子宫黏液性平滑肌肉瘤临床病理分析

子宫黏液性平滑肌肉瘤是一种罕见的子宫间叶来源的肿瘤,首先由King等[1]报道。子宫黏液性平滑肌肉瘤是平滑肌肉瘤的一种亚型,以大量的黏液样基质、低的有丝分裂数、轻度的细胞异型、浸润性的边界和具恶性的生物学行为为特征。现就我们遇到的3例子宫黏液性平滑肌肉瘤,结合文献报道,探讨其临床病理特征、免疫表型、鉴别诊断及预后。1材料与方法1.1材料收集浙江大学附属妇产科医院2001年1月~2005年10月子宫全切标本诊断为子宫黏液性平滑肌肉瘤3例。1.2方法标本用10%福尔马林固定,常规脱水,石蜡包埋,4μm厚切片,HE染色,光镜观察。并行免疫组化…     

子宫黏液性平滑肌肉瘤一例

患者女 ,5 2岁。下腹包块 3年 ,近 2个月肿块明显增大 ,伴下腹坠胀、双腿酸软、阴道出血 12ｄ于 2 0 0 1年 5月 2 5日入院。 3年前普查发现下腹部包块 ,既往月经规律 ,8个月前闭经。体检 :全身浅表淋巴结未扪及 ,腹部平坦 ,未扪及肿物 ,未发现移动性浊音。妇科检查 :子宫如孕 6个月大 ,质硬、压痛明显、活动性差、表面不规则。双附件未扪及包块。Ｂ超检查 :子宫前位 ,增大 ,形态不规则 ,被膜不光滑 ,肌层回声不均匀 ,于子宫前壁探及 11.3ｃｍ× 9.5ｃｍ、5 .5ｃｍ× 5 .9ｃｍ ,5 .2ｃｍ× 4.2ｃｍ的回声不均匀区 ,提示 :多发性子宫肌瘤。术…     

子宫黏液样平滑肌肉瘤的临床病理诊断

目的 :探讨子宫黏液样平滑肌肉瘤 (MLU)的临床病理特征及诊断、鉴别诊断要点。方法 :对 1例MLU进行大体、光镜及免疫组化观察 ,并复习相关文献。结果 :MLU的临床症状是盆腔包块及不规则阴道流血 ;其突出的病理特征是肿瘤切面呈弥漫性胶冻状外观 ,镜下见大量奥辛蓝阳性的黏液样基质 ,瘤细胞大部分梭形显平滑肌细胞分化 ,并浸润周围正常平滑肌组织和血管腔 ;免疫组化 :瘤细胞HHF35 ( )。结论 :MLU是子宫平滑肌肉瘤的一个罕见变型 ;由于大多数MLU缺乏细胞异型性和核分裂象计数很低 ,常易引起病理误诊 ,在冷冻切片中更是如此。鉴别诊断必须首先区别常见的子宫平滑肌瘤黏液变性或水肿变性 ,其次还应与任何黏液样软组织恶性肿瘤相鉴别     

子宫分割性平滑肌瘤1例临床病理分析

目的探讨子宫分割性平滑肌瘤临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例子宫分割性平滑肌瘤的临床病理特征、免疫表型,并复习相关文献。结果患者56岁,B超示盆腔低回声包块,大小15 cm×14 cm×9 cm。MRI示子宫后壁一宫底肌层内肿块影、子宫两旁不规则块状影来源不确定,子宫后壁及双侧附件平滑肌瘤变性?或子宫腺肉瘤合并双侧附件性索来源肿瘤?镜检见肿瘤组织呈分化良好的平滑肌组织形态,沿肌壁局限型分割性生长,无坏死,未见核分裂象,呈丛状分布,平滑肌瘤结节之间明显水肿,平滑肌绕血管或血管丛呈漩涡状或不规则排列。免疫表型:子宫分割性平滑肌瘤瘤细胞表达ER、PR、vimetin、H-caldesmon、desmin、SMA均强阳性,CD34血管阳性,CD117、DOG1、CD10、CD99、HMB-45、S-100、CK和EMA均阴性,Ki-67增殖指数1%。结论子宫分割性平滑肌瘤是平滑肌瘤中极为罕见的亚型,病理诊断尤其是术中冷冻切片易误诊。     

未分化子宫内膜肉瘤6例临床病理观察

目的 探讨未分化子宫内膜肉瘤(undifferentiated endometrial sarcoma,UES)的临床病理特点、诊断及鉴别诊断.方法 对6例未分化子宫内膜肉瘤的临床资料、组织学形态及免疫组化结果进行观察分析.结果 患者年龄49～71岁,平均年龄59岁,临床主要表现为宫腔内占位和阴道出血;肿瘤大体呈息肉样,突入宫腔;镜下见肿瘤组织分化差,细胞异型明显,核分裂象丰富(＞10个/10 HPF),坏死常见;免疫组化标记,肿瘤细胞CD10、ER、PR、desmin、SMA、CK、EMA阴性,vimentin、EGFR阳性;6例随访6个月～4年,3例死于肺转移.结论 未分化子宫内膜肉瘤是一种少见的子宫内膜间质肿瘤,具有高度恶性.肿瘤组织分化差,细胞异型明显,常见坏死,免疫组化标记有助于诊断与鉴别.     

低度恶性纤维黏液样肉瘤临床病理分析

目的:探讨低度恶性纤维黏液样肉瘤(low-grade fibromyxoid sarcoma,LGFMS)的临床病理特征和诊断要点.方法:分析3例LGFMS的临床资料,观察组织学形态、免疫表型及分子病理检测结果,讨论鉴别诊断并复习相关文献.结果:患者均为女性,中位年龄57岁,肿瘤分别位于右侧膝关节前方、右侧乳腺和左侧上颌窦.肿块最大径为2.2～10.0 cm,边界欠清,似有包膜,切面灰白,部分区域呈半透明状,质中有黏液感.镜下见肿瘤组织主要由梭形纤维母细胞样细胞构成,包括两种形态区域,其一为细胞稀疏的胶原样区域;其二为细胞相对较丰富的黏液样区域.肿瘤组织中可见较多弓形血管并伴有血管周玻璃样变性.瘤细胞形态温和,无明显异型性,核分裂象罕见.免疫组织化学染色结果示:vimentin,MUC4,CD99及bcl-2阳性表达,SMA,desmin,S100,CD34,ALK及myogenin阴性表达.发生于右侧乳腺的病例行荧光原位杂交(fluorescent in situ hybridization,FISH)检测,检出FUS基因易位.发生于右侧膝关节和乳腺的2个病例分别随访20个月和51个月,患者均无瘤生存.发生于左侧上颌窦的病例于手术后12个月死亡.结论:LGFMS常见于年轻人,但各年龄段患者均可受累.好发部位为下肢近端和躯干,少见于头颈部和乳腺.LGFMS具有温和的多样性的组织学形态,容易误诊为具有黏液样结构的其他梭形细胞肿瘤.LGFMS具有转移和复发的恶性生物学行为,治疗上需对肿块作完整切除并长期随访观察.     

子宫内膜间质肉瘤9例临床病理分析

目的 探讨子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)的临床病理特征、诊断、鉴别诊断及预后.方法 对9例ESS患者进行临床、病理资料分析、免疫组化检测及随访.结果 患者年龄39～64岁,中位46.3岁.临床主要表现为阴道流血及子宫增大/占位.肿瘤直径2.3～11 cm,平均4.6 cm.光镜下8例呈低度恶性子宫内膜间质肉瘤(low grade endometrial stromal sarcoma,LGESS),均由类似增殖期子宫内膜间质肿瘤细胞构成,细胞密集,异型性不明显,呈不规则舌状或岛状浸润肌层,并伴较多薄壁螺旋小血管;1例为高度恶性子宫内膜间质肉瘤/未分化子宫内膜肉瘤(high grade endometrial stromal sarcoma/undifferentiated endometrial sarcoma,HGESS/UES),肿瘤细胞直接替代子宫肌层,具有明显的细胞异型性,无LGESS常见的螺旋小血管.免疫组化检测显示肿瘤细胞CD10、vimentin均阳性,PR、ER大部分阳性,SMA和desmin及h-Caldesmon为极少数局灶阳性,S-100、CD34均阴性.术后随访7例(平均53个月),只有1例HGESS/UES死亡.结论 ESS是女性生殖道很少见的一种恶性肿瘤,恶性度相差很大.确诊主要依靠其临床病理特点,并辅以免疫组化标记综合分析.诊断时要与子宫内膜间质结节、平滑肌肿瘤、低分化癌等鉴别.     

子宫腺肉瘤的临床病理特点及鉴别诊断

目的：分析子宫Ｍｕｅｌｌｅｒｉａｎ腺肉瘤的临床病理特点及其鉴别诊断。方法：观察１３例子宫腺肉瘤临床病理特点，并进行组织化学和免疫组化染色分析。结果：病人年龄２６￣６７岁，主要表现为阴道不规则流血和子宫增大。肿瘤位于宫体内膜、宫颈内膜和子宫肌壁。镜下腺上皮呈Ｍｕｅｌｌｅｒｉａｎ上皮系列。间质细胞异型分级为Ⅰ￣Ⅱ级，核分型４￣３２个／１０ＨＰＥ，具有腺周套袖和息肉样突入腺腔的结构。４例伴肉瘤过度生长。     

黏液纤维肉瘤3例临床病理分析

目的 探讨黏液纤维肉瘤(myxofibrosarcoma,MFS)的临床病理学特点、免疫学表型及鉴别诊断.方法 对3例MFS进行临床病理学和免疫组化分析,并复习相关文献.结果 2例为男性,1例为女性,年龄分别为63岁、74岁及40岁;2例为左大腿包块,1例发生于头顶部;镜下黏液背景中肿瘤细胞多结节状分布,瘤细胞梭形,其间可见空泡状假脂母细胞和细长弓形血管,3例分别为低度、中度及高度恶性.免疫组化显示瘤细胞除vimentin弥漫强阳性外,MSA、α-SMA、Myoglobin、desmin、S-100、CD34、CD57、CD68、PCK、EMA及Bcl-2均为阴性.结论 MFS是一种纤维母细胞性恶性肿瘤,可以根据病理形态及免疫组化加以诊断及鉴别诊断.     

子宫内膜间质肉瘤5例临床病理分析

目的 探讨子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)的临床病理特点、诊断、鉴别诊断及免疫表型.方法 回顾性分析5例ESS患者的临床资料,探讨其病理特点、免疫表型及预后等相关因素.结果 5例ESS中,低度恶性4例,高度恶性1例,镜下见低度恶性密集排列的类似增殖期子宫内膜间质细胞围绕螺旋小动脉样的血管分布,核分裂少见,高度恶性瘤细胞大,异型性明显,小血管数量减少,核分裂多见,浸润明显,伴坏死,免疫表型:CD10、vimentin、ER、PR均阳性,CK、CD34、Melan-A均阴性.结论 ESS是女性生殖道少见肿瘤,术前常误诊为平滑肌瘤,确诊主要依靠组织病理学、免疫表型来判断肿瘤有无浸润及恶性程度的高低.CD10可作为ESS的鉴别诊断的重要标记之一.     

骨外黏液样软骨肉瘤病理与鉴别诊断附4例报道并文献复习

目的 探讨骨外黏液样软骨肉瘤(extraskeletal myxoid chondrosarcoma,EMC)的临床病理特点、免疫表型及病理诊断与鉴别诊断要点.方法 收集4例EMC,对其临床、病理组织学及免疫表型进行观察并复习相关文献.结果 4例患者均为成年男性,平均年龄40.2岁,平均病程约30.3个月.主要表现为无痛性或有触痛性软组织肿块,3例发生于下肢,1例发生于胸壁.病理检查:(1)眼观见瘤体最大径平均6.5 cm,切面呈多结节状,有黏液感,界清.(2)镜检见肿瘤呈分叶状生长,瘤细胞排列呈索状、簇状或纤细网状.3例间质富于黏液样基质,1例富于软骨样基质,血管较稀疏.免疫组化:4例均表达Vim(4/4),3例表达S-100蛋白(3/4),2例表达NSE(2/4),2例表达CgA和Syn(2/4),1例表达CKpan(1/4),1例Ki-67阳性率为50%,3例Ki-67阳性率均<5%,4例均不表达SMA和EMA.结论 EMC是一种罕见的软组织肿瘤,其诊断主要依靠发生部位和组织病理学特征,免疫组化标记可帮助诊断和鉴别诊断.     

Pulmonary myxoid leiomyosarcoma

A primary myxoid leiomyosarcoma arising from the peripheral bronchus of the right middle lobe was removed from a 20 year old man and examined by immunohistochemistry and electron microscopy. The tumor was well circumscribed, yellow-whitish focal polypoid growth into the bronchial lumen and consisted of spindle cells with abundant myxoid substance in the stroma. Intrapulmonary metastasis, invasion to the bronchial wall and a few mitotic figures were found. Immunohistochemically, several, but not all, tumor cells were positive against anti-vimentin, anti-S-100 protein, anti-myosin and anti-muscle specific actin. Ultrastructurally, tumor cells had thin filaments with dense bodies, pinocytic vesicles and discontinuous basal lamina. These findings indicate a myoxoid variant of leiomyosarcoma arising from mesenchyme in the peribronchial area.     

Primary cutaneous leiomyosarcoma: clinicopathological analysis of 36 cases

Massi D, Franchi A, Alos L, Cook M, Di Palma S, Enguita A B, Ferrara G, Kazakov D V, Mentzel T, Michal M, Panelos J, Rodriguez‐Peralto J L, Santucci M, Tragni G, Zioga A & Tos A P D
(2010) Histopathology 56, 251–262 Primary cutaneous leiomyosarcoma: clinicopathological analysis of 36 cases Aims: Cutaneous leiomyosarcomas (LMS) are rare in comparison with their deep‐seated soft tissue and uterine counterparts, and have been poorly characterized. The aim was to verify whether the clinical behaviour of purely dermal LMS is different from that of LMS with minimal subcutis invasion. Methods and results: Twenty‐one purely dermal LMS and 15 dermal LMS with minimal subcutis extension were analysed. Tumours developed in 27 men and nine women (age range 29–91 years); most tumours showed a fasciculated (n = 23), pilar‐type (n = 12) and pleomorphic (n = 1) pattern. During the follow‐up period (range 2–192, mean 41 months) recurrences occurred in 1/16 (6.2%) of tumours confined to the dermis and in 2/11 (18.1%) tumours with minimal subcutis extension. The three recurrent tumours were high‐grade LMS, two of which exhibited myxoid areas. One patient with a pleomorphic dermal LMS with minimal extension into fat developed distant metastases 15 years after diagnosis. Conclusions:  For LMS involving the skin, it is advisable to recognize and indicate in the histopathology report the depth of dermal and/or subcutaneous extension, since even minimal subcutaneous involvement may be associated with late local recurrences and/or distant metastases, and therefore appropriate and long‐term follow‐up is needed.     

Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases

Chen E, O’Connell F & Fletcher C D M
(2011) Histopathology  59 , 1135–1143
Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases Aims: To clinicopathologically characterize the dedifferentiated variant of leiomyosarcoma in a series of 18 cases. Methods and results: Dedifferentiated leiomyosarcoma was defined as showing features of low‐grade leiomyosarcoma associated with a discrete undifferentiated component lacking morphological or immunophenotypic features of myogenic differentiation. Tumours developed in 11 women and seven men, with an age range of 16–84 years (median, 64 years). Sites were retroperitoneum (eight cases), limbs (four), trunk (two) uterus (two), and paratesticular and prostate (one each). In 17 cases, dedifferentiation occurred de novo in the primary tumour. Tumour size ranged from 50 to 280 mm (median: 120 mm). Histologically, most showed discrete transition from well‐differentiated smooth muscle morphology to high‐grade pleomorphic morphology with no smooth muscle differentiation. Unusual features in the dedifferentiated component (epithelioid and rhabdomyoblast‐like morphology) were present in three cases. Heterologous osseous or chondro‐osseous elements were present in two cases. Dedifferentiated areas were negative for myogenic markers in all cases. Follow‐up for 13 cases (median, 36 months) showed local recurrence in 38% (5/13). So far, three patients have died of disease (median survival, 8 months), and metastasis developed in five of 13 cases. Conclusions: Dedifferentiated leiomyosarcoma has morphological parallels with other types of dedifferentiated sarcoma, and is clinically aggressive.     

Peritoneal washing cytology findings of disseminated myxoid leiomyosarcoma of uterus: report of a case with emphasis on possible differential diagnosis

We report the cytology findings of a rare case of myxoid leiomyosarcoma of the uterus with intraabdominal dissemination. The cytology showed uniformly dispersed spindly to polygonal "epithelioid" tumor cells focally linked by background matrix. Spindly tumor cells attaching to and radiating from branching capillary structures were also present. Occasionally, scattered "signet-ring" cells were found, mimicking metastatic poorly differentiated adenocarcinoma. The background mucoid substance was more conspicuous in the cell block sections. Gross and histologic examination of the surgical specimen revealed a large uterine leiomyosarcoma with prominent myxoid change. Ultrastructural study showed that the "signet-ring" appearance was mainly due to degenerative cytoplasmic change with ballooning of mitochondria, dilatation of endoplasmic reticulum, and elevation of outer nuclear membrane. In contrast to other spindle cell malignancies such as sarcomatoid mesothelioma, sarcomatoid carcinoma, or malignant melanoma, true sarcoma cells in general carry a distinctive cytologic appearance in washing/effusion fluid. In a correct clinical setting, the peculiar association with mucoid matrix and absence of classic lipoblasts should also raise the suspicion of metastatic myxoid leiomyosarcoma.     

骨外黏液样软骨肉瘤2例报道及文献复习

目的探讨骨外黏液样软骨肉瘤的临床病理学特征。方法对2例骨外黏液样软骨肉瘤进行光镜观察及免疫组化染色标记,并通过相关文献复习,对病理诊断及鉴别诊断等指标进行分析。结果1例发生于足底,1例发生于乳腺。光镜下肿瘤呈分叶状,边界清。细胞为圆形及短梭形,成束状排列于黏液样基质中,局部可见围血管形成玫瑰花结样结构。部分肿瘤细胞异型性明显。免疫表型:vimentin、NSE、Syn均呈阳性;例1EMA灶性阳性,例2阴性;S-100蛋白、CgA及CK均阴性。结论骨外黏液样软骨肉瘤为罕见的软组织恶性肿瘤,具特异性的组织病理学特点。主要发生于四肢,少数可发生于实质器官,至今未有乳腺原发病例报道。部分肿瘤细胞可发生间变导致诊断困难,须与脊索瘤、骨内软骨肉瘤、化生性癌及黏液性肿瘤等鉴别。     

浅表性平滑肌肉瘤临床病理分析

目的:探讨浅表性平滑肌肉瘤(superficial leiomyosarcoma,SLMS)的临床病理特点、诊断、鉴别诊断及治疗和预后.方法:回顾性分析13例SLMS的临床病理特征,并复习相关文献讨论.结果:男7例,女6例,平均年龄59岁.肿瘤位于四肢7例,躯干3例,头面部2例,阴囊1例,其中位于真皮的9例,皮下4例.肿瘤直径1~8(平均4.3)cm.局部复发5例,无远处转移病例.镜下:肿瘤呈结节状或弥漫状生长,根据肿瘤细胞不同分化分为三型:中分化(最多见,9例)、高分化和低分化型(较少,各2例).免疫组织化学:肿瘤细胞desmin,MSA,a-SMA和h-caldesmon阳性.结论:SLMS多发生于中老年人,预后较好,易发生局部复发,极少发生远处转移.治疗采用局部扩大切除.     

子宫浆液性乳头状癌24例临床病理分析

目的比较子宫浆液性乳头状癌(uterine papillary serous carcinoma,UPSC)和子宫内膜样癌(uterine endometrioid carcino-ma,UEC)的组织病理学和免疫组化表达,以了解两种肿瘤生物学行为的差异。方法对24例UPSC和54例UEC进行组织学复查和应用免疫组化SP法检测肿瘤的p53蛋白、ER和PR的表达。结果24例UPSC占子宫内膜癌的3·77%,平均年龄UP-SC组为60岁,UEC组为51·7岁(P<0·01),晚期癌(FIGOⅢ-Ⅳ)UPSC组占62·5%,UEC组占35·1%(P<0·025)。p53蛋白的表达UPSC组16例阳性(66·7%),UEC组10例阳性(18·5%),两组比较(P<0·01)。ER阳性表达UPSC组(8·3%),UEC组(42·5%),PR阳性表达UPSC组(12·5%),UEC组(35·2%),两组比较:ER(P<0·01),PR(P<0·05),差异有显著性。UPSC组p53蛋白表达与肿瘤分期、分级、及肌层浸润无明显关系,而UEC组Ⅲ~Ⅳ期肿瘤的p53蛋白的表达率高于Ⅰ-Ⅱ期(P<0·005)。UPSC的5年生存率为25%,UEC组5年生存率为80·9%(P<0.01),两组差异有显著性。结论UPSC为p53高表达,而缺乏雌激素和孕激素受体,为高度恶性的肿瘤。它的生物学行为不同于UEC,因而强调诊断时需和其他类型的子宫内膜癌相区别。     

骨原发性平滑肌肉瘤5例临床病理特征

目的：探讨骨原发性平滑肌肉瘤的临床病理学特征、诊断与鉴别诊断、治疗及预后。方法对5例骨原发性平滑肌肉瘤进行组织病理学、免疫表型及影像学分析,并复习相关文献。结果5例骨原发性平滑肌肉瘤发病年龄46~72岁,平均58岁;其中男性2例,女性3例;临床主要症状为疼痛,2例位于干骺端膝关节附近,1例位于股骨上段,1例位于骶骨,另1例位于上颌骨。镜下瘤细胞梭形,少量呈上皮样,瘤细胞均表达vimentin、SMA,其中2例表达desmin,S-100、MyoD1、CD68均阴性。结论骨原发性平滑肌肉瘤较少见,临床症状和影像学表现无特异性,确诊需借助免疫组化标记和电镜检查。     

246例子宫平滑肌肿瘤临床病理分析

目的:探讨子宫平滑肌肿瘤临床病理特征.方法:对246例子宫平滑肌肿瘤的临床病理资料进行回顾性分析.结果:子宫平滑肌肿瘤发病高峰年龄为40～50岁,可伴内膜增生或腺肌病,同时与卵巢、宫颈多种良恶性病变并存.246例子宫平滑肌肿瘤中,91.4%为普通型良性平滑肌瘤;6.9%为平滑肌瘤特殊组织学类型;0.8%为平滑肌肉瘤;0...