浅表性血管黏液瘤9例临床病理分析

目的 探讨浅表性血管黏液瘤(superficial angiomyx-oma,SA)的临床病理学特征.方法 回顾性分析9例SA的临床病理学特征、免疫表型、诊断及鉴别诊断、治疗及预后等,并复习相关文献.结果 9例患者年龄20～79岁,平均44岁,4例肿瘤位于外阴,位于腋窝、足背、头皮、颈部、眼睑各1例,随访2～59个月,...     

颈部浅表性血管黏液瘤一例

患者男,26岁。2年前发现左侧颈部有一包块并逐渐增大,于2005年11月25日入院。体检:患者健康状况良好。左侧颈部皮下软组织内触及一卵圆形肿物,约2．5 cm×1．5 cm,表面光滑,无压痛及波动感,与周围软组织无粘连,活动度好,皮肤无红肿及破溃。全身检查及实验室检查均正常。行肿物切除术,术中见:肿物位于皮下,呈椭圆形,表面光滑,质软,完整摘除肿物送病理检查。     

外阴浅表性血管黏液瘤2例

例1,42岁,发现左侧外阴部包块5年,进行性增大,有压痛。查体:左侧外阴可触及皮下包块,大小3.0 cm×3.0cm×2.5 cm,表面皮肤无红肿及破溃,与周围组织边界清。例2,26岁,左侧外阴部包块2年,逐渐增大,无明显不适。查体:左侧外阴皮下包块,大小0.5 cm×0.5 cm×0.5 cm,表面光滑,略呈分叶状,与周围组织边界较清。病理检查眼观:例1,带皮肿物1个,大小2.5 cm×2.0 cm×1.5 cm,肿物位于真皮及皮下,略呈分叶状,无包膜,边界较清,切面灰白色,实性,有黏液感,质中,基底部未     

侵袭性血管黏液瘤7例临床病理分析

目的探讨侵袭性血管黏液瘤(aggressive anginomyxoma,AAM)的临床病理特征与鉴别诊断。方法收集7例AAM,并与2例血管肌纤维母细胞瘤(AMFb)、1例浅表性血管黏液瘤作对照观察。免疫组织化学(采用SP法)所用抗体为vimentin、desmin、SMA、MSA、CD34、ER/PR、S-100蛋白、CK和CD68。结果7例AAM患者均为女性,23~54岁,中位年龄43·5岁。病变部位位于外阴部3例,累及阴道1例,位于盆腔及髂窝部2例,会阴累及坐骨结节1例。肿瘤直径3·5~17cm,平均7·5cm。位于皮下或较深部,呈浸润性生长,切面均质灰白或灰黄褐色,黏液胶冻样或肉质样。镜下瘤细胞幼稚,呈星形、梭形,弥漫或结节样分布,基质黏液样变性并见胶原纤维及为数众多的大小厚薄不一的血管分布其中,厚壁血管常透明变性,其周围常有增生肌纤维束,切面呈“袖套状”,间质可见肥大细胞和红细胞外渗,1例可见多核巨细胞,肿瘤边缘浸润等特点。组织化学奥辛蓝染色阳性,免疫组化:7例AAM vimentin均阳性,5例SMA和desmin阳性,4例MSA阳性,3例CD34阳性,4例中有3例ER/PR阳性,而S-100蛋白、CK、CD68均阴性,多核巨细胞CD68阳性。5例有随访,术后1~2年有3例复发,2例未见复发和转移,健在。结论侵袭性血管黏液瘤较为少见,好发于成年女性盆腔及会阴的软组织,侵袭性和复发性是其重要的临床特征。病理诊断易误诊或漏诊。免疫组化desmin、SMA、MSA、CD34等联合检测对确定AAM病变有所帮助,但与AMFb、浅表性血管黏液瘤在鉴别诊断上意义不大。     

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。     

鼓室血管球瘤2例临床病理特征分析

鼓室血管球瘤(glomus tumors,GT)属于鼓室部非常罕见的肿瘤,本文主要探讨本单位2例鼓室GT的临床、病理学特点,诊断及鉴别诊断并复习相关文献,以提高对其的认识和诊断水平,减少误诊.     

胃血管球瘤6例临床病理分析

目的 探讨胃血管球瘤(gastric glomus tumor,GGT)的临床病理学特征、诊断及鉴别诊断.方法 收集6例GGT临床资料,采用HE、免疫组化EnVision两步法染色,分析其临床病理学特征、免疫表型、预后等,并复习相关文献.结果 6例GGT男女性各3例,年龄34～60岁,中位年龄53.5岁,平均49.33...     

黏液纤维肉瘤7例临床病理分析

目的 探讨黏液纤维肉瘤临床病理特点。方法收集7例黏液纤维肉瘤患者的临床及病理资料,并对手术切除的标本进行多种抗体免疫组织化学染色,抗体包括CD117、CD34、CD99、actin、S-100蛋白、vimentin等。结果黏液纤维肉瘤多见于男性,年龄37～72岁,中位年龄52岁,主要以不完全纤维性间隔的多结节行为为特征性表现,黏液区与细胞密集区交错排列,细胞密集区可见特征性曲线型血管,肿瘤细胞围绕在其周围排列。免疫表型：vimentin、CD99阳性,其他抗体多呈阴性。结论黏液纤维肉瘤为一侵袭性较强的软组织肉瘤,与多种黏液性肿瘤需要鉴别,易局部复发,宜采取以手术切除为主的综合性治疗。     

指趾纤维黏液瘤3例临床病理分析

目的探讨指趾纤维黏液瘤(digital fibromyxoma, DF)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析3例DF的临床资料、病理学形态及免疫表型特征,并复习相关文献。结果 3例DF中男性2例,女性1例,年龄30～57岁,平均44岁,且病变均发生于手指。肿瘤最大径1.5～2 cm。镜下见肿瘤细胞呈梭形和星形纤维母细胞样,束状分布于黏液样基质内;黏液样基质富含纤细的血管,瘤细胞异型性不明显或仅轻度异型,核分裂象罕见。免疫表型:梭形和星形细胞表达CD34、CD10和CD99,灶性表达EMA,不表达MUC4、desmin和S-100蛋白,Ki-67增殖指数1%～2%。3例患者手术切除后均痊愈。结论 DF属少见的良性肿瘤,为防止局部复发,临床上应行肿瘤完整切除术。     

树突状纤维黏液脂肪瘤6例临床病理分析

目的 探讨树突状纤维黏液脂肪瘤(dendritic fibromyxolipoma, DFML)的临床病理学特征。方法 回顾性分析6例DFML的临床病理学特征、免疫表型、诊断及鉴别诊断等,并复习相关文献。结果 6例中男性4例,女性2例,年龄6～57岁,平均40岁;肿瘤直径1～7.5 cm,平均3.3 cm;肿瘤发生于腋窝、腿等体表部位。肿块多为类圆形,切面灰白、灰黄色,胶冻或黏液样。镜下形态学大致相同,均表现为在黏液样间质及绳索样(瘢痕样)胶原纤维背景中见混杂不同比例的小梭形细胞、星芒状细胞或成熟脂肪细胞。免疫表型：梭形细胞和星芒状细胞vimentin、CD34均强阳性,BCL-2弥漫/灶阳性,部分成熟的脂肪细胞S-100阳性,散在的肥大细胞CD117阳性,CK、desmin、SMA、HMB-45、MDM2、CDK4、p16、EMA、β-catenin、STAT6、CD56、NSE均阴性。Ki-67增殖指数均<1%。结论 DFML是一种良性脂肪瘤,是梭形细胞脂肪瘤的黏液样亚型,病理检查结合免疫表型有助于确诊,患者肿块切除后多可治愈。     

Superficial angiomyxoma: a c linicopathologic analysis of three cases

目的 探讨浅表性血管黏液瘤(superficial angiomyxoma,SA)的临床病理特征、免疫组化及鉴别诊断要点.方法 对3例SA进行临床病理学及免疫组化染色观察,结合文献对该肿瘤的临床表现、病理形态学特征以及鉴别诊断要点进行讨论.结果 SA主要位于皮肤真皮及皮下,镜下肿瘤略呈分叶状,黏液性基质中可见散在无异型的梭形和星形瘤细胞及较多的中、小薄壁血管,散在有多少不等的中性粒细胞浸润.免疫组化染色显示肿瘤细胞表达vimentin.结论 SA是罕见的黏液性肿瘤,位于皮肤表浅部位,可局部复发但不转移,诊断时应注意和其它黏液性软组织肿瘤鉴别.     

血管肌纤维母细胞瘤与侵袭性血管粘液瘤临床病理分析

目的：探讨血管肌纤维母细胞瘤（ＡＭＦＢ）的临床病理特点及与侵袭性血管粘液瘤（ＡＡ）的鉴别。方法：对５例ＡＭＦＢ和５例ＡＡ进行临床病理和免疫组化研究,对３例ＡＭＦＢ进行电镜观察。结果：ＡＭＦＢ位于外阴或腹股沟我,肿瘤边界清楚,大小０．８～４ｃｍ。光镜：肿瘤细胞呈梭形上皮样、束头及巢状排列,常围绕小至中等大小的薄壁血管周围。肿瘤有细胞密集区和细胞分散区。免疫组化：肿瘤细胞表达ｖｉｍｅｎｔｉｎ,ｄｅｓｍ     

胚胎发育不良性神经上皮瘤9例临床病理分析

目的 增进对胚胎发育不良性神经上皮瘤(dysemhryoplastic neroepithelial tumors，DNT)临床病理特征的认识。方法 复习9例DNT的临床表现、放射学、病理形态学及免疫组化特征。结果 9例均为20岁以前发病，以癫痫小发作为主要表现，肿瘤均位于大脑皮层内，影像学均无占位效应和瘤周水肿。组织病理学上，皮层内“特异性胶质神经元成分”呈结节状分布，例1、4～9为单纯型，例2、3为复合型。随访：例1术后5年无复发，例3术前2年病灶无变化，另7例仍在随访中。结论 DNT是以20岁前起病、难治性癫痫小发作、由“特异性胶质神经元成分”构成皮层发育不良灶为特征的良性神经上皮肿瘤，可被CT或MRI发现而确诊，绝大多数病例手术切除不复发，预后良好。复合型中极少数病例的胶质瘤成分有复发和恶变的可能。     

乳头状汗腺瘤9例临床病理分析

目的研究乳头状汗腺瘤的临床病理特点和免疫学表型。方法收集三峡大学第一临床医学院病理科1995～2009年存档和诊断的9例乳头状汗腺瘤的临床及病理资料并对切除的标本进行免疫组织化学染色,抗体包括ER、PR、CK(AE1/AE3)、CK5/6、CK7、CK20、CK(Low MW)、CK34βE12、EMA、p63、actin、CD10、S-100、Calponin、GCDFP-15和vimentin。结果9例患者平均发病年龄48岁,男女比例1∶8,主要发生于外阴及肛周。临床表现为单发的直径0.5～1.2 cm大小的半球形、孤立的、界限清楚的皮下结节;组织病理可见肿瘤由大小不一的腺泡、腺管和复杂折叠的乳头状结构组成,乳头的轴心为纤维血管。腺腔和乳头结构内衬单层柱状上皮细胞,可见顶浆分泌;在柱状细胞外周见梭形或立方形的肌上皮细胞,肌上皮与内层柱状细胞形成双层排列。免疫组化检测显示:9例肿瘤的内层柱状细胞均表达ER、PR、CK(AE1/AE3)、CK(Low MW)、CK7和EMA,7例表达CK34βE12,所有肿瘤的肌上皮细胞均表达p63、actin、CD10、S-100和Calponin,7例肿瘤的内层柱状细胞与肌上皮同时表达CK5/6,所有的肿瘤细胞均不表达CK20、GCDFP-15和vimentin,但纤维间质均表达vimentin。结论乳头状汗腺瘤是一种少见的皮肤良性肿瘤,临床表现多样,极易误诊。确诊依赖于组织病理检查。完整切除肿物后不复发。     

Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study

To investigate the histogenetical unifying theory of a single, pluripotential primitive cell for vulvar angiomyxoma, aggresive angiomyxoma, and angiomyofibroblastoma, an optical, immunohistochemical and ultrastructural study of a superficial angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma was performed. These three tumors showed immunohistochemical and ultrastructural overlapping features. The results of the study suggest that these three tumor entities probably arise on a common pluripotential primitive cell located around the vessels of connective tissue, which could show the capacity for modulating its penotype toward similar but distinct mature cell types.     

原发性性腺外卵黄囊瘤40例临床病理分析

目的：探讨原发性性腺外卵黄囊瘤( extragonadal yolk sac tumor, eYST)的临床病理特征、组织来源、形态学特点、免疫表型及鉴别诊断。方法回顾性分析40例原发性eYST的临床病理资料、形态学和免疫表型特征,结合文献对其临床病理特点进行分析。结果40例eYST中男性24例、女性16例,年龄6个月~42岁,平均12岁,≥12岁者17例,占42.5%。肿瘤分别位于纵隔16例(40.0%)、骶尾12例(30.0%)、腹膜后5例(12.5%)、松果体4例(10.0%)、阴道3例(7.5%)。40例患者中32例为纯YST(80.0%),8例(20.0%)含有1~2种其他类型的生殖细胞肿瘤(germ cell tumor, GCT)成分。结论原发性eYST少见,纵隔和骶尾是eYST最常见的解剖部位;发生在纵隔的肿瘤患者大部分限于成年男性,患者平均年龄明显大于骶尾、腹膜后、松果体和阴道肿瘤的患者(P<0.05),发生在其他部位的eYST多限于青春期前的儿童;一些成人eYST的病例包含其他类型的GCT成分,儿童eYST总是为纯YST;eYST表现出多形性的组织学特征,结合免疫表型对明确诊断、鉴别诊断有一定价值。     

Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 11 cases with long-term follow-up

Aims To report the clinicopathological and immunohistochemical features and longer term biological behaviour of aggressive angiomyxoma, an uncommon mesenchymal neoplasm occurring predominantly in the pelvi-perineal region of adults. Using immunohistochemistry, possible overexpression of CDK4 and MDM2 was analysed, which might point to (cyto)genetic alteration(s) in chromosome region 12q13–15, an area reported to be altered in this tumour entity.Methods and results Cases (n=11) of aggressive angiomyxoma were retrieved from the consultation files of the Comprehensive Cancer Centre of the Middle Netherlands (IKMN) panel for soft tissue tumours. Clinical and follow-up information were obtained, and immunohistochemical analysis was performed using antibodies directed against vimentin, cytokeratin AE1/AE3, desmin, -smooth-muscle actin, CD34, S-100 protein, oestrogen receptors, CDK4 and MDM2. Five patients were female (age range 24–47 years; median 39 years), and six patients were male (age range 36–69 years; median 44.5 years). Of 11 cases, 10 arose in the pelvi-perineal area and 1 arose in the abdominal cavity in close relation to the bladder. Morphology was consistent with previous reports of this entity. Immunohistochemically, 8 of 11 cases were desmin positive (5 of 5 positive in females; 3 of 6 positive in males), 6 of 11 cases were positive for -smooth-muscle actin, 5 of 11 cases were CD34 positive, 11 of 11 cases, irrespective of gender, were positive for oestrogen receptors and 3 of 11 cases were positive for cytokeratin AE1/AE3. Strong, diffuse nuclear positivity for CDK4 expression was present in all 6 cases tested, while only 1 of 11 cases tested for MDM2 showed weak focal positivity. Clinical follow-up in all cases (range 1–216 months; median 72 months) showed one local recurrence (9%) after 36 months. No metastases or tumour-related deaths were noted.Conclusions The sex distribution of cases reported in this study was roughly equal, in contrast to previous reports emphasising the predominance of this tumour in females. Our study confirms the local aggressive nature of aggressive angiomyxoma, although our local recurrence rate is lower than previous reports (9% versus 36–72%); no metastases and/or disease-related patient deaths were documented. All cases arising in females were positive for desmin, while three of the six cases arising in males were negative for desmin, supporting previous findings and indicating that the lesion may be somewhat different in males. The strong diffuse positivity for CDK4 in all six cases tested goes some way in implicating CDK4, either directly or indirectly, in tumourigenesis. The negative immunostaining for MDM2 would argue against functional amplification of this gene.     

Aggressive angiomyxoma: a second case of metastasis with patient's death

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient. We document this case and report a similar one, found in the literature, of a postmenopausal woman with pulmonary and mediastinic metastases. These cases may expand the current concepts of potential behavior of aggressive angiomyxoma.     

Aggressive angiomyxoma of the spermatic cord. Two unusual cases occurring in childhood

We report on two cases of aggressive angiomyxoma (AAM) of the spermatic cord occurring in two 13-year-old children. Clinically, the tumor simulated a mass of the spermatic cord. Histologically, it represented a poorly circumscribed, benign myxoid tumor, with a sparse population of stromal cells immunoreactive for vimentin and, focally, for smooth muscle actin. No immunostaining for desmin, S-100, p53, p21waf-1, c-Erb-B2 and estrogen-progesterone receptors was found. High proliferating cell nuclear antigen (PCNA) immunoexpression found in most of the tumor cells may explain the high risk of recurrence. AAM should be considered in the differential diagnosis of a spermatic cord mass occurring during infancy.     

Superficial Acral Fibromyxoma: Immunohistochemical and Ultrastructural Analysis of a Case,with Literature Review

ABSTRACT

Superficial acral fibromyxoma (SAFM) is an uncommon tumor of the superficial soft tissues of acral sites. SAFM is a proliferation of fibroblastic cells, within a myxoid to collagenous stroma. The published cases mostly expressed immunoreactivity for CD34, CD99, EMA, and, less frequently, CD10. The authors report an additional case that did not express any of the previously reported markers, including CD34, and antigens of mesenchymal stromal lineage. Ultrastructural study confirmed the tumor cells were typical fibroblasts with cytoplasmic intermediate filaments and numerous cisternae of rough endoplasmic reticulum. The authors describe the first example of SAFM, ultrastructurally studied, with pure fibroblastic immunoprofile.