乳腺软组织骨化性纤维黏液样肿瘤1例

1临床资料 患者,女性,26岁,因发现左乳肿块两年余入院。B超检查示：双乳小叶增生,左乳偏低回声团（大的病灶内伴钙化斑,RI值偏高）。专科体检左乳腺上方12点钟距乳头约3cm处扪及一肿块,大小3.0cm×2.0cm×2.0cm,表面光滑,边界尚清,活动可,无压痛,左乳外下5点钟位置乳晕边缘扪及一肿块大小约1．0cm×1．0cm×0．8cm,质中,边界清,活动可,无压痛。     

深部侵袭性血管黏液瘤一例

患者 女，15岁，因经期阴道脱出肿物4个月入院。肿物随月经来潮时脱出阴道，如鸽蛋大小，质中，无伴腹痛，无排尿、排便困难，于月经期第3天自行回纳。患者未婚，12岁月经初潮，个人史无特殊。腹部超声提示阴道肿物，大小4．0cm×1．9cm×3．2cm。术中见肿物约5．0cm×4．0cm×4．0cm大小，质中，表面似欠光滑，根部附于左侧阴道壁中上段，在肿物基底部扪及质地稍硬条索状物，     

侵袭性血管黏液瘤研究进展

侵袭性血管黏液瘤（aggressive angiomyxoma,AA）是一种少见的、通常发生在生育期女性外阴和盆腔等部位的间叶源性肿瘤。虽然具有局部浸润和局部复发的生物学行为特点,但目前的观点认为其并非恶性肿瘤。2003年第3版和2013年第4版世界卫生组织（WHO）肿瘤分类将其纳入骨和软组织肿瘤类别,并将其划入分化不确定的肿瘤类型。迄今为止国内外报道的AA病例数已超过350例,这些报道均以病例报告并文献复习的形式为主。本文将结合最新文献就AA的研究进展进行综述。      

阴道侵袭性血管黏液瘤临床病理观察

目的：探讨侵袭性血管黏液瘤（aggressive angiomyxoma,AAM）的临床病理特征。方法：回顾性分析1例侵袭性血管黏液瘤的临床病理学资料。结果：采取手术治疗,术中见：肿瘤无包膜,剖面均一灰白色、胶质样,有黏液性间质分隔。病检示：镜下见瘤细胞呈梭形或星形疏松排列于黏液间质背景中,有厚壁血管。免疫组化显示：Vimentin（＋）,Actin（＋）,CD34（＋）,HMB45（-）,S-100（-）,Myoqlobin（-）。结论：AAM是一种罕见的、局部浸润性间质肿瘤。侵袭性、复发性是其重要特征,需提高首次诊断的正确性,首次治疗以局部广泛切除为宜,且需长期随访监测。     

低度恶性纤维黏液性肉瘤一例并文献复习

目的：分析低度恶性纤维黏液样肉瘤的临床病理特征及鉴别诊断。方法：对一例低度恶性纤维黏液样肉瘤进行大体、光镜及免疫组化观察并结合文献复习。结果：瘤细胞排列呈漩涡状,散布在富于血管的纤维黏液样间质中,黏液区与纤维区明显过渡。免疫组化肿瘤细胞SMA（-）、EMA（-）、S-100（-）、CD99（-）、CD68（-）。结论：低度恶性纤维黏液样肉瘤是g 种非常少见的软组织肿瘤,常被误诊为良性肿瘤,需与黏液样纤维肉瘤I级、黏液样神经纤维瘤、恶性纤维组织细胞瘤等鉴别诊断。     

低度恶性纤维黏液性肉瘤一例并文献复习

目的:分析低度恶性纤维黏液样肉瘤的临床病理特征及鉴别诊断.方法:对一例低度恶性纤维黏液样肉瘤进行大体、光镜及免疫组化观察并结合文献复习.结果:瘤细胞排列呈漩涡状,散布在富于血管的纤维黏液样间质中,黏液区与纤维区明显过渡.免疫组化肿瘤细胞SMA(-)、EMA(-)、S-100(-)、CD99(-)、CD68(-).结论:低度恶性纤维黏液样肉瘤是一种非常少见的软组织肿瘤,常被误诊为良性肿瘤,需与黏液样纤维肉瘤Ⅰ级、黏液样神经纤维瘤、恶性纤维组织细胞瘤等鉴别诊断.     

低度恶性纤维黏液样肉瘤的临床病理特征

目的:了解低度恶性纤维黏液样肉瘤的临床病理特征.方法:收集2例低度恶性纤维黏液样肉瘤的临床资料,手术切除组织光镜切片观察,另作9项免疫组织化学标记,CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68.结果:2例男女各1例, 肿瘤由梭形细胞区和星形细胞黏液样区构成,1例有典型的巨形菊形团样结构.肿瘤呈浸润性生长,易复发.免疫组织化学标记瘤组织CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68阴性,Vimentin阳性.结论:低度恶性纤维黏液样肉瘤的诊断靠病理组织学和免疫组织化学标记,手术切除为主要的治疗方法.     

低度恶性纤维黏液样肉瘤的临床病理特征

目的：了解低度恶性纤维黏液样肉瘤的临床病理特征。方法：收集2例低度恶性纤维黏液样肉瘤的临床资料,手术切除组织光镜切片观察,另作9项免疫组织化学标记,CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68。结果：2例男女各1例,肿瘤由梭形细胞区和星形细胞黏液样区构成,1例有典型的巨形菊形团样结构。肿瘤呈浸润性生长,易复发。免疫组织化学标记瘤组织cKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68阴性,Vimentin阳性。结论：低度恶性纤维黏液样肉瘤的诊断靠病理组织学和免疫组织化学标记,手术切除为主要的治疗方法。     

侵袭性血管黏液瘤临床病理分析

目的探讨侵袭性血管黏液瘤的临床和病理特点以及预后.方法分析3例女性生殖器侵袭性血管黏液瘤的临床病理资料,并进行免疫组织化学染色分析.结果 1例发生于外阴,2例发生于会阴及/或盆腔.组织学上见梭形或星形细胞增殖,间隔以广泛疏松的黏液样基质、明显的管径大小不一的血管及胶原纤维.免疫组织化学检测显示,瘤细胞vimentin(+++)(3/3),SMA(+/-～+)(3/3),PCNA(+/-～+)(2/3),S-100(-),CD68(-).结论侵袭性血管黏液瘤是一种少见的中间性(局部侵袭性)间叶性肿瘤,生长缓慢,但常局部浸润,易复发,彻底的局部切除可减少复发率.免疫组织化学提示其肌纤维母或纤维母细胞来源可能性大.需与血管肌纤维母细胞瘤、浅表性血管黏液瘤、细胞性血管纤维瘤、纤维上皮性息肉、浅表性肌纤维母细胞瘤及其他许多肿瘤鉴别.     

宫颈原发性腺泡状软组织肉瘤临床病理观察

目的:探讨腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征。方法:对1例ASPS进行大体、组织病理学和免疫组化染色观察,并复习相关文献。结果:患者,女,33岁,ASPS发生于宫颈。镜下肿瘤细胞排列成腺泡状,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。PAS染色,瘤细胞胞质内可见棒状结晶体。免疫组化:Vim阳性,desmin、SMA、myoglobin、CgA、S-100、EMA、AEI/AE3、CD68、和HMB45(-)全部为阴性。结论:宫颈ASPS是一罕见的恶性软组织肿瘤,结合临床病理学特征及免疫组化,可作出正确诊断。     

腺泡状软组织肉瘤的影像学特征及临床病理表现

背景与目的:腺泡状软组织肉瘤罕见,其影像学表现至今尚未见系统研究,本文旨在探讨腺泡状软组织肉瘤(alveolar soft part sarcomas,ASPS)的临床特征和影像学表现,以提高诊断的准确率。方法:回顾性分析10例经病理证实的腺泡状软组织肉瘤的临床特征和影像学表现,所有患者(术前或活检前)均行X线、CT或MR检查。其中,9例行X线平片检查,9例行CT检查,6例行MRI检查。所有切除或活检组织均行HE染色,5例患者有比较完整的免疫组化结果。结果:ASPS发病年龄较轻,80%(8/10)为30岁以下。多表现为无痛性肿块。3例就诊时已有肺转移。70%(7/10)发生于下肢深部软组织以及臀部。另3例分别位于胸壁、颈部及眼眶内。ASPS的CT表现为软组织肿块影,增强后呈明显不均匀强化。MRI表现为T1WI等或略高信号,T2WI高信号,肿瘤内外可见血管流空信号,增强后肿瘤呈不均匀明显强化。镜下ASPS是由嗜伊红色的大多边形上皮样细胞组成,呈特征性的器官样或腺泡状排列,腺泡之间为衬覆单层扁平内皮细胞的裂隙状或血窦样毛细血管网。免疫组化显示3例神经元特异性烯醇化酶(NSE)阳性,两例抗淀粉酶消化染色(PAS)阳性,1例MyoD1横纹肌特异肌调节蛋白阳性(胞质染色),1例Desmin结蛋白阳性。结论:ASPS虽然是少见软组织肉瘤,但影像学很有特点,结合临床、影像和病理表现是诊断的关键。     

增生性筋膜炎9例临床病理分析

目的:探讨增生性筋膜炎(PF)临床病理特点及鉴别诊断,提高对增生性筋膜炎的认识,避免误诊.方法:回顾性分析9例增生性筋膜炎的临床情况、组织学形态及免疫表型并复习相关文献.结果:PF好发于四肢及头颈部,发病年龄为32 ～ 80岁.临床表现为伴有疼痛,迅速生长浅表孤立性肿块.镜下特点为黏液样背景、增生的纤维母细胞/肌纤维母细胞、间质散在分布具有诊断意义的神经节样大细胞;免疫组化梭形细胞及神经节样大细胞表达Vimentin、SMA和H-Caldesmon.结论:PF是一种较少见的良性病变,手术切除后罕见复发,不转移,其特殊的临床表现及病理形态特征,易被误诊为恶性肿瘤.应加强对该病变的认识,避免误诊.     

肢体恶性软组织肿瘤的保肢治疗

目的 探讨四肢恶性软组织肿瘤保肢治疗的可行性。方法 22例肢体恶性软组织肿瘤患者中,滑膜肉瘤9例,横纹肌肉瘤及恶性纤维组织细胞瘤各5例。透明细胞肉瘤2例,恶性黄色肉芽肿1例。其中股部9例,臀部4例,膝部、足跟部各3例,小腿外侧、腕部、足背部各1例。16例行肿瘤扩大切除术,6例行肿瘤扩大切除皮瓣修复术。术后采用CVADIC方案行化疗,16例行放疗。结果 19例获得平均4年2个月的随访,2例复发,2例死亡(1例为复发后再次手术患者)。结论 肿瘤切除彻底是四肢恶性软组织肿瘤保肢治疗的前提;放疗可以改善局部控制率;CVADIC方案是治疗恶性软组织肿瘤的可行方案;肿瘤切除后软组织缺损修复,应选择操作简单、就近取材、效果良好的方法。     

The clinical role of molecular genetics in soft tissue tumor pathology

Cytogenetic and molecular analysis of soft tissue tumors has yielded a wealth of information over the past decade. Some of the genetic aberrations that have been identified appear to be fairly specific for individual tumor types. It is because of this specificity that these findings harbor the promise to become useful as diagnostic and/or prognostic markers. Technical advances that allow the application of cytogenetic and molecular techniques to archival material have been crucial in this respect.Molecular genetics has already become an integral part of the work-up of some tumors, e.g., small cell sarcomas of childhood, which demonstrate fairly characteristic translocations, often involving the Ewing's sarcoma gene. Some genetic abnormalities have become established as prognostic marker, such as the deletion of the short arm of chromosome 1 for neuroblastomas.Soft tissue tumor pathology has also benefitted from major advances in identifying genes that are critical in mesenchymal differentiation or cell cycle control. MyoD is a good example of a such a gene, that has become useful as a diagnostic tool in rhabdomyosarcomas.Beyond potential practical applications of cytogenetic and molecular analyses in the diagnosis of these tumors, we also review their impact on several philosophical concepts concerning soft tissue neoplasia.     

软组织软骨瘤临床病理学特征分析

目的:探讨软组织软骨瘤(STC)临床病理学特征及其组织学发生机制.方法:对11例STC的临床表现、组织学形态及免疫组化表型进行分析.结果:临床上主要表现为局部缓慢性生长的无痛性肿块,少数伴有局部压痛,术后罕见复发.影像学显示,与骨无关联的软组织内肿块伴有程度不等的钙化.镜下肿瘤组织境界清楚,分叶状,由成熟透明软骨或纤维软骨构成,可伴有程度不等的钙化、黏液变及囊性变;软骨瘤细胞形态不一,多无异形性,核分裂像罕见;少数软骨小叶周边梭形间质细胞密集,无胞周空晕或陷窝状结构.免疫组化显示,所有成分均表达vimentin,成熟软骨细胞及梭形软骨细胞还部分表达S100及CD34;软骨结节周边梭形间质细胞部分表达a-SMA、MSA及CD34,散在表达CD163;透明软骨基质CollagenⅡ表达程度不等,Collagen Ⅰ及CollagenⅢ呈阴性表达或局灶弱阳性表达;纤维软骨基质及钙化区表达Collagen Ⅰ及CollagenⅢ,CollagenⅡ表达阴性或局灶弱阳性表达.结论:STC是一种较为少见的软组织良性软骨性肿瘤,STC的形成可能最初由间充质细胞演化为具有CD34阳性表达的梭形间质细胞,继而向软骨方向分化形成STC,但这一演化机制有待深入研究.     

Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group

Background:Alveolar soft part sarcoma (ASPS) is a rare malignanttumor and little is known about its clinical features and management. Wereport on a series of 19 pediatric patients managed over 20 years. Patients and methods:Primary conservative surgery was performedin all patients and was radical in nine, non-radical in three; seven patientsunderwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In twocases radical surgery was performed after primary chemotherapy. Radiotherapywas delivered to 8 patients, chemotherapy to 15. Results:After a median follow-up of 74 months, the five-yearsurvival was 80% for the whole series, 91% for patients withlocalized disease, 100% for patients with tumor 5 cm, and31% for those >5 cm; 16 of 19 patients were alive (12 of 12 withgrossly-resected tumor in first continuous remission). Chemotherapy achievedtwo partial remission among seven evaluable patients. Conclusions:Pediatric ASPS has a more favorable prognosis thanits adult counterpart. In this series, tumor size correlates with metastaticdisease at onset and is the major factor influencing survival. Surgery is themainstay of therapy. The effectiveness of adjuvant therapy remains to beestablished, though radiotherapy may be advisable in cases of inadequatesurgery.     

Cytodiagnosis of soft tissue tumors

The only acceptable definitive diagnosis of a soft tissue mass is histologic or cytologic examination. In recent years, fine-needle aspiration cytology is used in more and more centers for diagnosis of soft tissue masses. We studied 196 aspiration cytologies performed on soft tissue lesions. Out of these, in 48 cases a definitive surgical procedure or open biopsy for histology and further evaluation were performed. There were 25 sarcomas and 23 benign tumors. There was one false negative cytologic result in this group; no false positive cytologies were detected. It seems that cytodiagnosis of soft tissue masses performed by an experienced pathologist is the method of choice, permitting a good diagnostic evaluation, with almost none of the traumatic and oncologic disadvantages of the other methods of biopsy.