子宫黏液性平滑肌肉瘤6例临床病理分析

目的探讨子宫黏液性平滑肌肉瘤(uterine myxoid leiomyosarcoma,UML)的临床病理特征、诊断、鉴别诊断。方法对6例UML进行HE、免疫组化En Vision法及特殊染色,并复习相关文献及随访。结果 6例患者年龄36~57岁(平均47.33岁),肿块直径4.5~14 cm(平均9.25 cm)。低倍镜下肿瘤细胞排列呈片状、束状、簇状或星网状,间质见大量黏液样基质;瘤细胞呈梭形、短梭形、卵圆形。胞质少至中等,胞质嗜酸性,部分肿瘤细胞可见胞质内空泡。核分裂象多少不等(2~20个/10HPF),可见坏死(3/6)。瘤细胞浸润子宫肌壁平滑肌组织,侵犯脉管,并可见脉管内瘤栓,其中1例细胞具有上皮样特征。免疫表型:肿瘤细胞α-SMA、desmin、h-caldesmon、ER、PR均阳性,CD10、S-100、CD34均阴性,Ki-67增殖指数10%~40%。结论 UML属于罕见恶性间叶源性肿瘤,确诊主要依靠形态学及免疫表型。临床及病理均需与黏液性平滑肌瘤、黏液性子宫内膜间质肿瘤及黏液性纤维肉瘤等鉴别,治疗以手术切除为主,并辅以化疗,预后较好。     

骨内侵袭性血管黏液瘤的诊断及与其他黏液性骨肿瘤的鉴别

目的探讨骨内侵袭性血管黏液瘤(aggressive angio-myxoma,AAM)的临床病理特征、诊断及鉴别诊断。方法对1例骨内AAM的临床、影像学和病理特征进行观察,并通过HE、免疫组化染色将其与1例软组织AAM、1例黏液样软骨肉瘤(myxofibrosarcoma,MFS)、1例黏液纤维肉瘤(myxofi-brosarcoma,MFS)、2例黏液性脂肪肉瘤(myxoid/round cellliposarcoma,ML/RCL)及4例肌内黏液瘤(intramuscularmyxoma,IM)进行对比分析。结果镜下骨内AAM由稀疏排列的细胞及富含黏液的水肿性间质组成,细胞呈星形或梭形,部分细胞呈肌纤维母细胞样,细胞核小,梭形,细胞间可见疏松排列的纤细红染的胶原成分,胶原间可见大小不等扩张的血管,局部可见肿瘤浸润骨皮质进入周围肌肉组织。免疫表型:所有肿瘤组织均表达vimentin,骨内AAM与软组织AAM尚表达SMA、actin,未检测到ER、PR的表达,软组织AAM表达PR及CD34,两者均未检测到desmin的表达。黏液样软骨肉瘤及黏液性脂肪肉瘤尚表达S-100,黏液纤维肉瘤及肌内黏液瘤尚表达CD68。结论骨内AAM罕见,诊断时应结合组织学及免疫组化特点并与其他含黏液的肿瘤相鉴别。     

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…     

鼻咽低度恶性纤维黏液样肉瘤2例报道并文献复习

目的探讨鼻咽低度恶性纤维黏液样肉瘤(Low-gradefibromyxoid sarcoma,LGFMS)的临床病理特征、免疫组化、诊断、鉴别诊断和预后。方法对2例鼻咽LGFMS进行组织学和免疫组化观察并结合文献复习进行分析。结果 2例均为女性,年龄分别为39岁和40岁。CT检查鼻咽部占位性病变。镜下肿瘤由纤维区和黏液样区混合组成,二者呈交错相间排列;纤维性区瘤细胞排列呈旋涡状;黏液样区瘤细胞散在分布;细胞核轻度异型。PAS染色黏液样间质区和肿瘤细胞均为阴性。免疫组化标记vimentin、CD34阳性。随访无复发。结论鼻咽LGFMS少见,是一种来源于纤维母细胞、进展缓慢的低度恶性软组织肉瘤,易误诊为良性。生物学行为属于低度恶性肿瘤。病理诊断依赖于组织化学和免疫组化检查。     

子宫炎性肌纤维母细胞肿瘤3例临床病理学分析

目的 探讨子宫炎性肌纤维母细胞肿瘤(inflammatory myofibroblastic tumor, IMT)的临床病理特征。方法 对3例子宫IMT行HE染色、免疫组化及FISH检测,并结合相关文献总结其临床病理特征、鉴别诊断、预后与治疗。结果 患者年龄分别为37、35和40岁,均以阴道不规则出血和(或)月经紊乱就诊,影像学示肿瘤位于子宫颈或宫腔。镜检：瘤细胞呈梭形、胞质淡染、可见核仁,瘤细胞低倍镜下均具有平滑肌瘤样形态,2例肌纤维母细胞样成分和平滑肌样成分相互交错分布,局灶黏液样变性,1例间质呈疏松黏液样变性,伴淋巴细胞及浆细胞等炎细胞浸润;核分裂象≤5个/10 HPF。免疫表型：ALK和SMA阳性(3/3),desmin、H-Caldesmon阳性(2/3),CD10灶阳性(1/3),Ki-67增殖指数5%～10%。FISH检测示：ALK基因断裂重排(3/3)。随访时间分别为75、9、15个月,2例无瘤生存,1例复发2次。结论 子宫原发IMT罕见,临床表现及形态学易误诊为子宫平滑肌肿瘤,明确诊断需要细致形态学辨识,并通过免疫组化及FISH检测证实。     

子宫上皮样滋养细胞肿瘤的临床病理特征

目的探讨子宫上皮样滋养细胞肿瘤(epithelioid trophoblastic tumor,ETT)的临床病理特征。方法收集7例子宫ETT的临床病理资料,进行病理学分析,并复习相关文献。结果 7例ETT发病年龄31～45岁(平均37.6岁),距前次妊娠6～216个月(平均94个月),临床表现闭经和阴道不规则流血。大体上肿瘤呈膨胀性生长,直径0.5～10cm,镜下显示典型的上皮样瘤细胞巢、"地图样"坏死、"血管瘤细胞套样"结构,4例合并子宫平滑肌瘤。免疫组化:CK18、CD10、p63均呈阳性,hPL、PLAP、CD146、hCG、inhibin部分病例灶状阳性,Ki-67增殖指数5%,p53阳性率4例5%。结论 ETT是罕见的具有恶性潜能的绒毛膜中间型滋养细胞肿瘤,需与子宫上皮样平滑肌肉瘤、宫颈鳞癌及其它滋养细胞疾病等鉴别,诊断和鉴别诊断主要依据临床病史、病理学形态及免疫组化。     

肾透明细胞肉瘤的临床病理及免疫表型特征

目的 探讨肾透明细胞肉瘤（clear cell sarcoma of the kidney,CCSK）的临床病理特点、免疫表型特征及鉴别诊断。方法 应用HE和免疫组化vimentin、bcl-2、desmin、S-100蛋白、CD99、CD34、CDll7、CK、EMA染色,观察2例CCSK的病理组织学形态,并复习文献。结果 镜下见瘤细胞为上皮样或短梭形,被分枝状纤维血管间质分隔成巢团状,部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示：瘤细胞vimentin和bcl-2弥漫阳性,余为阴性。结论 CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。     

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。     

伴有特殊形态子宫内膜间质肉瘤10例临床病理特征分析

目的探讨低级别子宫内膜间质肉瘤(low grade endometrial stromal sarcoma,LGESS)变异形态的临床病理特点。方法回顾性分析10例LGESS的临床病理特征、免疫表型特征并对患者进行随访。采用免疫组化En Vision两步法检测CD10、vimentin、ER、PR、SMA、desmin、H-caldesmon、α-inhibin和Ki-67的表达,并复习相关文献。结果10例LGESS平均发病年龄47. 5岁,肿块最大径4. 1～12. 2 cm;镜下除经典形态外,8例见黏液变,4例伴平滑肌分化,3例呈印戒细胞样形态,2例伴纤维化,2例间质出现石棉样纤维,2例微囊性变,1例伴广泛玻璃样变性,1例同时出现性索样、腺管样、腺瘤样瘤样、梁状、囊性变、伴横纹肌分化等形态,1例子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)卵巢转移灶见蜕膜样形态。10例LGESS均表达CD10、vimentin,1例ER、PR阴性,ESS瘤细胞中Ki-67增殖指数均10%,1例伴性索样分化ESS局部α-inhibin阳性,在伴有平滑肌分化的区域H-caldesmon阳性(4/10)、desmin(4/10)阳性、SMA(6/10)阳性。结论 LGESS特殊变异形态给病理医师带来病理诊断上的挑战,联合应用免疫组化及基因检测可为临床提供更精准的病理诊断。     

关节旁黏液瘤临床病理特点

目的探讨关节旁黏液瘤(Juxta-articular myxoma,JAM)临床病理特点和鉴别诊断。方法对1例JAM进行组织形态学观察、免疫组化标记并复习文献。结果肿块位于左胫骨内侧髌骨下缘,不规则组织7cm×3cm×2cm,境界不清,切面周围为淡黄色脂肪组织,中央大部分区域呈黏液胶冻状。镜检:梭形、星芒状纤维母细胞样瘤细胞稀疏散在分布于丰富的黏液样基质中,细胞形态良善,间质血管稀少。部分区域血管丰富。散在有形状、大小不同的囊性腱鞘囊肿样腔隙。肿瘤界限不清,内有脂肪组织陷入。特殊染色:黏液样基质阿辛蓝弥漫(+)。免疫表型:瘤细胞Vim(+),部分瘤细胞α-SMA、CD34(+)。随访8个月未见复发。结论 JAM为良性病变,组织形态和免疫表型类似于肌内黏液瘤,见于膝、肩、肘、踝、髋等大关节旁。约有1/3病例复发,故长期随访是必要的。需与黏液性脂肪肉瘤、黏液纤维肉瘤、骨外黏液性软骨肉瘤、低度恶性纤维黏液样肉瘤等鉴别,避免过度治疗。     

Uterine myxoid leiomyosarcoma within a leiomyoma

A case of myxoid leiomyosarcoma of the uterus arising in a leiomyoma is reported. Although the tumor showed very low mitotic activity ranging from zero to 2/10 HPF, the presence of infiltrative pattern of growth and a high MIB-1 index (60% of cells positive) established the diagnosis. Myxoid leiomyosarcoma may arise in leiomyoma.     

Myxoid variant of epithelioid leiomyosarcoma of the uterus.

A rare case of myxoid variant of epithelioid leiomyosarcoma of the uterus in a 76-year-old woman is reported. Palpation and computed tomography revealed an enlarged uterus. Total hysterectomy and bilateral salpingo-oophorectomy were performed, and a hemispheric tumor, measuring 2.7 x 2.5 x 1.8 cm, was found protruding into the uterine cavity of the upper uterine segment. The tumor contained a prominent myxoid stroma and epithelioid tumor cells, which were round and polygonal in shape and showed positive immunoreactivity for desmin and vimentin. High mitotic activity was observed in the tumor cells. Electron microscopic examination revealed bundles of filaments in the cytoplasm and fine reticular material in the extracellular matrix. Histochemically, the myxoid stroma contained abundant acid mucopolysaccharide. The tumor cells were considered to originate from smooth muscle cells, while the myxoid stroma expressed varying differentiation of uterine mesenchymal cells. The patient is currently well with no evidence of recurrence or metastasis one year after the operation.     

Myxoid leiomyosarcoma of the uterus: a case report and review of the literature.

A case report of myxoid leiomyosarcoma of the uterus in a 54-year-old woman is presented as a rare variant of uterine sarcoma. Only 14 cases have been described in the literature. These tumors have a striking myxoid appearance and exhibit highly malignant behavior despite their low mitotic index. Although it is difficult to establish the nature of the neoplastic cells in the myxoid areas, both light microscopical and immunohistochemical characteristics showed features of smooth muscle cells in some of the cellular areas. Our patient had a tumor which arose in association with hyalinized benign leiomyomatous lesion. Like other reported cases of myxoid leiomyosarcoma, the tumor behaved aggressively.     

Myxoid Variant of Epithelioid Leiomyosarcoma of the Uterus

A rare case of myxoid variant of epithelioid leiomyosarcoma of the uterus in a 76-year-old woman is reported. Palpation and computed tomography revealed an enlarged uterus. Total hysterectomy and bilateral salpingo-oophorectomy were performed, and a hemispheric tumor, measuring 2.7 X 2.5 X 1.8 cm, was found protruding into the uterine cavity of the upper uterine segment. The tumor contained a prominent myxoid stroma and epithelioid tumor cells, which were round and polygonal in shape and showed positive immunoreactivity for desmin and vimentin. High mitotic activity was observed in the tumor cells. Electron microscopic examination revealed bundles of filaments in the cytoplasm and fine reticular material in the extracellular matrix. Histochemically, the myxoid stroma contained abundant acid mucopolysaccharide. The tumor cells were considered to originate from smooth muscle cells, while the myxoid stroma expressed varying differentiation of uterine mesenchymal cells. The patient is currently well with no evidence of recurrence or metastasis one year after the operation. Acta Pathol Jpn 41: 778-783, 1991.     

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.     

Inflammatory myofibroblastic tumor of the uterus with prominent myxoid change

A surgical case of inflammatory myofibroblastic tumor arising in the uterine corpus and exhibiting prominent myxoid change of the stroma is reported. The patient was a 63-year-old woman with a large tumor mass that filled the uterine cavity and measured 11 cm in maximal dimension. The tumor had a gelatinous appearance and consisted of a loose proliferation of stellate or polygonal cells on a myxomatous background. Fascicular proliferation of spindle cells was also observed focally, and a chronic inflammatory cell infiltration was seen in many areas. Tumor cells had mild atypism and were immunoreactive for vimentin, alpha-smooth muscle actin, and anaplastic lymphoma kinase (ALK). Focal immunoreactivity for high-molecular-weight caldesmon (h-caldesmon) was also noted. The patient has been free from recurrence for 8 months. Inflammatory myofibroblastic tumor of the uterus occasionally shows prominent myxoid change of the stroma, and differentiation from myxoid leiomyosarcoma is problematic in these cases. Based on the immunoreactivity of tumor cells for ALK and h-caldesmon, the present tumor was considered inflammatory myofibroblastic tumor showing a focal phenotypic transition from myofibroblasts to smooth muscle cells.     

Fibromyxoid variant of endometrial stromal sarcoma with atypical bizarre nuclei

Endometrial stromal sarcoma (ESS) is the second most common malignant uterine mesenchymal tumor. It affects women primarily in the perimenopausal age group. ESSs are morphologically heterogeneous. The distinction between uterine smooth muscle tumors such as cellular leiomyoma and myxoid leiomyosarcoma and low-grade ESS can be problematic when stromal sarcomas show prominent smooth muscle differentiation and abundant myxoid stroma, respectively. We herein present a rare case of fibromyxoid variant of ESS, which was misdiagnosed as hydropic leiomyoma on intraoperative frozen section examination. Grossly, the uterine mass consisted of intracavitary and intramural portions. The intracavitary portion with extensive hydropic degeneration mimicked a hydropic leiomyoma. In contrast, the intramural portion displayed an obvious tongue-like myometrial invasion. Histologically, the tumor consisted of both cellular (20%) and myxoid (80%) areas. In the cellular areas, oval to spindle-shaped tumor cells with bland nuclear features were found to surround concentrically a rich vascular network of arterioles, a characteristic of ESS. In addition, two relatively well-circumscribed nodular lesions showing atypical bizarre nuclei were identified in the myxoid area. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD10. The present case indicates a wide morphological spectrum of ESS. Fibromyxoid variant of ESS should be considered in the differential diagnosis of intracavitary and/or intramural uterine mesenchymal tumors with myxoid differentiation. It is important to avoid confusion between fibromyxoid ESS and myxoid leiomyosarcoma because of the differences in their clinical course, treatment, and prognosis.     

Myxoid leiomyosarcoma of the liver

This is the first report of a myxoid leiomyosarcoma arising in a cirrhotic liver. The tumor was resected from a 64-year-old man. On gross examination, it was soft and hemorrhagic. The tumor was composed of deceptively benign-looking smooth muscle cells with clear cytoplasm suspended in a myxoid stroma with foci of hemorrhage. Immunohistochemistry and electron microscopy confirmed that this was a smooth muscle cell neoplasm. The abundance of glycogen and ultrastructural signs of smooth muscle differentiation were considered consistent with an immature smooth muscle cell phenotype consistent with the diagnosis of myxoid leiomyosarcoma. Since myxoid leiomyosarcomas are aggressive tumors, it is important to recognize them histologically and also bear in mind that these tumors can occur even in unusual extrauterine locations such as a cirrhotic liver.     

Myxoid leiomyosarcoma of the urinary bladder

A 16-year-old male patient with hematuria was found to have a 3-cm mass on the right posterolateral wall of the urinary bladder. After a biopsy specimen disclosed a myxoid tumor, a partial cystectomy was performed. The specimen contained a gelatinous tumor that, on microscopic examination, was a myxoid leiomyosarcoma similar to the neoplasm recently described in the uterus and several other locations. This tumor, which is the first of its type (to our knowledge) reported to occur in the bladder, must be distinguished from other neoplasms and nonneoplastic lesions that occur in this organ. The patient is well two years postoperatively.