萎缩性隆突性皮肤纤维肉瘤5例临床及组织病理分析

目的:探讨萎缩性隆突性皮肤纤维肉瘤(DFSP)的临床及组织病理特征。方法:对西京皮肤医院确诊的5例萎缩性DFSP进行临床及组织病理学分析。结果:萎缩性DFSP主要表现为缓慢生长的界限清楚的萎缩性斑块,部分皮损可伴有皮下结节或外生性结节。临床上萎缩性DFSP容易误诊,其中4例误诊为硬斑病。萎缩性DFSP组织病理表现为真皮明显变薄,真皮内肿瘤细胞不形成典型的席纹状排列,而是平行于表皮,肿瘤细胞可以不浸润脂肪组织。免疫组化显示肿瘤细胞CD34阳性,CD68、S-100蛋白阴性。结论:相对于经典型DFSP而言,萎缩型DFSP的皮损不呈经典的结节性改变,而是表现为萎缩斑,容易误诊。组织病理表现为真皮萎缩,肿瘤细胞不呈席纹状排列,容易误诊为瘢痕或其他纤维细胞肿瘤。     

36例乳房外Paget病临床特点分析

 目的:对乳房外Paget病进行回顾性研究，为临床诊治提供帮助。方法：对2013年1月至2018年12月我院确诊的36例乳房外Paget病患者的临床资料进行回顾性分析。结果：36例患者男女比例为3 ∶1；平均发病年龄(65.72±10.41)岁；病程6个月至10年，中位病程4年；皮损多为单发，阴囊常见；主要表现为红斑、糜烂；既往误诊率60.61%；免疫组化以CK7、CEA、EMA阳性表达为主；不伴有转移者30例，伴有转移者6例；30例行局部扩大切除术+术中冰冻治疗，其中27例扩切范围明确，扩大切除范围以1.0 cm、2.0 cm为主，扩切2.0 cm的一次切净率为90.91%。术后有2例复发。结论：该病男性好发，老年多见，皮损多为单发，误诊率高，免疫组化有提示原发或继发意义，病情进展缓慢但仍可发生转移，治疗以局部扩大切除术+术中冰冻为主，建议扩切范围在2.0 cm及以上。该病术后仍有较高复发率。     

萎缩型隆凸性皮肤纤维肉瘤

报告2例萎缩型隆凸性皮肤纤维肉瘤。例1男,42岁。腹股沟斑块4年,新生结节3个月;例2男,47岁。左胸部结节10年余。皮损组织病理检查、免疫组化及弹性纤维染色证实两例患者均为萎缩型隆凸性皮肤纤维肉瘤。     

冷冻、二氧化碳激光治疗28例晕痣临床观察

我们于2001年1月~2004年1月采用液氮冷冻、二氧化碳激光治疗28例晕痣,计40片皮损,并随访观察0.5~2年,现报道如下。临床资料28例患者均来自本所白癜风专科门诊,男15例,女13例,年龄6~40岁,其中6~14岁12例,15~20岁11例,21~40岁5例,病程1周至半年,平均2.5个月;晕痣数目:28例患者中,1片皮损者20例,2片皮损者5例,3片皮损者2例,4片皮损者1例;晕痣分布部位:面部8片,颈部15片,胸部10片,背部2片,两前臂5片;皮损特点:本病为圆形或椭圆形边缘清楚的白斑,中央有一黑痣,白斑大小直径0.5~2 cm,黑痣大小直径0.1~0.5 cm,稍高出皮面呈半球形损害。28例患者均无…     

自血局部注射治疗局限型白癜风54例

我们应用自血局部注射治疗局限型白癜风5 4例,取得较好效果,报道如下。临床资料　5 4例白癜风患者来自2 0 0 0年3月～2 0 0 3年2月我院皮肤科门诊,男32例,女2 2例,年龄16～4 8岁,平均2 4 .6岁;病程3周～6年,病程<6个月者8例,1～2年者2 6例,>3年者2 0例,平均病程1.6年。皮损发生于面颈部者2 0例、手背部11例、肩及胸背部8例、下腹部9例、四肢6例。以患者最大皮损面积直径分组:皮损面积直径<3cm 12例、3～5cm 2 1例、5 .1～8cm 12例、8cm以上9例。皮损数目1块14例、2～5块2 8例、6～8块12例。治疗方法:皮损处碘伏消毒,用5ml注射器于患者前臂抽…     

先天性黏液型隆突性皮肤纤维肉瘤一例并文献复习

先天性DFSP临床罕见,其临床表现可类似血管性病变。黏液型先天性隆突性皮肤纤维肉瘤(congenital dermatofibrosarcoma protuberans,DFSP)是DFSP独特而罕见的亚型,具有其特征性病理组织学改变,生物学行为属交界恶性/潜在低度恶性。先天性黏液型DFSP更为罕见,国外仅有2例散发病例报道。本文报道一例并回顾相关文献。患儿,女,1岁9个月。出生后即有背部肿物,曾于外院误诊为血管瘤,予盐酸噻吗诺尔眼药水外敷治疗1年余,效果不佳。肿物不断增大,就诊我科后,予手术切除肿物并行病理组织学及免疫组织化学检查,诊断为先天性黏液型隆突性皮肤纤维肉瘤。考虑病理回报底切缘最近距离仅0.25 mm,根据指南行肿瘤边缘1 cm扩大切除治疗,随访2年未发现复发及转移。     

萎缩性隆突性皮肤纤维肉瘤3例临床病理分析

目的分析萎缩性隆突性皮肤纤维肉瘤(DFSP)的临床病理特征,并应用二代测序技术探索新的融合基因检测方法。方法回顾性分析本院诊断的3例萎缩性DFSP的临床特征、组织病理学特点及其免疫组织化学染色结果,并运用目标捕获技术对其中1例出现结节的DFSP病例进行融合基因检测。结果萎缩性DFSP临床主要表现为界限清楚的萎缩性红斑,可逐渐在红斑基础上发生结节。组织病理学检查示:肿瘤位于真皮层,由类似良性的梭形细胞组成,呈不规则条索状排列,不形成典型的席纹状结构。免疫组织化学染色:肿瘤细胞CD34弥漫阳性,不表达CD68和S-100。融合基因检测出17号染色体的COL1A1基因的31号内含子和22号染色的PDGFB基因的1号内含子发生断裂,形成融合基因。结论萎缩性DFSP的皮损不呈经典型DFSP的隆凸状外观,而是表现为萎缩斑或硬化斑,且组织病理表现为肿瘤细胞不呈典型席纹状排列,可不浸润至皮下脂肪组织,容易误诊。以二代测序为基础的融合基因检测技术,可以精准发现基因的断裂点,从而提高DFSP诊断率。     

皮肤混合瘤43例临床病理分析

目的探讨皮肤混合瘤的临床、病理特征及治疗方法。方法回顾性分析本院2013-2017年43例皮肤混合瘤患者的临床表现、误诊情况、病理表现、治疗和转归等资料。结果 43例皮肤混合瘤患者中男27例,女16例;皮损均为单发,临床表现为直径1.5~3.1cm半球形皮下结节;皮损分布于鼻部最多,为22例;初诊误诊43例(100.00%),43例均行手术切除,随访1~4年,无复发。结论皮肤混合瘤临床表现及发病部位有一定特征,完整切除为其主要治疗方法。     

深在型环状肉芽肿20例临床及组织病理分析

目的:探讨深在型环状肉芽肿临床及组织病理特征。方法:对2009年12月—2014年9月间西京皮肤医院经组织病理确诊的20例深在型环状肉芽肿患儿资料进行回顾性分析。结果:20例患儿中男9例,女11例,年龄1~12岁,平均3.2岁,病程1个月~5年。临床表现均为皮下结节。组织病理均表现为皮下脂肪内上皮样细胞形成栅栏状肉芽肿性结节,20例均可见黏蛋白沉积,18例可见纤维蛋白样坏死,10例可见不同程度的嗜酸性粒细胞浸润。对所有患儿进行电话随访,时间为4个月~5年,其中6例皮损完全消退,6例有新发皮损,1例皮损未见明显变化,3例失访。结论:深在型环状肉芽肿主要发生于儿童,表现为头部或四肢的皮下结节。组织病理表现为皮下脂肪栅栏状上皮样细胞肉芽肿,可见纤维蛋白样坏死和黏液沉积,常伴有嗜酸性粒细胞浸润。     

麻风病人皮肤及神经内肉芽肿的组织学比较

选择有明显皮损及神经粗大的22例病人,取1.5cm长的皮下神经分支和皮损标本。用HE和Fite—Faraco染色,其临床分型为TT—2,BT—14,BB—1,LL—3和I—l,病期6个月～15年,除一例BL用DDS治疗2年外都未经治疗。结果发现皮损与神经的病理分型相符的14例,分别为TT—1,BT—9,BB—0,BL—1,LL—3,其中BT型病人神经内的肉芽肿及干酪样坏死均较皮损内的多,而皮损内的巨细胞较神经内多,其他病理特征相同;4例BL/LL病人有2例神经内的BI高于皮损,但组织学图像类似。皮损与神经病理分型不相符的8例病人     

Dermatofibrosarcoma protuberans association with nuchal-type fibroma

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a hypercellular, storiform, CD34-positive low-grade sarcoma with honeycomb entrapment of fat, which typically involves the trunk and extremities. Nuchal-type fibroma (NTF ) is a paucicellular, CD34-positive fibrous tumor with fat entrapment, which may occur in both nuchal and extranuchal locations and in association with Gardner syndrome. METHODS: We report the association of DFSP with NTF in a 43-year-old male with no personal or family history of Gardner syndrome. RESULTS: The patient had a past history of a DFSP removed from his back, which recurred 2 years later and was re-excised. Additionally, the patient had a typical NTF, in the posterior neck, removed at the same time. Histopathologic examination of the recurrent back lesion demonstrated a composite lesion with typical appearances of DFSP, centrally, blending into an NTF-like appearance, peripherally. Both components expressed CD34 and CD99, and lacked elastin. A review of the microscopic slides of the patient's previously excised DFSP revealed an identical lesion with surrounding NTF-like areas. CONCLUSION: While an association between NTF and fibromatosis has recently been reported, this is to our knowledge the first report of an association between NTF and DFSP. The morphologic findings suggest that there may be a continuum between these two CD34-positive lesions that have a tendency to infiltrate adipose tissue and recur.     

Improving precision of resection by pre‐surgery inspections with contrast‐enhanced ultrasound for dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon malignant soft tissue tumor. Although metastasis is rare, it has significant topo‐aggressive characteristics with a high recurrence rate. The common treatment of DFSP is surgical resection. This study reported a case of a 22 years old woman who presented DFSP with single irregular red plaque in the right occipital area. The plaque is about 2 cm × 2 cm in diameter with an irregular and unclear border. In order to minimize the range of resection area, the contrast‐enhanced ultrasound (CEUS) was used to precisely identify the tumor affected area before the surgery treatment. The result shows that CEUS depicted clear structure of the tumor, which successfully helped surgeons to reduce the resection range from 3 cm to 2 cm in diameter. The tumor has been completely removed by one surgery operation only, and no relapse was found up to now. Pre‐surgery CEUS inspection of DFSP could be an effective and economical method to minimize the patient's hurts in the surgical treatment of DFSP.     

Report of Two Cases of Recurrent Scalp Dermatofibrosarcoma Protuberans and Literature Review

Dermatofibrosarcoma protuberans (DFSP) are rare malignant skin tumor, and scalp DFSP is even lesser than 5% of all DFSP, therefore, being seldom reported. We recently treated two cases of recurrent scalp DFSPs. One was a 38-year-old male, who accept lumpectomy for the first time; however, it recurred 9 months later. We then performed a wide excision resulting in no recurrence in the subsequent 4 years. Another patient was a 26-year-old female, who accept an in situ tumorectomy for the first time, and 2 years later; the recurrent mass became 9 × 9 cm in size. We gave her another operation, but only 3 months later local recurrence appeared. For the both cases, we collected their case histories, intraoperative findings, pathologic detections, and follow-up results, all of which may help the dermatologists to extend knowledge about this rare disease. Moreover, an exhaustive review of the literature is included with emphasis on diagnosis, different diagnosis and treatments.     

Giant cell fibroblastoma in a child misdiagnosed as a dermatofibroma

We report a 9-year-old African-American boy with a giant cell fibroblastoma of the shoulder that was incorrectly diagnosed as a keloid and dermatofibroma. Initial misdiagnosis led to a delay of 4 years in the correct diagnosis, with the tumor producing significant local destruction. We review herein the clinical manifestations, histologic findings, histogenesis, relationship to dermatofibrosarcoma protuberans (DFSP), treatment, and differential diagnosis of giant cell fibroblastoma (GCF). This information is important in correctly diagnosing this uncommon, benign, but locally aggressive and recurrent tumor of childhood. The clinician should consider GCF and DFSP when the pathologic diagnosis of dermatofibroma is made in lesions more than 2 cm in diameter, or when this diagnosis is made in a prepubertal child.     

Benign cellular fibrous histiocytoma with erosion of the phalanx

Fibrohistiocytic tumors are characterized by the presence of fibroblast like spindle cells and histiocytes. The benign fibrous histiocytoma (dermatofibroma, BFH) as well as the malignant dermatofibrosarcoma protuberans (DFSP) and the malignant fibrous histiocytoma (MFH) belong to this group. A recurrent painful, hard 2 cm tumor on the left hallux of a 54-year-old woman led to an erosion of the underlying phalanx. The patient had suffered from ingrown toenails for more than 10 years. Histologically there was a deep penetrating fibrohistiocytic tumor that grew in a storiform pattern with interspersed foam cells. The tumor was CD34 negative and mitoses were scarce. The diagnosis was benign cellular fibrous histiocytoma (BZFH). BZFH belong to the group of BFH with a high recurrence rate especially after incomplete removal. Damage to the underlying bone has not been reported so far.     

Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

Recurrent Dermatofibrosarcoma Protuberans with Myxoid and Fibrosarcomatous Changes Paralleled by Loss of CD34 Expression

A 38-year-old woman complained of recurrent nodules on her anterior chest wall. She noticed the first nodule at the age of 12 and has since undergone surgical removal a total of 31 times. Finally, wide resection of parts of the rib cartilage and sternum and chest wall reconstruction were required. Histopathological examination of a series of surgical specimens were reevaluated. Immunohistochemical staining of the tumors by anti-CD34 antibody provided a definite diagnosis of dermatofibrosarcoma protuberans (DFSP). A noteworthy result was that her DFSP did not always express CD34; the recurrent tumors with myxoid and fibrosarcomatous changes lost their CD34 expression.