血管肌纤维母细胞瘤临床病理学观察

目的 探讨血管肌纤维母细胞瘤( angiomyfibroblastoma, AMF )的临床病理特点及其诊断与鉴别诊断.方法 通过对7例AMF的临床表现、组织学观察与免疫组化研究,并复习相关文献总结AMF的临床病理特征及鉴别诊断.结果 7例均为女性,年龄25～52岁,部位在大阴唇(3例)、宫颈管(3例)和腹股沟(1例),大小1.6～6.0 cm;眼观:肿瘤边界清,无包膜,质地韧,切面灰白,局部有黏液感.镜检:肿瘤细胞分布稀疏不一,呈密集的血管区与少细胞的水肿区相交替分布.瘤细胞肥胖,呈梭形或短梭形,可见胞质突起或波浪状弯曲,瘤细胞围绕丰富的血管,大多是薄壁小血管或毛细血管,间质可见胶原束.免疫表型:瘤细胞vimentin和desmin弥漫阳性,α-SMA和CD34局灶阳性,发生于宫颈的3例ER和PR弱阳性,所有病例CK、S-100蛋白阴性.结论 AMF是发生女性外阴、生殖道的良性软组织肿瘤;需要与侵袭性血管黏液瘤、富于细胞性的血管纤维瘤、青春期前外阴纤维瘤、浅表宫颈阴道肌纤维母细胞瘤等软组织肿瘤相鉴别.     

血管肌纤维母细胞瘤临床病理特征

目的：探讨血管肌纤维母细胞瘤的临床病理形态特征及鉴别诊断。方法：对2例血管肌纤维母细胞进行组织病理学、免疫组织化学研究,结合文献资料分析本病的临床表现、病理形态特点及鉴别诊断。结果：血管肌纤维母细胞瘤呈大片黏液背景、丰富薄壁海绵样血管和梭形上皮样细胞即肌纤维母细胞,后者胞质丰富,呈嗜酸性,核卵圆或杆状,两端对称变细或稍钝,瘤细胞分布有明显的疏密区,密集区聚于血管周围,成束或链状排列、疏松区弥散于黏液背景中,且瘤细胞常与胶原纤维伴行。结论：血管肌纤维母细胞瘤为良性肿瘤,好发于女性外生殖器,起源与肌纤维母细胞相关,既向纤维,又向平滑肌分化,且伴有丰富的海绵样薄壁血压和间质黏液变性。     

胃炎性肌纤维母细胞瘤临床病理分析

目的:探讨胃炎性肌纤维母细胞瘤(gastric inflammatory myofibroblastic tumor,GIMT)的临床病理特征、诊断与鉴别诊断.方法:对2例GIMT进行临床病理分析及免疫组织化学研究,并复习相关文献.结果:例1患者为女性,72岁,临床表现为上腹不适,胃镜见靠近胃体前壁息肉状肿块突向胃腔,行胃次全切除术及毕罗Ⅰ式重建.例2,男性,43岁,主诉左上腹痛,CT扫描显示胃-食管交界区黏膜下广基或透壁性新生物.两例患者临床分别诊断为胃肠间质瘤及胃癌,而术后病理却证实为GIMT.大体所见:灰白色结节状肿物边界不清,质地不均,表面被覆完整黏膜.镜下显示肿瘤由密集的梭形细胞和大量的慢性炎细胞组成.这些梭形细胞呈束状或席纹状排列,胞质空泛或淡嗜酸性,核呈卵圆形,具有模糊的核仁,核分裂象难以见到;而浸润于黏液样基质中的炎性细胞主要由淋巴细胞、浆细胞组成,部分区域形成淋巴滤泡,类似于炎性或反应性病变.免疫组织化学显示肿瘤细胞弥漫表达vimentin和h-caldesmon,部分表达间变性淋巴瘤激酶(anaplastic lymphoma kinease,ALK),不表达CD34,CD117及desmin.两例经随访4～6个月均未见肿瘤复发及转移.结论:GIMT是一种罕见的具有独特病理改变的纤维性病变,易与胃肠间质瘤、平滑肌瘤等混淆.     

外阴血管肌纤维母细胞瘤

患者女 ,38岁。发现外阴肿物 1年多 ,近半年肿物明显增大。临床考虑“巴氏腺囊肿” ,术中见肿物位于外阴左侧大、小阴唇间中下 1/ 3部位 ,与周围有界限 ,行单纯肿物切除。病理检查 :椭圆形肿物 4 0ｃｍ× 3 0ｃｍ× 1 8ｃｍ ,表面略粗糙 ,切面灰白色 ,实性 ,质较韧 ,未见纤维走行。镜下观察 :肿瘤有清楚边界 ,瘤细胞肥胖呈短梭形、锥形或上皮样 ,胞质中量 ,淡嗜酸或嗜双色 ,有单极或双极胞质突起 ,一些细胞呈波状弯曲 ,数个细胞聚集时细胞界限不清。细胞核圆形或卵圆形 ,偶有双核或多核 ,染色质细 ,核仁小 ,无明显细胞异形 ,未见核分裂。瘤…     

肾上腺肌纤维母细胞瘤

目的：观察肌纤维母细胞瘤形态及免疫组化特点。方法：对１例儿童肾上腺的肌纤维母细胞瘤进行光镜观察和免疫组化染色。结果：ＨＥ中瘤细胞呈梭形、不规则形，排列无序，间杂以淋巴细胞等浸润及胶原纤维束；免疫组化显示瘤细胞Ｖｉｍ（＋），ＳＭＡ＋（＋），ＣｇＡ（－），ＥＭＡ（－）。手术彻底切除肿瘤后，患者临床症状消失。结论：肌纤维母细胞瘤是由既具有平滑肌细胞特征，又具有纤维母细胞特征的独立性肿瘤。     

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。     

阴囊血管肌纤维母细胞瘤一例

患者男 ,6 0岁。因发现阴囊右侧包块 5年于 1999年 11月入院。体检 :阴囊右侧有一 5ｃｍ× 5ｃｍ× 4ｃｍ大小肿块 ,界清。ＣＴ示 :阴囊内偏后方的类椭圆形软组织肿块 ,边界光整、清楚。双侧睾丸未见异常。手术所见 :阴囊内右睾丸外下方有一肿块 ,包膜完整 ,双侧睾丸正常。病理检查 :送检灰褐色卵圆形肿块一个 ,5ｃｍ× 5ｃｍ×3ｃｍ ,包膜完整 ,表面光滑 ;切面呈灰褐色 ,均质状 ,有光泽 ,质地软。镜下观察 :肿瘤由围绕血管分布的短梭形、卵圆形、不规则形细胞及散在分布的中等大小薄壁血管组成 (图 1)。肿瘤细胞胞质较丰富 ,略呈嗜酸性 ,细…     

腹腔脏器炎性肌纤维母细胞瘤的临床病理观察

目的探讨腹腔脏器炎性肌纤维母细胞瘤(inflammatorymyofibroblastictumor,IMT)的临床病理特征、组织发生和预后。方法分析10例IMT的临床病理学资料,所有病例行HE染色和免疫组化染色。结果腹腔脏器IMT的组织学分为三型:(1)黏液样-血管型,(2)梭型细胞密集型,(3)少细胞纤维瘢痕型。肿瘤细胞表达vimentin、SMA、MSA、CKpan和ALK,其阳性率分别为100%、90%、90%、60%和20%。10例IMT中有5例做了根治性切除术,另有5例单纯肿瘤切除,随访无1例复发。结论腹腔脏器IMT易被临床医师误诊为晚期癌。免疫组化表型是该肿瘤与其他软组织肿瘤鉴别的重要依据。腹腔脏器IMT一般具有良性病变的生物学行为,根治性手术可能治愈。     

肌上皮瘤与肌纤维母细胞瘤

肌上皮细胞与肌纤维母细胞都是具有双重形态与功能特性的细胞，不仅具有上皮细胞、纤维母细胞的特征，还具有肌细胞的部分特点。人们早已发现这两种细胞广泛存在于人体各种组织并参与许多重要的病理过程〔１〕。近年来由这两种细胞构成的真性肿瘤也越来越引起人们的兴趣和...     

上颌窦炎性肌纤维母细胞瘤诊断与治疗

目的 探讨上颌窦炎性肌纤维母细胞瘤(IMT)的临床特征与诊疗方法。方法 回顾性分析2010年1月—2014年1月安徽医科大学第一附属医院耳鼻咽喉头颈外科收治的6例上颌窦IMT患者的临床资料,并结合文献学习,探讨其诊断与治疗方法。6例均行鼻窦CT检查,其中1例行鼻窦MRI检查;影像学检查发现病灶中心均位于上颌窦,其中男4例,女2例;年龄38~81岁,中位年龄58.5岁;均表现为鼻塞、头痛、鼻出血等非特异性临床症状与体征;均接受手术治疗,术式为局部扩大切除。结果 6例患者术后随访8~47个月。 其中1例术后18个月复发,并恶变为肌纤维母细胞肉瘤,目前带瘤生存;余5例随期间行常规体格检查及内镜检查,未见复发。本组病例CT及MRI检查均显示肿瘤为软组织来源,其中5例伴有不同程度骨质破坏,3例伴周围肌组织受侵。术后病理显示瘤细胞主要由梭形纤维细胞及炎性细胞组成,免疫组织化学染色肌源性蛋白阳性表达。结论 上颌窦IMT较罕见,并缺乏特异性临床表现;影像学检查能提供肿瘤的范围信息,在定性诊断方面有一定的价值,确诊主要依靠病理及免疫组织化学检查。其治疗以手术切除病灶为主,避免大范围损伤性手术;术后据个体情况可选择包括激素治疗或放化疗等综合治疗措施,整体预后较好。     

Aspiration cytology of the collagenized variant of mammary myofibroblastoma: a case report with review of the literature

Myofibroblastoma of the breast is a rare benign stromal neoplasm, which occurs primarily in men. Classical myofibroblastoma is a circumscribed, nonencapsulated tumor comprised of bipolar fusiform cells arranged randomly, or in fascicles alternating with broad collagenous bands. Additional histologic variants (the cellular, collagenized, infiltrative, and epitheloid types) have been described. Several case reports describe the cytopathologic features of the classical and cellular variants. We report on a 70-yr-old woman, who presented with a circumscribed mass in her left breast. Aspiration biopsy showed paucicellular smears with singly distributed atypical spindle-shaped cells and rare fragments of collagenized stroma, raising suspicion of a phyllodes tumor. Histologic examination revealed spindle-shaped cells distributed in a diffusely collagenized stroma. Some nuclear atypia was present. To the best of our knowledge, this is the first case reporting the cytologic features of the collagenized variant of myofibroblastoma. Although we believe a specific diagnosis of myofibroblastoma can be rendered in a male based on the typical cytologic and clinical findings in the classical type, the variant forms are difficult to classify accurately and require excision for a definitive diagnosis.     

Intranodal palisaded myofibroblastoma – a rare case report and literature review

Intranodal palisaded myofibroblastoma (IPM) is a benign mesenchymal neoplasm originating from smooth muscle cells and myofibroblasts. The inguinal region is the most common site of this rare tumor. As there are only about 89 such cases reported in the literature, the precise etiology and pathogenesis have yet to be explained adequately. It is characterized by spindle cells, amianthoid fibers, and by the proliferation of hemosiderin‐containing histiocytes in the lymph node. A nodular lesion was excised from the inguinal region of a 47‐year‐old female patient with the clinical diagnosis of lymphoma and/or metastase. Macroscopic examination of a section of the lesion demonstrated a solid appearance. Microscopic examination revealed spindle‐cell proliferation, amianthoid fibers, hemosiderin pigment, and extravasated erythrocytes. Nuclei of the spindle cells displayed a palisaded appearance. Compressed lymphoid tissue was observed around the lesion. Neoplastic cells were identified by the presence of vimentin, SMA, Cyclin D1, and beta‐catenin. The Ki67 index was less than 1%. Histological examination confirmed the diagnosis of IPM. Although IPM is benign, it is frequently confused with metastatic lesions and lymphomas.     

浅表肢端纤维黏液瘤的临床病理特征

目的 探讨浅表肢端纤维黏液瘤(SAF)的临床病理学特点、免疫表型和鉴别诊断.方法 对1例发生于左手中指末端SAF的临床表现、组织形态和免疫学表型进行回顾性分析,并复习文献.结果 患者男,62岁.因左手中指背侧末端肿块伴疼痛就诊,曾有外伤史.术中见肿块近甲床,并深达骨膜.大体观察,肿块周界不清,直径约2 cm,切面呈灰白色,实性,质韧.镜下观察,肿瘤位于真皮层内,略呈分叶状.瘤细胞由梭形至星形纤维母细胞样细胞组成,呈杂乱状分布于黏液样基质内,局部区域可呈条束状或疏松的席纹状排列.黏液样基质内含有丰富的纤细血管,并可见较多散在的肥大细胞.瘤细胞异型性不明显或仅显示轻度的异型性,核分裂象罕见.肿瘤内也未见坏死.免疫组织化学标记显示,梭形和星形细胞表达波形蛋白、CD34和CD99,灶性表达CD10,不表达上皮细胞膜抗原、肌动蛋白、结蛋白和S-100蛋白.结论 SAF好发于成年人指趾末端.熟悉其临床病理特点则有助于与其他发生于指趾的软组织黏液性肿瘤相鉴别.临床上宜将SAF作完整性切除,以预防局部复发.     

栅状肌纤维母细胞瘤3例报道及文献复习

目的：观察栅状肌纤维母细胞瘤的临床病理特征，探索该肿瘤的组织来源及石棉样纤维的性质和来源。方法：对３ 例栅状肌纤维母细胞瘤进行组织形态学和免疫组织化学研究，结合文献对本病的诊断标准、鉴别要点及组织来源进行探讨。结果：栅状肌纤维母细胞瘤的组织学特点为梭形肿瘤细胞呈交叉束状、栅栏状排列，间质内有较多出血、散在的肥大细胞，肿瘤中出现石棉样纤维。免疫组化见肿瘤细胞及石棉样纤维星芒状突起中ａｃｔｉｎ、ｖｉｍｅｎｔｉｎ 呈阳性表达，ｄｅｓｍｉｎ、Ｓ１００ 、ＦａｃｔｏｒⅧ、ｃｙｔｏｋｅｒａｔｉｎ 呈阴性表达。结论：栅状肌纤维母细胞瘤是一种具有独特临床病理特征的肌纤维母细胞瘤，该肿瘤来源于肌纤维母细胞或特殊平滑肌细胞亚型。石棉样纤维中心为血管周围胶原的变性，星芒状纤维为增殖的肌纤维母细胞突出的中间丝和微丝。     

乳腺癌肉瘤1例报道及文献复习

目的探讨乳腺癌肉瘤的临床病理学特征及其鉴别诊断。方法对1例乳腺癌肉瘤进行组织病理学和免疫表型观察并复习文献,了解该肿瘤的特征。结果镜检显示病变边界清楚,以软骨肉瘤为主,其边缘有少量分化较差的浸润性导管癌成分。免疫表型：软骨肉瘤细胞vimentin强阳性,S-100蛋白阳性,ER、PR,c-erbB-2和CK（AE1／AE3）均阴性;癌细胞CK（AE1／AE3）、ER、PR及c—erbB—2均阳性,vimenfin、S—100蛋白阴性。结论原发于乳腺的癌肉瘤较罕见,诊断需与乳腺恶性分叶状肿瘤以及软组织肉瘤鉴别。     

肺原发性绒毛膜癌1例报道及文献复习

目的 探讨肺原发性绒毛膜癌临床病理特征、诊断标准、治疗及临床转归。方法 分析1例肺原发性绒毛膜癌的临床表现、组织病理学及免疫表型特征,并进行文献复习。结果 患者,男性,53岁。临床表现咳嗽、咳血痰。影像学检查示右肺下叶肿块,全身他处未见异常。病理学检查发现肿物几乎均可见出血、坏死,其中有少数肿瘤细胞呈簇团状或小片状分布。瘤组织由中等大小、胞质透明的细胞（细胞滋养层细胞）和大而异型、胞质丰富、粉染或空泡状的多核巨细胞（合体滋养层细胞）构成。免疫表型显示瘤细胞,特别是多核巨细胞表达CK（AE1／AE3）、CK7、HCG、PLAP、Ki-67及p53,但不表达CEA、TTF1、CK20、AFP等。术后联合化疗,无瘤生存12个月,仍在随访中。结论 肺原发性绒毛膜癌是一种罕见的高度侵袭性肿瘤,具有其独特的临床病理特征,手术联合化疗是最有效的治疗方案。     

Rosai-Dorfman disease of the pericardium: a case report and review of literature

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disease of unknown etiology. It typically presents as massive lymphadenopathy with a predilection for the cervical lymph nodes of children and young adults. However, extranodal involvement is not uncommon and may cause confusion with other neoplasms or reactive disease. We describe here a unique case of extranodal RDD manifesting as a pericardial mass in a 69-year-old man. The lesion was detected by computed tomography during a periodic examination of the chest. Subsequently positron emission tomography scan showed mild increase of flurodeoxyglucose uptake. Clinically, it was supposed to be a mesothelioma. Histological examination showed the typical features of RDD confirmed by the staining of S100 protein, which highlighted the emperipolesis of the characteristic histiocytes. To the best of our knowledge, pericardial RDD represents an extremely rare condition and should be included in the differential diagnosis of pericardial neoplasms.     

肾集合管癌1例报道及文献复习

目的 探讨起源于肾髓质集合管的肾集合管癌，分析其病理形态学特征及鉴别诊断。方法 对1例肾集合管癌进行大体、光镜及免疫组化观察并结合文献分析。结果 镜下肿瘤呈弥漫性或腺管状、乳头状排列。肿瘤纤维性间质反应性增生。免疫表型肿瘤细胞高分子CK、EMA、vimentin、溶菌酶阳性。结论 肾集合管癌是一种起源于肾髓质集合管的罕见的肾肿瘤，恶性度高，预后差。     

Superficial myofibroblastoma of the lower female genital tract: report of a series including tumours with a vulval location

AIMS: To describe the clinical and pathological features of 12 further cases (in 11 patients) of superficial cervicovaginal myofibroblastomas (SCVM), rare tumours that hitherto have been described only in a single series of cases involving the cervix and vagina. METHODS AND RESULTS: The patients' ages ranged from 23 to 80 years. Ten tumours were located in the vagina and two in the vulva. Three patients had been taking tamoxifen. The tumours ranged in size from 2 to 45 mm and morphologically were well-circumscribed lesions composed of bland ovoid to spindle-shaped cells, often with wavy nuclei. These cells were arranged in a variety of architectural patterns and were set in a finely collagenous stroma. Five cases exhibited stromal oedema or myxoid change and in eight cases hyalinized areas with thick, dense collagen bundles were present. Immunohistochemically, there was positivity with vimentin (11 of 11 cases tested), CD34 (six of 12 cases), desmin (nine of 12 cases) and oestrogen receptor (nine of 11 cases). All cases tested were negative for smooth muscle actin, S100, h-caldesmon, HMB45, and CD31. In this study we expand on the morphological spectrum of these rare lesions and reiterate their association with tamoxifen. CONCULSIONS: Since these lesions may occur on the vulva, as well as the cervix and vagina, we propose the term 'superficial myofibroblastoma of the lower female genital tract'.     

多囊性肾细胞癌1例报道及文献复习

目的：阐明多囊性肾细胞癌的病理形态学特征及鉴别诊断要点。方法：报道1例多囊性肾细胞癌,结合文献对本病的临床病理特征进行探讨。结果：肿瘤由厚的假纤维被膜环绕,瘤体全部由大小不等的囊腔及无膨胀性实性结节的薄的间隔构成,囊腔被覆透明细胞,异形性小,间隔内含聚集的透明细胞上皮巢,结论：多囊性肾细胞癌是一种罕见的有低度恶性倾向的肿瘤,术前影像学,细胞学检查及术后的肉眼特征均与囊性肾瘤,囊性部分分化性肾母细胞瘤相似,最重要的是本瘤缺乏肉眼所见的膨胀性实性结节,显微镜下的关键特征是间隔内可见聚集的透明细胞巢。