1、Mammary-type myofibroblastoma乳腺型肌纤维母细胞瘤乳腺型肌纤维母细胞瘤一、Definition 定义定义A benign mesenchymal neoplasm良性间叶性肿瘤 composed of spindle-shaped cells with features of myofibroblasts,由梭形有肌纤维母细胞特征的细胞构成的embedd in a stroma that contains coarse bands of hyalinized collagen间质内含有玻璃样变的粗大胶原纤维条带 and conspicuous mast cells
2、可见大量的肥大细胞and admixed with a variable amount of adipose tissue.并伴有数量不等的脂肪组织。the tumour is histologically identiacal to myofibroblastoma of breast.其组织学特点与乳腺的肌纤维母细胞瘤相似。二、Epidemiology 流行病学流行病学Lesion have arisen in adults with an age range of 35 to 67years(median 55.5 years)and a male predilection(8males
3、,2females).发病年龄见于35-67成年人（平均55.5）男性多见（8例男性，2例女性）The extrammary location of some myofibroblastomas has only recently been defined when 10 cases were reported.乳腺外的肌纤维母细胞瘤直到最近才有10 例报告。Therefore,conclusions related to epidemiology could alter with increased tumour recognition因此随着对肿瘤认识的增加，流行病学方面也会发生改变。三、
4、sites of involvement 受累部位受累部位The most common location of mammary-type myofibroblastoma is the inguinal/groin area.乳腺型肌纤维母细胞瘤最常见于会阴/腹股沟区域。Other reported sites include abdominal wall，buttock,back and vaginal wall.其他部位有腹壁，臀部，背部和阴道壁。Lesions arise most commonly in subcutaneous tissue.最常见的发病部位是皮下组织。Howeve
5、r,cases have arisen deep to abdominal wall muscle，in the posterior vaginal wall and in a paratesticular location.但也可位于腹壁肌肉，阴道后壁和睾丸旁。There is an apparent predilection for myofibroblastomas to arise along the putative anatomic“milk-line”that extends from axilla to medial groin.肌纤维母细胞瘤明显好发于从腋窝至腹股沟中部的“白
6、线”四、Clinical features 临床特点临床特点The tumours generally present as either painless masses or incidental lesions that are detected during surgical procedures such as inguinal hernia repair.多为无痛性的肿块或由外科进行腹股沟疝修补术时偶然发现隐匿肿块。Occasional lesions are tender or painful 偶尔病变区触痛或疼痛。Tumours have been described to be
7、 present for up to a year before clinical presentation.肿瘤就诊前可能已经存在达1年之久There are no imaging data无影像学资料数据五、Aetiology 病因学病因学Unknown 病因不明 It has been postulated that myofibroblastomas arising in the breast may be related to a patients hormonal status,in that lesions are relatively common in older men.e
8、.g.in the setting of gynaecomastia and anti-androgen therapy.据推测发生于乳腺的肌纤维母细胞瘤与患者的激素水平有关.这种情况多见于老年男性。如：男性乳腺发育和抗雄激素治疗的患者。Mammary-type myofibroblastoma of soft tissue arises most commonly in older adult males.软组织的乳腺型肌纤维母细胞瘤常见于老年男性The apparent predilection for origin of myofibroblastomas along a putativ
9、e milk-line sugests the possibel existence of hormonally-responsive mesenchymal tissue明显好发于从腋窝至腹股沟中部的解剖学“白线”区域。暗示这一区域存在对激素敏感的间叶组织细胞。六、Macroscopy 肉眼大体观肉眼大体观Reported lesions ranged in size from 2 to 13cm(median 5.8cm).已报告病例的肿块直径从到13cm，平均5.8cmThe tumour are well circumsribed and firm.界清，质硬The colour ca
10、n be variable(white,pink,tan or brown)色泽多变，可为白色，粉红色，黝黑色棕褐色The cut surface may be whorled or nodular.切面可呈漩涡状或结节样Soft“mucoid”-appearing areas reflecting myxoid change were present in one case 一例病变 质软、粘液变。七、Histopathology 组织学组织学Tumours are unecapsulated but well circumscribed.肿瘤无包膜，但是分界清楚They are compo
11、sed of an admixture of spindle cells and adipose tissue由梭形细胞与脂肪细胞混合构成And are morphologically identical to mammary myofibroblastoma形态类似于乳腺肌纤维母细胞瘤The spindle cells histologically resemble myofibroblasts 梭形细胞特点类似于肌纤维母细胞and are characterized by oval to tapered nuclei 其核形从梭形至卵圆形with finely dispersed chro
12、matin，small nucleoli核内染色质均匀散布，常见小核仁Eosinophilic to amphophilic cytoplasm 胞浆从嗜伊红到嗜双色性And poorly defined cytoplasmic borders 胞质界限不清 The spindle cells are frequently wavy in contour 梭形细胞的轮廓常呈波浪样and generally are arranged in variably sized fascicles一般排列成不同大小的束状The stroma is collegenous with broad bands
13、of coarse hyalinized collagen that often adopt a zig-zag pattern基质为边缘粗糙的，带状的，玻璃样变的胶原带，胶原带，常形成Z字锯齿样图案Stromal mast cells are usually numerous 基质内常有大量肥大细胞Epithelioid change of the lesion cells 病变细胞常呈上皮样变And focal nuclear atypia with enlarged nuclei and multinucleation have been described.局灶上皮样细胞核不典型性，伴
14、核增大，多核Such morphologic variation is well recongnized in mayofibroblastoma of breast以上形态学的变化在乳腺肌纤维母细胞瘤已有清楚的认识。The blood vessels in myofibroblastoma are generally not conspicuous，being small 肌纤维母细胞瘤肿块内的血管不明显，常为小血管And commonly having a perivascular lymphocytic inflitrate并且，血管周围有淋巴细胞渗出。in contrast to th
15、e prominent medium to large vessels with markedly hyalinized walls that are characteristic cellular angiofibroma 与之不同的是，细胞性血管纤维瘤内有醒目的中等血管和大血管,并且血管壁显著地玻璃样变Or the large branching“haemangiopericytomatous”blood vessels that are seen in lipomatous haemangiopericytoma而脂肪瘤样血管外周细胞瘤组织内有大的分支状“血管外周细胞瘤样”血管。two
16、potential morphologic mimics.以上二种肿瘤在形态学上与肌纤维母细胞瘤有相似之处。八、Immunophenotype 免疫表型免疫表型As is characteristic of the breast counter part，the typical immunphenotype of extramammary myofibroblastoma is diffuse co-expression by the spindle cells of desmin and CD34.乳腺外的肌纤维母细胞瘤的免疫表型对应于乳腺肌纤维母细胞瘤（双阴性），乳腺外的肌纤维母细胞瘤的梭
17、形细胞弥漫性，双表达desmin（肌间线蛋白）和CD34Expression of smooth muscle actin is seen in a third of case1/3的梭形细胞表达 SMA(平滑肌动蛋白)。九、Prognostic factors 预后预后All tumours have followed a benign course following marginal local excision.在肿块切除后，遵循良性肿瘤的经过However,the reported follow-up time is limited(up to 26months)只是术后随访时间有限（最长达26个月）九、鉴别诊断鉴别诊断细胞性血管纤维瘤（cellular angiofibroma）孤立性纤维性肿瘤（solitary fibrous tumour）血管肌纤维母细胞瘤（angiomyfibroblastoma）梭形细胞脂肪瘤（spindle cell lipoma）
