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。     

血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错分布的细胞密集区和细胞稀疏区组成，其间血管丰富，多为毛细血管至中等大薄壁血管。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。     

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…     

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。     

外阴血管肌纤维母细胞瘤三例

我院妇产科于 1997～ 1998年收治 3例外阴的软组织肿瘤 ,其中 2例行术中冷冻切片检查 ,手术切除标本常规 4%甲醛固定 ,石蜡包埋 ,3μｍ连续切片 ,除ＨＥ染色外 ,又进行了数种抗体的免疫组化染色 (ＬＳＡＢ法 )。其中 1例取少量新鲜瘤组织迅速放入 2 5 %缓冲戊二醛中固定 ,送透射电镜检查 ,3例均取得随访结果。 3例女性外阴血管肌纤维母细胞瘤的主要临床特点如表 1。病理检查 :其中 2例行冷冻切片检查 ,3例行免疫组织化学染色 ,1例取少量新鲜瘤组织行透射电镜检查。肿物表面光滑 ,有一层菲薄的纤维性包膜。肿物剖面粉红 ,隐约可见微细血管 ,…     

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3例并文献复习

目的 探讨血管肌纤维母细胞瘤(angiomyfibroblastoma,AMF)的临床病理特征、诊断和鉴别诊断.方法 对3例AMF进行组织学观察和免疫组化标记,抗体为vimentin、SMA、MSA、ER、PR、CD34、desmin、S-100蛋白和CK.结果 3例均表现为外阴部肿块.眼观:肿瘤境界均清楚;镜检:肿瘤均由相互交替分布的细胞密集区和细胞稀疏区组成,其间血管丰富,多为毛细血管至中等大薄壁血管.瘤细胞呈巢状或束状围绕血管排列.免疫表型:瘤细胞vimentin、SMA、ER和PR均阳性,CD34在血管壁呈阳性、瘤细胞阴性,MSA、desmin、S-100蛋白和CK均阴性.结论 AMF是一种少见的好发于外阴的良性软组织肿瘤,需与侵袭性血管黏液瘤、富细胞性血管纤维瘤等鉴别.     

血管肌纤维母细胞瘤与侵袭性血管粘液瘤临床病理分析

目的：探讨血管肌纤维母细胞瘤（ＡＭＦＢ）的临床病理特点及与侵袭性血管粘液瘤（ＡＡ）的鉴别。方法：对５例ＡＭＦＢ和５例ＡＡ进行临床病理和免疫组化研究,对３例ＡＭＦＢ进行电镜观察。结果：ＡＭＦＢ位于外阴或腹股沟我,肿瘤边界清楚,大小０．８～４ｃｍ。光镜：肿瘤细胞呈梭形上皮样、束头及巢状排列,常围绕小至中等大小的薄壁血管周围。肿瘤有细胞密集区和细胞分散区。免疫组化：肿瘤细胞表达ｖｉｍｅｎｔｉｎ,ｄｅｓｍ     

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…     

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。     

Cytokeratin immunolocalization and lectin binding studies in oesophageal squamous dysplasia

Aggressive angiomyxoma is a distinctive soft tissue tumour associated with a high risk of local recurrence but lacks metastatic potential. This tumour occurs nearly exclusively in the soft tissues of the pelvis and perineum of adult women. The line of differentiation is not firmly established, but a fibroblastic/myofibroblastic origin has been proposed. We report 16 new cases of aggressive angiomyxoma of the pelvic soft tissue in women. In all cases bundles of cells, most often adjacent to vessels, with histological features of smooth muscle cells were identified. In 11 of 14 cases the myoid bundles were immunoreactive for desmin; they were also postive for smooth muscle actin in 10 of 11 cases. In 13 of 14 cases lesional stromal cells showed immunoreactivity for desmin. Three cases showed areas with histological features similar to those of angiomyofibroblastoma of the vulva, thus representing previously undescribed morphological overlap between these two entities. We conclude that aggressive angiomyxoma and angiomyofibroblastoma are related neoplasms in a spectrum of tumours showing myofibroblastic origin. Furthermore, the demonstration of immunoreactivity for desmin in aggressive angiomyxomas implies that this antibody is not helpful in discriminating between these two tumours, and the principal means of distinction remains histomorphological analysis.     

Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study

To investigate the histogenetical unifying theory of a single, pluripotential primitive cell for vulvar angiomyxoma, aggresive angiomyxoma, and angiomyofibroblastoma, an optical, immunohistochemical and ultrastructural study of a superficial angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma was performed. These three tumors showed immunohistochemical and ultrastructural overlapping features. The results of the study suggest that these three tumor entities probably arise on a common pluripotential primitive cell located around the vessels of connective tissue, which could show the capacity for modulating its penotype toward similar but distinct mature cell types.     

Angiomyofibroblastoma of the vulva: A clinicopathologic study of seven cases

A clinicopathologic and immunohistochemical review was made of seven cases of angiomyofibroblastoma. The patients were middle-aged women who had a slowly growing mass, measuring 1.5–6 cm in maximum dimension, located sub-cutaneously in the vulva. The tumors were well-demarcated and characterized by well-vascularized, alternating hyper-cellular and hypocellular edematous areas composed of bland, plump spindle- or oval-shaped stromal cells frequently aggregated around small blood vessels. An epithelioid appearance of the stromal cells was seen in two cases. Immunohistochemically, the stromal cells were consistently positive for vimentin and desmin, but negative for muscle specific actin, a-smooth muscle actin, myosin, cytokeratins, S-100 protein or von Willebrand factor. Ultrastructurally, the plump stromal cells had a small amount of peripherally located rough endoplasmic reticulum, numerous pinocytotic vesicles and abundant intermediate filaments, on which immunogold probes for desmin were localized, whereas fine filaments were few and there were no electron dense plaques. Thus, while the proliferating stromal cells expressed an immunohistochemical profile of peculiar myoid differentiation, ultrastructural findings differed from those of smooth muscle cells or those seen in typical myofibroblasts. At 1–4 years after surgery, there was no evidence of recurrence.     

Angiomyofibroblastoma of the vulva: A mitotically active variant?

A case of angbmyofibrobiastoma in a 48-yearold woman is reported. The tumor occurred as a left vulval mass and was treated by simple excision. It was located in the subcutaneous tissue of the left vuiva and was well circumscribed, measuring 2.8 × 2.7 × 2.5 cm. Microscop Ically, the tumor was composed of hypocellular and cellular arees with well-developed small vessels. Spindle or polygonal cells were arranged with perlvascular accentuation In an edematous or fibrocollagenous background. Some spindle-shaped or polygonal stromal cells were also arranged in epithelioid nests. In some areas, mitosas were frequent (maximum 3/10 high-power field). lmmunohistochemicaily, the stromal cells were positive for vimentln and desmin, but negative for α-smooth muscle actin, S-100, neurofilament, estrogen receptor, progesterone remptor, CD31 and CD34. The average labeling index of Kl-67 In stromal cells was 3.1%. Ultrastructural analysis demonstrated that the stromal cells adhered with primitive Junctions and contained lntermediate filaments with no focal density In the cytoplasm. These findings were consistent with anglomyofibrobiastoma, although previously reported cases did not show so many mitoses. Therefore, this case was suggested to be a mitotically active variant.     

Myxoid leiomyoma of the vulva mimicking aggressive angiomyxoma

A case of vulvar leiomyoma with extensive myxoid change in a 40 year old female is described. The tumor had a unique connection with a non-degenerative leiomyoma that compressed the rectum and the bladder. Scattered smooth muscle cells in a loose myxoid stroma were immunoreactive for desmin. Fibroblast-like spindle cells were immunoreactive for vimentin but not for desmin. The initial, although incorrect, pathological diagnosis of the tumor was aggressive angiomyxoma based on the similarity in both clinical and pathological aspects with this more invasive tumor. Myxoid vulvar leiomyoma should also be differentiated from angio-myoflbroblastoma. The key to the differential diagnosis is the presence of interlacing smooth muscle cells and an awareness of tendency toward myxoid change in vulvar leiomyomas.     

肺黏液表皮样癌12例临床病理分析

目的 探讨肺黏液表皮样癌(pulmonary mucoepidermoid carcinoma,PMC)临床与病理学特征、诊断与鉴别诊断、治疗及预后.方法 对12例PMC进行组织形态学和免疫组织化学检查,结合文献复习进行分析并随访.结果 12例PMC中7例行支气管镜活检显示黏膜下肿物,呈息肉样突向管腔,部分表面可见破溃,界限较清.病理形态显示12例PMC均由黏液细胞、鳞状细胞和中间型细胞构成,细胞分化程度较高,PAS、AB-PAS染色阳性.免疫组化染色示肿瘤细胞CAM5.2、HCK和p63均(+),Ki-67增殖指数为10%左右.12例均行手术治疗,均未见淋巴结转移,随访其中10例均无复发或死亡.结论 PMC较少见,为低度恶性肿瘤.临床症状无特异性,容易误诊.支气管镜活检有助于早期诊断,手术切除预后较好.     

Clinicopathological features and differential diagnosis of chondrogenic tumours

Chondrogenic or cartilaginous tumours represent the most common bone tumours and can be diagnostically challenging especially on scarce biopsy material. As morphologically they may overlap, correlation between radiological and pathological findings and discussion in a multidisciplinary tumour board is mandatory for a final diagnosis. The aim of this review is to discuss clinical and pathological features that are important in the differential diagnosis of benign (chondromesenchymal hamartoma of chest wall, osteochondroma, enchondroma, periosteal chondroma) and intermediate (synovial chondromatosis, central or secondary peripheral atypical cartilaginous tumour) or malignant cartilaginous tumours (central or secondary peripheral chondrosarcoma grades 1–3, periosteal chondrosarcoma, dedifferentiated chondrosarcoma, clear cell chondrosarcoma and mesenchymal chondrosarcoma). The 5^th edition WHO classification proposed a new concept for cartilage tumours in which the term atypical cartilaginous tumour is used for low grade tumours located in the long and short tubular bones of the appendicular skeleton, as they behave as locally aggressive lesions and therefore should not be considered full blown malignant or treated aggressively. Histologically similar tumours in the axial skeleton (including pelvis, scapula and skull base) are now diagnosed as chondrosarcoma grade 1. Immunohistochemistry is of limited value in the distinction between the different cartilaginous tumours. Molecular diagnostics can be helpful when used on small biopsies to distinguish mesenchymal chondrosarcoma (with HEY1-NCOA2 fusion) from other undifferentiated round cell sarcomas, or dedifferentiated chondrosarcoma (often with IDH mutation) from other high grade sarcomas.