1乳腺型肌纤维母细胞瘤

nullMammary-type myofibroblastomaMammary-type myofibroblastoma 乳腺型肌纤维母细胞瘤 一、Definition 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 null and conspicuous mast cells 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。 null二、Epidemiology 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,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。null 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。 null三、sites of involvement 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。 nullLesions arise most commonly in subcutaneous tissue. 最常见的发病部位是皮下组织。 However,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“白线” null四、Clinical features 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。nullTumours have been described to be 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 null五、Aetiology 病因学 Unknown 病因不明 It has been postulated that myofibroblastomas arising in the breast may be related to a patient’s hormonal status,in that lesions are relatively common in older men .e.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。 null 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 putative 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。 null六、Macroscopy 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5.8cm The tumour are well circumsribed and firm. 界清，质硬 The colour can be variable(white,pink,tan or brown) 色泽多变，可为白色，粉红色，，黝黑色棕褐色 nullThe 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。 null七 、Histopathology 组织学 Tumours are unecapsulated but well circumscribed . 肿瘤无包膜，但是分界清楚 They are composed 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 nullThe 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 chromatin，small nucleoli 核内染色质均匀散布，常见小核仁 nullEosinophilic 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 nullThe stroma is collegenous with broad bands 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，伴核增大，多核 nullSuch 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。 nullThe 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。null in contrast to the 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 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。 null八、Immunophenotype 免疫型 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形细胞弥漫性，双表达desmin （肌间线蛋白）和CD34 Expression of smooth muscle actin is seen in a third of case 1/3的梭形细胞表达 SMA(平滑肌动蛋白)。 null九、Prognostic factors 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）null九、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）nullnullnullnullnullnull