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.     

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     

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。     

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。     

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,...     

血管肌纤维母细胞瘤的临床病理和鉴别诊断

目的　探讨血管肌纤维母细胞瘤 (AMF)的临床病理特征和鉴别诊断。方法　通过 4例AMF的病理形态和免疫组化研究 ,结合复习文献总结AMF的临床病理特征和鉴别诊断。结果　肿瘤境界清楚 ,直径 <5cm ,瘤细胞梭形或卵圆形 ,常围绕血管排列 ,呈疏密交替分布特征。免疫表型 :desmin、SMA、vimentin、CD34、ER和PR阳性或部分阳性 ,而MSA、S 10 0蛋白、Mac387和CK均阴性。结论　AMF是一种主要发生于生育期女性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相鉴别     

血管肌纤维母细胞瘤16例临床病理分析

目的探讨血管肌纤维母细胞瘤（angiomyofibroblastoma,AMF）的临床病理、免疫组化特征、组织来源以及鉴别诊断。方法通过16例AMF的临床表现、病理形态和免疫组化研究,并结合文献复习,总结AMF的临床病理、免疫组化特征及鉴别诊断。结果16例AMF,大体境界清楚,无包膜,可见一层假纤维膜包绕;组织学上呈疏松水肿样,丰富薄壁海绵样血管,肿瘤细胞多样性,梭形及上皮样细胞多见,常围绕血管排列呈疏密交替分布特征。免疫组化结果：瘤细胞desmin及vimentin呈弥漫强阳性表达,actin、CD34、ER和PR染色稍弱、呈灶状分布,而S-100蛋白、NF和CK均阴性。结论AMF临床上多无明显症状;发病部位以女性外阴及盆腔最多见,但全身多处部位也可见;其生物学行为及组织来源尚无定论;术前很难确定诊断AMF,需与侵袭性血管黏液瘤、富于细胞性血管纤维瘤等相鉴别。     

胚胎发育不良性神经上皮瘤病理形态学观察

目的 探讨胚胎发育不良性神经上皮瘤(DNT)临床病理特点、诊断及与低级别胶质细胞瘤鉴别诊断。方法 对2例DNT和5例少枝细胞瘤进行光镜、免疫组化和特殊染色观察。结果 2种肿瘤组织形态类似，主要由类似分化好少枝细胞瘤样细胞构成。与少枝细胞瘤不同，DNT的组织学特点是瘤组织黏液变明显，多见网状微囊变。瘤组织间散在分布分化好的神经细胞，多位于黏液丰富的微囊内，如同浮蛙。分化的神经细胞及部分小细胞Syn阳性。肿瘤邻近的大脑皮质神经细胞层次有不同程度紊乱。结论隔DNT与低级别少枝胶质细胞瘤不易鉴别，DNT预后良好，只需手术切除即可，不需辅以有潜在危害的化疗或放疗。因此，两者的鉴别诊断具有重要的临床意义。     

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。     

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。     

Superficial angiomyxoma: report of four cases, including two subungueal tumors

We report four cases of superficial angiomyxomas, including two cutaneous tumors and two subungueal tumors. Histological analysis revealed a recently described tumor, so called superficial angiomyxoma. This is a myxoid paucicellular tumor lobulated and poorly circumbscribed, containing numerous small blood vessels surrounded by a mixed inflammatory cell infiltrate with notable neutrophils. Those tumors are positive for CD34. The differential diagnosis includes myxoid neurothecoma, myxoid neurofibroma and, for ungueal tumors, superficial acral fibromyxoma.     

Angiomyxoma: always myxoid, sometimes aggressive

Angiomyxoma is a distinct soft tissue tumor characterized by the presence of prominent myxoid matrix and numerous thin-walled blood vessels. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. It is a benign tumor and total excision is curative. Recurrence is rare except for aggressive angiomyxomas. A 12-year-old girl with a 10-year history of a subcutaneous mass on the left gluteus measuring 4.5x4x3 cm had been referred. The tumor was encapsulated and was located in the reticular dermis and subcutaneous tissue, composed of stellate cells with mucinous stroma. Thin-walled blood vessels were prominent. Immunohistochemically, tumor cells were immunoreactive for vimentin. No immunoreactivity was present for estrogen receptor, CD34, smooth muscle actin, S-100 protein and desmin. The purpose of this report is to present a classical example of an isolated superficial angiomyxoma and discuss the differential diagnosis, because of its relatively infrequent occurence.     

Myxoid dermatofibroma on a great toe: a case report

Dermatofibroma is a common benign fibrohistiocytic tumor with many clinicopathological variants. Myxoid dermatofibroma is one of these variants, which is characterized by marked stromal mucin deposition. This report presents a case of myxoid dermatofibroma on a great toe that had been slowly growing for two years. Histopathologically, the relatively well-circumscribed dermal tumor was separated from the epidermis by a small grenz zone. The tumor tissue consisted of oval to spindle-shaped cells with well-defined cell borders and spindly condensed nuclei. No cytologic atypia or mitotic figures were found. Although most of the tumor cells were embedded in a prominently myxoid stroma, typical features of classic dermatofibroma including a storiform growth pattern and more densely packed collagen were observed at the periphery. Immunohistochemically, the tumor cells showed positive staining for CD68 and CD99, and negative staining for CD34 and S-100. Histopathological differential diagnoses of myxoid dermatofibroma include soft tissue neoplasms with myxoid tumor stroma, such as superficial acral fibromyxoma, cellular digital fibroma, superficial angiomyxoma, myxoid dermatofibrosarcoma protuberans and low-grade fibromyxoid sarcoma. Immunohistochemical staining can be useful in the differential diagnosis of these tumors. This case highlights the challenges encountered in the histopathological interpretation of myxoid dermatofibroma. Pathologists should keep in mind the diagnosis of myxoid dermatofibroma when dealing with myxoid neoplastic lesions arising on acral sites.     

Superficial angiomyxoma with trichofolliculoma

We report a case of a superficial angiomyxoma with a trichofolliculoma, found on the trunk of a 51-year-old man. The tumor, measuring 4 cm in maximum diameter and located in the reticular dermis and subcutaneous tissue, was composed of a conglomerate of multiple myxomatous nodules with mucinous stroma and stellate cells. Thin-walled blood vessels were prominent. The histologic examination also revealed abortive hair follicles radiating from the wall of a keratin-filled cyst. Immunohistologically, the stromal cells were positive for vimentin but negative for S-100, smooth muscle a-actin, and desmin. With these findings, the diagnosis of superficial angiomyxoma with trichofolliculoma was made.     

血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错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。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。     

Myxoid leiomyoma of the vulva mimicking aggressive angiomyxoma

A case of vulvar leiomyoma with extensive myxoid change in a 40 year old female is described. The tumor had a unique connection with a non-degenerative leiomyoma that compressed the rectum and the bladder. Scattered smooth muscle cells in a loose myxoid stroma were immunoreactive for desmin. Fibroblast-like spindle cells were immunoreactive for vimentin but not for desmin. The initial, although incorrect, pathological diagnosis of the tumor was aggressive angiomyxoma based on the similarity in both clinical and pathological aspects with this more invasive tumor. Myxoid vulvar leiomyoma should also be differentiated from angio-myoflbroblastoma. The key to the differential diagnosis is the presence of interlacing smooth muscle cells and an awareness of tendency toward myxoid change in vulvar leiomyomas.     

Fine-needle aspiration cytology of superficial angiomyxoma (myxoid perifollicular fibroma): Report of a case

A case of superficial angiomyxoma arising in the areola of a 49-yr-old woman is presented, including both fine-needle aspiration cytology (FNAC) and histological study. The aspirate yielded a rich myxoid background with isolated fusiform cells, showing oval, monomorphic nuclei with minimal atypia. Sometimes these cells were arranged in small fragments without any specific pattern. The absence of mitotic figures, atypia, or cutaneous and breast epithelium suggested a benign myxoid stromal neoplasm. The final diagnosis was established on the excised mass by histopathologic study. The report of this entity in FNAC and the differential diagnosis with other myxoid lesions are discussed, and the feasibility of its cytological diagnosis by FNAC, helped by appropriate clinical information. © 1995 Wiley-Liss, Inc.     

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.