. Plain radiographs of the long bones revealed well-circumscribed benign-appearing osteolytic lesions with sclerotic margins, whereas in the skull, a poorly defined lytic aggressive-looking lesion was observed. In the long bones, the lesions showed remodeling of the affected bone with matrix calcification, simulating bone infarcts. Microscopically, mature adipose tissue with fat necrosis, absence of hematopoietic elements, and dystrophic calcification corresponding to the calcified matrix seen on the plain radiographs were seen. The osteolytic skull lesion had large caliber thin-walled vasculature with occasional fibrin thrombi mimicking intramuscular hemangiomas of soft tissue. On plain radiographs, an intraosseous lipoma is usually seen as a rather benign-appearing osteolytic bone lesion with well-defined margins and a heavily calcified/ossified dense matrix. Plain radiographs alone cannot establish the diagnosis of intraosseous lipoma as it mimics several other benign and malignant bone lesions. Intraosseous lipoma often contains calcified necrotic fat with little mature adipose tissue and characteristically induces expansion/remodeling of the affected bone.     

低度恶性中央性骨肉瘤的病理诊断与鉴别诊断

目的 探讨低度恶性中央性骨肉瘤(LGCOS)诊断与鉴别诊断要点.方法 收集9例LGCOS(均为会诊病例),对其临床、病理组织学和影像学进行观察和分析,并复习相关文献.结果 临床特点:9例患者中男性3例,女性6例,平均年龄约31岁.影像学均表现为成骨和溶骨混合性骨质破坏,边界不清,5例伴软组织影,3例有骨膜反应.9例均有局部疼痛和(或)肿胀,4例发生在股骨,胫骨、腓骨、颈椎、腰椎、上颌骨各1例.均行手术切除,术后未予放疗等辅助治疗,随访时间2～43个月,其中4例分别于术后25、18、8和13个月复发.病理检查:(1)大体检查:5例为碎组织,4例为块状切除标本,切面质地较韧,有不同程度的沙砾感,均无鱼肉样外观.(2)镜下观察:肿瘤由两种基本成分,即纤维成分和新生编织骨;纤维细胞密度较低,轻度异形,9例中均查见个别核分裂象;新生骨小梁均为编织骨,6例骨小梁宽大,有平行排列倾向,3例骨小梁较小,骨小梁周边均缺乏骨母细胞围绕;纤维成分和编织骨比例不一,两种成分有移行现象.病变呈浸润性生长,9例均有髓腔侵犯,3例伴有邻近软组织侵犯.结论 LGCOS细胞异形不明显、肿瘤性骨小梁较宽大成熟,易漏诊,诊断时应充分做到临床、影像学和病理三结合.临床特点:病程较长,局部缺少特征性表现.影像学:成骨和溶骨混合性病变,边界不清,骨皮质破坏或伴有邻近软组织影.病理形态:大体上缺少鱼肉样外观;镜下肿瘤性纤维成分轻度异形,可见核分裂象,肿瘤性骨小梁周边缺乏骨母细胞围绕,有平行排列倾向,纤维性成分和新生骨型小梁有直接移行现象;病变呈浸润性生长.     

低度恶性中央性骨肉瘤的病理诊断与鉴别诊断

目的 探讨低度恶性中央性骨肉瘤(LGCOS)诊断与鉴别诊断要点.方法 收集9例LGCOS(均为会诊病例),对其临床、病理组织学和影像学进行观察和分析,并复习相关文献.结果 临床特点:9例患者中男性3例,女性6例,平均年龄约31岁.影像学均表现为成骨和溶骨混合性骨质破坏,边界不清,5例伴软组织影,3例有骨膜反应.9例均有局部疼痛和(或)肿胀,4例发生在股骨,胫骨、腓骨、颈椎、腰椎、上颌骨各1例.均行手术切除,术后未予放疗等辅助治疗,随访时间2～43个月,其中4例分别于术后25、18、8和13个月复发.病理检查:(1)大体检查:5例为碎组织,4例为块状切除标本,切面质地较韧,有不同程度的沙砾感,均无鱼肉样外观.(2)镜下观察:肿瘤由两种基本成分,即纤维成分和新生编织骨;纤维细胞密度较低,轻度异形,9例中均查见个别核分裂象;新生骨小梁均为编织骨,6例骨小梁宽大,有平行排列倾向,3例骨小梁较小,骨小梁周边均缺乏骨母细胞围绕;纤维成分和编织骨比例不一,两种成分有移行现象.病变呈浸润性生长,9例均有髓腔侵犯,3例伴有邻近软组织侵犯.结论 LGCOS细胞异形不明显、肿瘤性骨小梁较宽大成熟,易漏诊,诊断时应充分做到临床、影像学和病理三结合.临床特点:病程较长,局部缺少特征性表现.影像学:成骨和溶骨混合性病变,边界不清,骨皮质破坏或伴有邻近软组织影.病理形态:大体上缺少鱼肉样外观;镜下肿瘤性纤维成分轻度异形,可见核分裂象,肿瘤性骨小梁周边缺乏骨母细胞围绕,有平行排列倾向,纤维性成分和新生骨型小梁有直接移行现象;病变呈浸润性生长.