软组织骨化性纤维粘液瘤一例

软组织骨化性纤维粘液瘤一例李懿堂，李光容，杨红，胥维勇，杨果患者女性，３１岁。发现左肩部肿块５年。肿块开始为绿豆大小，质软，生长缓慢，近２年增大如蚕豆，质变坚硬，有压痛。肿块位于左肩近脊柱部位，与皮肤粘连，直径１．５ｃｍ，边界清楚，部分有分叶。局部切...     

软组织骨化性纤维黏液样肿瘤2例报道及文献复习

目的 探讨软组织骨化性纤维黏液样肿瘤(ossifying fibromyxoid tumor of soft tissue,OFT)的临床病理、免疫表型特征及其诊断和鉴别诊断.方法 对2例OFT临床病理资料进行分析、光镜观察及免疫组化标记,并复习相关文献.结果 2例患者临床表现均为皮下缓慢生长的肿块.手术完整切除肿块,大体标本表面均有一层不完整的骨壳.术后病理组织学检查见肿瘤边界清楚,有纤维性假包膜,包膜内有一层薄的不连续的由成熟化生的板层骨构成的骨小梁,肿瘤略呈分叶状,内见有圆形、卵圆形、短梭形瘤细胞呈巢状、索状、网格状、镶嵌状排列,核染色质细致,未见明显核分裂象,有明显纤维黏液样背景的间质.免疫表型:vimentin、S-100蛋白均(+),CK(-).结论 OFT是一种分类尚未确定的罕见肿瘤,属中间型肿瘤.其特征性的骨壳结构、独特的细胞形态及排列方式、免疫表型具有诊断意义.     

软组织骨化性纤维粘液样瘤八例临床病理分析

目的：研究软组织骨化性纤维粘液样瘤（OFT）的病理形态学特点和免疫组织化学表型,并探讨其组织发生,方法：采用光镜观察（HE）和免疫组织化学标记（LSAB法）结合临床资料对8例软组织骨化性纤维粘液样瘤进行临床病理学分析。结果：8例患者均为中老年人,年龄43－78岁,平均63岁,临床上多表现为四肢近端皮下缓慢性生长的肿块,组织学上,该肿瘤归纳起来有以下3个持征性的形态,比较独特,具有诊断性意义：（1）肿瘤境界清楚,有一层厚的纤维性假包围绕,多数病例于包膜内可见一层薄的不连续性骨壳,由成熟的化生性板层骨组成;（2）肿瘤的实质由多个大小不一,细胞密度不均的小叶组成,小叶内的瘤细胞呈圆形,卵圆形或短梭形,胞质淡染或呈嗜伊红色,核染色质细致,核分裂象偶见;（3）瘤细胞呈特征性的巢状,条束状或纤细的网格六排列,肿瘤的基质呈特征性的纤维粘液样,7例行免疫组织化学标记,结果显示瘤细胞强阳性表达波形蛋白和神经元特性烯醇化酶（7／7）,多数表达S－100蛋白（6／7）,部分表达结蛋白（2／7）,7例均有随访资料,2例分别于术后2年及15年复发。结论：软组织骨化性纤维粘液样瘤是一种好发于中老年人的具潜在低度恶性的软组织肿瘤,其特征性的骨壳结构,独特的细胞形态及其排列方式具有诊断意义。免疫组织化学检测结果支持OFT起源于雪旺细胞。     

骨化性纤维黏液样肿瘤临床病理观察及文献复习

目的 提高对软组织骨化性纤维黏液样肿瘤(ossifying fibromyxoid tumor,OFT)的认识.方法 对4例OFT采用光镜观察及EnVision两步法免疫组化标记,并结合文献对其良恶性进行讨论.结果 4例OFT均为中年男性,平均年龄43.5岁.临床表现为四肢皮下缓慢生长的肿块.3例肿瘤镜下境界清楚,有一层厚的纤维性假包膜围绕,包膜内可见一层薄的不连续性骨壳,由成熟的化生性板层骨组成;肿瘤实质由多个大小不一的小叶组成,小叶内瘤细胞呈特征性巢状、条索状或纤细网格状排列,肿瘤间质呈黏液样.瘤细胞呈上皮样圆形、卵圆形或短梭形,细胞境界较清楚,胞质淡染或嗜酸性.3例核染色质细致,核分裂象偶见(＜2个/50 HPF);1例表面仅见无骨壳的纤维性包膜,肿瘤中心见不规则骨样基质,细胞密度增高,胞核有异型,核分裂＞2个/50 HPF.免疫组化结果显示瘤细胞均表达vimentin(4/4),多数表达S-100蛋白(3/4),部分表达desmin、CD56及CKpan.3例典型OFT随访12～38个月未复发,1例非典型/恶性OFT术后18个月复发.结论 OFT是一种好发于中老年人的软组织肿瘤,具有一定的组织形态学特征,需与神经鞘膜瘤、皮肤混合瘤及低度恶性纤维黏液样肉瘤鉴别.     

表浅性血管黏液瘤一例

患者男,60岁.因右足第一趾外伤后经久不愈10年,时有痒感,于2008年4月8日就诊于浙江省嘉兴市中医医院.检查见右足第一趾背侧有一5 cm×5 cm×3 cm肿块,表面有溃疡形成,肿块境界尚清,质稍硬,易出血.全身检查未见异常.患者既往健康,无心脏黏液瘤,家族中无类似疾病患者.当日行肿物切除术,术中见右足第一趾肿块,深达骨膜,质稍硬,有包膜,境界较清晰,甲床破坏.     

腰部黏液样隆突性皮肤纤维肉瘤1例

患者女性,47岁,发现左侧腰部肿物2个月,于2017年12月13日入院。患者2个月前发现左腰部有一肿块,约桂圆大小,无疼痛,2个月后肿块逐渐增大,略感不适。体检:左侧腰部皮下可触及一大小5.0 cm×5.0 cm×2.0 cm肿块,质软,无压痛,边界清,活动可,于2017年12月14日行肿块完整切除术,术后未进一步治疗。术后2个月余,肿瘤复发,于2018年2月5日再次入院行肿块扩大切除术,术中见肿块大小2 cm×2 cm×1.5 cm,切缘术中快速冷冻病理检查为阴性。     

黏液炎性纤维母细胞肉瘤一例

患者男,90岁.因发现左前臂肿块并逐渐增大半年于2008年8月18日就诊.体检:左前臂皮下可触及-4 cm×2cm×2 cm大小肿块,活动欠佳,无压痛,行肿块局部切除术,术中见肿块位于皮下深层,边界不清.     

肺微囊性纤维黏液瘤：3例报道

肺的黏液样肿瘤非常少见。转移性肉瘤，尤其是骨外黏液样软骨肉瘤或肺唾腺型混合瘤的变型是最常见的以黏液样表现为主的肺肿瘤。目前，唯一详尽描述的肺良性黏液样肿瘤是黏液样错构瘤。仅有少数原发性肺黏液瘤与英文文献报道一致。然而，这些病例未能很好得到病理学的证实，有些病例可能是黏液样错构瘤或其它黏液样肿瘤。作者报道了3例具有显著微囊结构的肺良性黏液样肿瘤，并详细描述了它们的组织学和免疫组织化学特征。3例常规X线检查均为偶发的肺周围孤立性结节，其中包括2例年龄分别为45和65岁的女性和1例33岁的男性。临床检查其它部位无肿瘤生长。外科切除术后平均随访48个月无复发。肿瘤大小为1．0～2．3cm（平均1．4cm）。     

胃丛状纤维黏液瘤2例

<正>例1女性,66岁,近期无明显诱因出现上腹痛并向后背放射,间断反酸、恶心。于就诊前3天出现黑便5~6次/日,并伴头晕、心慌、腹胀。体格检查:神清,轻度贫血貌。实验室检查:红细胞3.2×10~(12)/L、白细胞5.8×10~(12)/L、血红蛋白68 g/L。CT示胃窦壁增厚,呈软组织肿物影突入胃腔内,腹腔及腹膜后未见肿大淋巴结。胃镜示胃窦大弯侧可见1枚球状隆起2~3 cm,表面黏膜糜烂,考虑间叶源性肿瘤;行胃     

Fine‐needle aspiration features of ossifying fibromyxoid tumor in the breast: A case report and literature review

Ossifying fibromyxoid tumor (OFT) is a very rare soft tissue tumor which is characterized by incomplete peripheral mature bone shell. To date, cytological features of OFT have been poorly studied with only seven case reports. In this study, an additional case of OFT investigated by fine‐needle aspiration is presented. A 75‐year‐old man with advanced nasopharyngeal carcinoma presented with peripherally calcified right breast mass. Smears were hypercellular and stroma‐rich. Tumor cells were mainly dispersed, with epithelioid morphology and eccentrically located nuclei. In the background, there was abundant eosinophilic myxoid secretion. No necrosis, atypia, or mitotic activity was found. The tumor showed diffuse S‐100, CD10, STAT6 expression and focal desmin, estrogen receptor (ER), and progesteron receptor (PgR) expression. Fluorescence in situ hybridization study revealed PHF1 rearrangement in 9% of cells. Cytological characteristic of OFT is quite distinctive and precise diagnosis can be made, especially when it is coupled with compatible radiological findings.     

Atypical ossifying fibromyxoid tumor unusually located in the mediastinum: report of a case showing mosaic loss of INI-1 expression

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue tumor. OFMT mostly arises in subcutaneous tissue or skeletal muscle of the extremities and is extremely unusual in the mediastinum. OFMT is classified as typical, atypical, or malignant as tumor aggressiveness increases. Herein, we presented a case of atypical OFMT that developed in the mediastinum of a 43-year-old woman. Because of its predominant hypercellular area and some tumor cells with high nuclear grade, it was not a typical OFMT. However, it did not have a sufficient number of mitotic figures to be classified as malignant. Hence, we classified it as atypical OFMT with some apparent characteristic features of OFMT, such as the presence of spicules of bone at the periphery of the tumor. Upon immunohistochemistry, it was positive for vimentin, S-100 protein, and CD10, which was consistent with a diagnosis of OFMT. Particularly noteworthy was the mosaic loss of INI-1 expression. Some OFMT and other exceptionally rare tumors have been reported to exhibit mosaic INI-1 loss. Inactivation of INI-1 gene and deregulation of PHF1 gene are thought to be involved in tumorigenesis of OFMT. Therefore, we speculated that the mosaic loss of INI-1 observed in the present case might also be related to a kind of abnormality of INI-1 as was reported previously.     

颈静脉球瘤1例及副神经节瘤文献复习

目的探讨颈静脉球瘤的临床病理特征和副神经节瘤的生物学行为。方法对1例颈静脉球瘤进行组织学观察和免疫组化标记,同时复习相关文献。结果颈静脉球瘤的组织学改变类似于其他部位的副神经节瘤,免疫组化标记:瘤细胞Syn、CgA和NSE均阳性,EMA、CK、HMB45、CD34和CD99均阴性。结论颈静脉球瘤是一种起源于副神经节的少见肿瘤,呈侵袭性生长,有低度恶性的生物学行为。根据肿瘤的组织学特征,结合CT或MR I影像学资料和免疫组化标记可以作出诊断。     

Paratesticular solitary fibrous tumor: a case report and review of literature

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually arising from the pleura, but has been reported in diverse extrapleural sites. Urogenital localization is rare, and only several cases of paratesticular SFT have been reported. In the present report, we present the case of a 61-year-old male suffering from a paratesticular SFT. A surgical excision of the lesion was performed. The tumor was well circumscribed and consisted of a mixture of bland spindle cells and dense collagen bands. Immunohistochemical studies showed positive reactivity for CD34, CD99 and vimentin, but stained negative for CD117, S100, SMA, HMB45, Desmin and CD68. All these clinicopathologic features are suggestive of the diagnosis of paratesticular SFT.     

肾素瘤1例临床病理及文献复习

目的：阐述肾素瘤的病因，病理特征及鉴别诊断要点。方法：对１例肾素瘤进行大体、光交易、免疫组化镜观察，并复习有关文献。结果：肾素瘤包膜完整光下酪似血管外皮瘤；免疫线化，部分细胞Ｖｉｍｅｎｔｉｎ呈阳性反应，瘤组织的腺样及乳头样结构区瘤细胞ｃｙｔｏｋｅｒａｔｉｎ呈阳性反应。电下瘤细胞胞浆内可见多量圆形分泌颗粒及棱莆和梯形结晶样物。结论：肾素瘤是发生于变更的肾小球入球小动脉血管平滑肌细胞的罕见衣性肿瘤，明     

Papillary glioneuronal tumor: a case report and review of the literature

Papillary glioneuronal tumor is a recently described central nervous system neoplasm that almost always occurs adjacent to the lateral ventricle. We present a case of this rare entity, representing the 21st case of this lesion, which exhibits a mixed astrocytic and neuronal differentiation. This case was an incidental finding in a young woman who presented secondary to a traumatic injury to the left eye. Histologic evaluation after surgical removal showed a cystic tumor consisting of 2 distinct components: a unique pseudopapillary architecture admixed with foci of solid areas. The pseudopapillae were composed of thick hyalinized vessels enclosed by a single layer of glial fibrillary acid protein-positive astrocytes and variously sized synaptophysin-positive and chromogranin-negative neuronal cells in the interpapillary regions. Abundant Rosenthal fibers, foci of calcification, areas of hemosiderin deposition, gliosis, areas of vascular proliferation associated with piloid gliosis, and chronic inflammatory infiltrate were identified. The combination of cytologic benignity, lack of necrosis, and low proliferative index as evidenced by immunohistochemistry using antibody to Ki-67 confirmed the low malignant potential of this tumor. Knowledge and precise classification of this entity are important to avoid unnecessary use of chemo- and/or radiotherapy for treatment.     

Papillary glioneuronal tumor: a case report and review of the literature

We report a papillary glioneuronal tumor occurring in the right frontal lobe of a 26-yr-old woman and we review the pertinent literature. Papillary glioneuronal tumor (PGNT) is a rare cerebral neoplasm, identified in approximately 37 cases to date. In 2007, the World Health Organization (WHO) classified the PGNT as a grade I neuronal-glial tumor because of its biphasic neurocytic and glial components and indolent clinical course. Patients commonly present with headaches or seizures, but may be asymptomatic with the mass discovered incidentally upon neuroimaging. Histology demonstrates a pseudopapillary architecture with a single or a pseudostratified layer of glial cells overlying hyalinized vasculature with interpapillary regions of neurocytic or ganglion cells. Peripheral eosinophilic granular bodies, Rosenthal fibers, hemosiderin, and areas of calcification are often noted. The PGNT displays moderate cellularity and is typically devoid of necrosis, microvascular proliferation, and mitoses. Its immunohistochemical profile includes glial fibrillary acidic protein (GFAP)-positive glial cells, synaptophysin-positive interpapillary neurocytes, and MIB-1 labeling in the range of 1-2%.