黏液性隆突性皮肤纤维肉瘤

报告1例黏液性隆突性皮肤纤维肉瘤.患者男,64岁.左下肢膝关节部位出现肿块30年.皮肤科检查:左侧膝关节部佗可见一直径约10 cm大肿块,表面有大小不一的结节,并有糜烂和溃疡.肿块边界不规则,向周围正常组织浸润.皮损组织病理检查:真皮内弥漫性梭形细胞增生,细胞间隙明显增大.阿新蓝染色示大量黏蛋白沉积;免疫组化染色结果示CD34阳性.诊断:黏液性隆突性皮肤纤维肉瘤.     

检测COL1A1-PDGFB融合基因协助诊断萎缩型隆突性皮肤纤维肉瘤

报告1例萎缩型隆突性皮肤纤维肉瘤。患者女,24岁。患者2年前无明显诱因胸部皮肤出现红斑,逐渐变大,中央萎缩。皮肤科检查:胸部一3 cm×4 cm暗紫红色近圆形斑块,表面光滑,中央萎缩,触之质地韧,无凸起包块,无触痛。皮损组织病理检查:表皮轻度乳头瘤样增生,真皮萎缩变薄,真皮及皮下脂肪可见短梭形细胞增生,增生细胞和表皮之间可见肿瘤细胞无浸润带;真皮浅、中层区域肿瘤细胞数目较少,排列稍稀疏,细胞呈波浪状排列,平行于表皮;真皮深部和皮下脂肪层可见密集排列的梭形细胞增生,梭形细胞密集排列呈车幅状、席纹状或束状。免疫组化示肿瘤细胞强阳性表达CD34。采用反转录-聚合酶链式反应(RT-PCR)检测COL1A1-PDGFB融合基因阳性。诊断:萎缩型隆突性皮肤纤维肉瘤。     

一家三口同患羊痘

报告一家三口同患羊痘。例1.患者男,41岁。有病羊接触史,左手背及右前臂脓疱、结节伴痛痒2周。皮肤科检查：左手背一直径约1 cm乳头瘤样结节;右前臂可见数个直径1～1.5 cm扁平脓疱。皮肤镜检查：镜下可见皮损中央溃疡结痂区及周围黄白色环状区域,可见点状血管。皮损组织病理检查：表皮细胞内及细胞间水肿、空泡形成及气球状变性;真皮血管内皮细胞和表皮细胞胞质可见嗜酸性包涵体。例2和例3患者分别为例1父亲及母亲,皮肤科检查与例1类似。例2发病前有病羊接触史,例3否认发病前有病羊接触史。3例患者结合典型临床表现及皮肤镜、组织病理检查结果,均诊断为羊痘。     

幼年隆突性皮肤纤维肉瘤

报告1例幼年隆突性皮肤纤维肉瘤。患儿男,8岁。左侧腹股沟皮肤红色结节5个月。皮肤科检查：左侧腹股沟皮肤见一花生米大的半球形粉红色结节,表面光滑,境界清楚,结节质中等,与皮肤粘连,浸润感明显。皮损组织病理检查：表皮基本正常,真皮有增生的梭形细胞组成的细胞团块,部分梭形细胞呈特征性席纹状排列,未见明显细胞异形及核分裂象。免疫组化：增殖核抗原（Ki-67）(5%+),CD34(+),S-100蛋白（-）。分子生物学检测：COL1A1-PDGFB融合基因比例70%。诊断：隆突性皮肤纤维肉瘤。治疗上予扩大切除术辅以术后放疗,随访无复发。     

色素性隆突性皮肤纤维肉瘤

报告1例色素性隆突性皮肤纤维肉瘤患者.患者男,30岁.左上臂蓝色斑块半年就诊.皮肤科检查:左上臂三角肌处一蓝红色斑块,表面轻微萎缩凹陷.皮损组织病理检查:真皮内有一瘤细胞团块,细胞核呈梭形,核大、深染,无明显异形,排列成车辐状或条索状,含黑素的树突状细胞不规则分布于瘤细胞之间.免疫组化染色示CD34(+),波形蛋白(vimentin)(+).诊断为色素性隆突性皮肤纤维肉瘤.     

儿童隆突性皮肤纤维肉瘤

报告1例儿童隆突性皮肤纤维肉瘤。患儿女,6岁。右上肢斑块1年。皮肤科检查：右上肢一蓝红色斑块,表面轻度萎缩凹陷。皮损组织病理：表皮及真皮萎缩,真皮和皮下脂肪层可见形态和大小均匀一致的梭形细胞形成的肿瘤细胞团块,深入和分割皮下脂肪,形成特征性蜂窝和花边表现,细胞异形性不明显,少许核分裂象,树突状细胞不规则分布于瘤细胞间。免疫组化示CD34(+),S-100蛋白（-）,HMB45(-),melan-A（-）,白细胞共同抗原（LCA）（-）,角蛋白（CK）(-),CD1a（-）。诊断：儿童皮肤纤维肉瘤。治疗：肿瘤扩大切除术,术后随访1年未复发。     

纤维肉瘤型隆突性皮肤纤维肉瘤一例

摘要] 报告1例纤维肉瘤型隆突性皮肤纤维肉瘤(DFSP-FS)。患者女,36岁。背部反复丘疹、结节12年。曾经2次手术切除。组织病理检查示瘤细胞排列呈涡纹状,形态较一致,呈梭形,部分区域内瘤细胞排列成鱼骨状,异型性及分裂相均较前者为高。免疫组化检查示涡纹状结构区域内瘤细胞CD34弥漫强阳性,鱼骨状区域仅个别细胞CD34表达阳性。诊断：纤维肉瘤型隆突性皮肤纤维肉瘤。行广泛切除术,术后9个月,未见复发。     

隆凸性皮肤纤维肉瘤

报告2例隆凸性皮肤纤维肉瘤。例1.女,8岁。右侧大腿结节6个月。皮肤科检查:右侧大腿内侧一红色结节,表面光滑,无溃疡,质韧,活动度一般,与周围组织分界不清,无触压痛。皮损组织病理:真皮内见大量致密分布的梭形细胞呈席纹样排列,局部向脂肪组织浸润,并见散在核分裂象。免疫病理:梭形肿瘤细胞CD34表达呈强阳性。诊断:儿童隆凸性皮肤纤维肉瘤。予局部扩大切除肿瘤,术后随访至今9个月未见复发或转移。例2.男,14岁。右侧胸腹部皮肤肿块14年。皮肤科检查:胸腹部右侧一红褐色浸润性肿块,表面光滑,质韧,与周围组织分界不清。皮损组织病理:表皮大致正常,真皮内大量梭形肿瘤细胞浸润,局部呈漩涡状排列,界限不清,并不规则地向皮下脂肪侵袭。免疫病理:梭形肿瘤细胞CD34表达呈强阳性。诊断:隆凸性皮肤纤维肉瘤。予Mohs显微外科手术切除肿瘤,局部皮瓣转移修复,术后随访至今35个月未见复发或转移。     

皮肤利什曼病

报告1例皮肤利什曼病。患者男,44岁。左上肢及左腹部红斑、丘疹及溃疡伴疼痛10个月。皮肤科检查:左腹部一直径约12 cm斑片,中央可见直径5~6 cm溃疡,表面覆黄色较厚痂壳,可见大量浆液性渗出;左前臂一5 cm×8 cm斑片,中央一手掌大溃疡,表面覆有黑褐色及黄色痂壳,未见明显渗出。皮损组织病理检查:表皮大致正常,真皮浅层可见大量组织细胞浸润,细胞内可见大量利什曼小体样结构。诊断:皮肤利什曼病。     

上皮样肉瘤1例

患者男,35岁。左上肢结节伴疼痛4个月。皮损组织病理示：表皮无明显变化,真皮内瘤细胞呈栅栏状排列,其中央可见坏死的黏蛋白沉积。瘤细胞呈梭形,少数有明显异型,未见上皮样细胞。免疫组化染色示：肿瘤细胞表达上皮膜抗原（EMA）、细胞角蛋白（CK）、细胞角蛋白18（CK18）、波形蛋白（Vimetin）和CD34,不表达CD68和结蛋白。     

色素性神经纤维瘤六例

6例色素性神经纤维瘤中男3例,女3例,年龄8～55岁.6例患者的皮损均表现为褐色斑片或斑块,3例皮损位于左颈部及躯干、四肢,1例皮损位于右腋窝及左上臂,1例皮损位于左前臂伸侧,1例皮损位于腰臀部,皮损2 cm×3 cm至30 cm×40 cm.3例合并Ⅰ型神经纤维瘤病.皮损组织病理主要表现为表皮轻度角化过度,基底层色素增加,真皮和皮下脂肪组织内可见肿瘤细胞呈弥漫性分布,肿瘤组织无包膜,主要由大量的梭形细胞构成.肿瘤组织中散在或簇集状分布黑素细胞,胞质内可见黑素颗粒.免疫组化显示S100(+)和波形蛋白(+).6例患者均未治疗.色素性神经纤维瘤少见,临床和组织病理上,需与Becker痣、先天性黑素细胞痣、色素性隆突性皮肤纤维肉瘤等鉴别.     

Mycobacterial spindle cell pseudotumor of the skin

BACKGROUND: Mycobacterial spindle cell pseudotumor is a rare tumor-like lesion characterized by the proliferation of spindle cells engorged with mycobacterial microorganisms. To our knowledge, only a few cases of cutaneous lesions have been described in the literature. CASE REPORT: A 58-year-old Japanese woman presented with a nodule in her left forearm. She had systemic lupus erythematosus and interstitial pneumonia for 17 years, treated with prednisolone and azathioprine. She was also receiving insulin treatment for diabetes mellitus. RESULTS: Histologically, the lesion was located in the deep dermis with extension into the subcutaneous fat tissue and was composed mainly of spindle cells focally showing a vaguely storiform pattern. In limited focal areas, foam cells, epithelioid histiocytes, and multinucleated giant cells were seen sparsely. Ziehl-Neelsen staining showed numerous acid-fast bacilli within the spindle cells and epithelioid histiocytes. The acid-fast bacilli were determined by culture and polymerase chain reaction to be Mycobacterium intracellulare. CONCLUSIONS: We emphasize that mycobacterial spindle cell pseudotumor should be included in the differential diagnoses of a spindle cell lesion in the skin, especially in immunosuppressed patients.     

肢端纤维软骨黏液样肿瘤1例

【摘要】 患者男,44岁,左手小指皮下结节3年。皮肤科检查：左手小指末节背侧一0.4 cm × 0.4 cm皮下结节,触之橡皮样硬度,无压痛,末端指间关节活动不受限。术后组织病理：肿瘤含有丰富的纤维样、软骨样、黏液样间质。肿瘤细胞呈卵圆形至短梭形,核仁不明显,未见有丝分裂。细胞排列杂乱或呈小团簇状。免疫组化：肿瘤细胞表达波形蛋白、CD34、转录因子ERG、SOX9,不表达S100、P63、广谱角蛋白（AE1/AE3）、上皮膜抗原、平滑肌肌动蛋白、结蛋白,Ki67 增殖指数小于1%。诊断：肢端纤维软骨黏液样肿瘤。     

肌周皮细胞瘤1例

 报告肌周皮细胞瘤1例。患者女,54岁,因“左前臂皮下肿物1年余,缓慢增大1个月”就诊。皮肤科检查：左侧前臂见一隆起性结节,约1.0 cm×0.5 cm×0.2 cm大小,质软,边界清楚,与周围组织无粘连,表面皮肤完整,无红肿、破溃、糜烂,压痛阳性,未触及血管搏动。皮损组织病理：真皮内见大量椭圆形和梭形肌样细胞团,血管呈裂隙样,分枝状增生,瘤细胞呈同心圆状围绕血管生长。免疫组化:SMA （+）、desmin（+）、CD34(血管+)、D2 40(-)、Ki 67(<5%)。结合临床表现、组织病理及免疫组化结果,诊断为肌周皮细胞瘤。予手术切除,随访1年未见复发。     

Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceus

A 54-year-old man presented with a recurrent swelling on the right occipital region of the scalp. Two months earlier, the patient had undergone an initial local excision of the lesion which had enlarged progressively over the previous 2 years on a hairless patch which had been present since birth. On examination, a 5 x 4-cm, pinkish, firm, ulcerated swelling was seen on the right occipital region with a scar running over it. The lesion was not fixed to the underlying bone and there was no regional lymphadenopathy. X-Ray of the skull was normal and no evidence of metastatic disease was identified. Wide local excision of the tumor was performed and it was sent for histopathologic examination. Specimens and slides of the earlier surgery performed elsewhere were also studied. The specimen of the initial surgery consisted of skin-covered tissue with an exophytic firm growth measuring 6 x 5 x 4 cm. The skin surface was rough with areas of ulceration. No necrosis was noted grossly. Microscopically, three distinct lesions were seen. One was a well-circumscribed tumor located in the superficial dermis with lobules of basaloid cell aggregates with peripheral palisading and with no epidermal connection. The lobules were surrounded by cellular fibrous tissue (Fig. 1). Unlike basal cell carcinoma, however, no cleft between the cellular aggregates and stroma was noted. Foci of pigmentation were seen within cellular lobules and these features were consistent with a diagnosis of tricho-blastoma. The second tumor was seen adjacent to the first, and consisted of duct-like structures and cystic spaces with papillary projections. These were lined by double-layered epithelium with stromal infiltration by plasma cells, which are features of syringocystadenoma papilliferum (Fig. 2). The third lesion was a spindle cell sarcoma which formed the major part of the lesion, diffusely infiltrating the dermis and subcutaneous tissue, elevating and ulcerating the overlying skin. The tumor consisted of interlacing fascicles of spindle cells with oval to elongated nuclei having finely dispersed chromatin and inconspicuous nucleoli. The tumor cells were seen encircling the sweat glands, without destroying them (Fig. 3). Nuclear pleomorphism was minimal, with a mitotic rate of 9-10 per high-power field. A small area of epidermal hyperplasia with acanthosis and papillomatosis overlying malformed highly placed sebaceous glands was the only evidence of a pre-existing nevus sebaceus. The status of the surgical margins was not clearly discernible. The wide excision specimen of the recurrent swelling consisted of a skin-covered nodule with ulceration, measuring 3 x 4 x 3 cm, with a gray-white whorled cut surface. No necrosis was noted grossly. Multiple sections showed only spindle cell sarcoma infiltrating the skin and subcutaneous tissue, morphologically similar to the earlier tumor, with ulceration of the overlying skin. The surgical margins were free from tumor. Immunohistochemical stains on the spindle cell sarcoma showed positive staining for smooth muscle actin (SMA) (Fig. 4), vimentin, epithelial membrane antigen (EMA), and S100. The tumor cells were negative for cytokeratin (CK), HMB45, desmin, glial fibrillary acidic protein (GFAP), CD34, and CD68. Correlating the histomorphology and immunohistochemical findings, a diagnosis of cutaneous leiomyosarcoma with tricho-blastoma and syringocystadenoma papilliferum arising on nevus sebaceus was made. The patient received postoperative radiotherapy and is disease free 8 months after diagnosis.     

黏液性皮肤纤维瘤

报告1例黏液性皮肤纤维瘤.患者女,43岁.右前臂丘疹2周余.皮肤科检查:右前臂一直径约0.8 cm结节.组织病理检查示表皮不规则增生,真皮内可见大量梭形细胞增生,局部胶原排列疏松,阿新蓝染色阳性.免疫组化染色结果示波形蛋白(vimentin)阳性,CD31、CD34、平滑肌肌动蛋白(SMA)和S-100蛋白阴性.诊断为黏液性皮肤纤维瘤.     

隆突性皮肤纤维肉瘤一例

患者女,52岁,左下腹皮肤红色结节、斑块10年,皮疹增多半年。体检：患者左下腹部皮肤凹凸不平,表面可见数个黄豆到指头大小的红色丘疹及结节,表面光滑,质软如橡皮样;皮下可触及多个大小不一的结节及肿块,质地中等,表面皮肤光滑呈黄红色。皮损组织病理：真皮全层和皮下组织可见大量梭形肿瘤细胞呈席纹状排列,累及皮肤附属器,部分肿瘤细胞可见少数核分裂象。免疫组化显示,肿瘤细胞CD34、波形蛋白及BCL-2阳性,NSE （-）, Syn（-）、 S-100（-）、 SMA（-）及ⅩⅢa阴性。根据临床、组织病理及免疫组化表现诊断为隆突性皮肤纤维肉瘤。采用肿物扩大根治切除术 ＋ 植皮术,疗效满意,随访5个月未复发。     

Intravascular myopericytoma

BACKGROUND: Myopericytoma is a benign tumor composed of cells that show apparent differentiation towards putative perivascular myoid cells called myopericytes. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults. METHODS: We describe a myopericytoma that was unusual in its intravascular location. RESULTS: A 54-year-old man presented with a 10-year history of a painful slowly growing 1.5-cm nodule in the subcutaneous tissue of the thigh. Histologic examination of the excised lesion showed that is was entirely contained within the lumen of a vein. It was composed of a proliferation of myoid-appearing spindle cells, which were arranged in a striking concentric pattern around numerous blood vessels, in a manner that accentuated the vessel walls. This pattern is characteristic of myopericytoma. In some areas, fascicles of spindle cells, embedded in a myxoid stroma, bulged into the lumina of lesional vessels, reminiscent of myofibroma/myofibromatosis. Lesional spindle cells were diffusely positive for smooth muscle actin, focally positive for CD34 and were negative for desmin, cytokeratin, S100 protein, HMB-45 and CD31. CONCLUSION: This case illustrates that myopericytoma can be entirely intravascular in its location.     

黏液炎性纤维母细胞肉瘤

【摘要】 患者男,73岁。左小腿红斑、斑块伴微痒2年,加重为结节、肿块伴疼痛半年余。皮肤科检查：左小腿伸侧、腓侧中下1/3处多个大小不一红斑、斑块、结节、肿块。肿块质地坚韧,基底深在,可活动,边界不清,压痛明显。肿块表面皮肤部分外观正常。组织病理学检查：表皮未见明显异常;真皮中下部及皮下脂肪层可见梭形细胞及大小不一、胞体大、胞质红染、核大、浓染不规则的细胞,呈片状分布,其中可见病理性核分裂象,间质内可见淡蓝染物质,其间杂有淋巴细胞浸润。间质阿新蓝染色阳性。免疫组化染色：梭形细胞及异形大细胞弥漫性强阳性表达波形蛋白（Vim）,30% ~ 40%瘤细胞表达Ki67,CD34和CD68部分细胞阳性,而白细胞共同抗原、人黑素瘤S100蛋白、pCK、CD31、平滑肌肌动蛋白及结蛋白均为阴性。诊断：黏液炎性纤维母细胞肉瘤。     

掌跖蕈样肉芽肿一例

患者男,41岁。手足反复脓疱和脱屑1年,左足趾肿物4个月。体检：双手拇指、食指为主及掌侧大鱼际肌部位、双足趾及双足跖前1/3处可见不规则淡红色斑片,表面有小片状白色鳞屑,局部红斑基础上见深在性小水疱、脓疱,破溃后形成点状或融合成片状浅糜烂面,表面结黄褐色痂,部分趾指甲增厚、变形明显。左足第4趾掌跖关节前端一约4 cm × 3.5 cm × 3.5 cm肿物,质稍硬,表面糜烂,伴较多脓性分泌物渗出及新生肉芽组织生长,混合后形成较厚黑色血痂,触痛明显。甲床和足趾肿物组织病理检查：表皮、真皮全层和皮下脂肪弥漫性致密小到中等大淋巴细胞浸润,细胞有异形,其间混杂少量炎性细胞,亲表皮现象明显,见Pautier微脓疡。免疫组化检查：异形淋巴样细胞LCA、波形蛋白、CD3、CD45RO、CD4、Bcl-2均阳性,CD8、CD5、CD10均为散在阳性,Ki-67 ＞ 80%阳性,CD79a、CD20、CD30、细胞角蛋白、S-100、Bcl-6、间变性淋巴瘤激酶、HMB45、CD1a、P63均为阴性。诊断：掌跖蕈样肉芽肿。