隆突性皮肤纤维肉瘤一例

患者女,27岁。右侧背部出现红斑、结节5年。组织病理检查示:表皮轻度增厚,表皮突延长,基底层色素增多,真皮中下部及皮下组织大量梭形细胞团块,核大,深染,胞质丰富,核浆比例增大,呈漩涡状排列,侵袭性生长。免疫组化结果示:CD34(+)、Ki67 0~10%(+)。诊断:隆突性皮肤纤维肉瘤。     

隆突性皮肤纤维肉瘤1例

患者女,27岁。左前胸部皮肤肿块11年,逐渐增大、增多。组织病理:真皮全层和皮下组织广泛的梭形肿瘤细胞呈束状和漩涡状排列,部分肿瘤细胞核大、深染。诊断为隆突性皮肤纤维肉瘤。     

隆突性皮肤纤维肉瘤一例

患者,宠,40岁。主因右乳外侧紫红色斑块6年,于2010年11月12日就诊。6年前患者自觉右乳外上疗皮下有一花生大小的皮下结节,无痒痛,禾予以诊治。1年前逐渐增大至鸽蛋大小的紫红色肿物,时何隐痛。     

隆突性皮肤纤维肉瘤1例

1病历摘要 患者男,33岁.因背部皮肤肿块20余年,渐增大,于2008年12月至我科就诊.患者20余年前无明显诱因背部出现一黄豆大白色丘疹,稍隆起,无不适,未予处理.5年前丘疹明显增大并隆起于皮肤,于当地医院行手术治疗,组织病理诊断为"纤维瘤",术后2年复发,且结节迅速增大,遂于当地医院行二次手术切除,现因再次复发就诊.     

隆突性皮肤纤维肉瘤1例

隆突性皮肤纤维肉瘤是一种起源于真皮纤维组织的恶性肿瘤,临床少见,现将笔者诊治的1例报告如下。     

隆突性皮肤纤维肉瘤1例

1临床资料患者,男,31岁,农民。主因右上臂皮肤肿物1年余于2007年9月19日就诊。患者1年前无意中发现右上臂皮肤出现一米粒大肿物,无不适,未做任何诊治,肿物逐渐长大,近半月来感轻微疼痛,来院就诊要求手术切除治疗。体格检查:一般情况好,全身     

隆突性皮肤纤维肉瘤

隆突性皮肤纤维肉瘤是一种起源于皮肤并可扩展至皮下组织的局限性低度恶性的纤维肉瘤,临床少见。我科收治1例成年女性胸部隆突性皮肤纤维肉瘤患者,现报告如下。     

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。     

隆突性皮肤纤维肉瘤一例

临床资料 患者,女,46岁,因腰部皮损4年,逐渐增人,偶伴腰痛于2011年6月28日来我科就诊。患者4年前无明显诱因腰部出现蚕豆大结节,尢明显自觉症状,未予特殊治疗。此后皮损逐渐增大,并偶伴腰痛,遂于2011年6月28日来我科门诊就诊。     

隆突性皮肤纤维肉瘤二例

例1男,38岁。枕后肿块12年,质硬,表面皮肤光滑。例2女,54岁。右上臂紫红色肿块11年,质中等,表面毛细血管扩张。组织病理示:真皮及皮下组织大量组织细胞和胶原纤维呈蓆纹状、轮辐状、漩涡状排列。免疫组化:CD34+,Ki67+。两例均诊断为隆突性皮肤纤维肉瘤。     

15例隆突性皮肤纤维肉瘤临床及病理分析

目的:分析15例隆突性皮肤纤维肉瘤(DFSP)患者的临床、病理表现。方法:对我院近20年来明确诊断的15例隆突性皮肤纤维肉瘤患者资料进行回顾性分析,分析其临床和病理组织学特点及治疗情况。结果:15例DFSP中男∶女为13∶2,平均年龄38.4岁,肿瘤为单发或多发性结节。最基本的组织学特点是病变在真皮,皮下组织内浸润性生长,瘤细胞形成典型的席纹状结构;免疫组化标记示vimentin阳性率100%(15/15)、CD34阳性率100%(15/15)、肿瘤细胞Ki-67阳性率大于10%比率为60%(9/15),CK、S-100、FVⅢa、CD68均阴性。其中12例行局部切除术,3例行扩大切除加植皮或转移皮瓣修补术。全组病例复发8例(53.3%),均为行局部切除术者。结论:DFSP是一种好发于真皮的低度恶性软组织肿瘤,免疫组化标记有助于明确诊断,外科手术是其主要的治疗方法,但局部切除常易复发。     

A Case of Myxoid Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a slowly growing dermal spindle cell tumor and its myxoid variant, a rare type of DFSP, is characterized by extensive myxoid degeneration. We present the case of a 69-year-old woman with a multinodular reddish plaque on her trunk. Histopathologically, the tumor was located in the dermis and consisted of uniform spindle-shaped cells, showing strongly positive reaction for CD34, and negative for both S-100 and desmin. In addition to the typical storiform pattern, prominent myxoid stromal changes were demonstrated. Herein, we report an interesting case of myxoid DFSP, rarely reported in the dermatology literature.     

Dermatofibrosarcoma Protuberans Arising from a Burn Scar

Malignant neoplasms arising in burn scars are well known. In previous literature, 25 cases of burn scar sarcomas were reported. However, dermatofibrosarcoma protuberans is very rare and only two cases have been reported. A 43-year-old Korean man presented with multiple erythematous clustered plaques and nodules and a skin-colored subcutaneous mass on the chest after a severe burn injury at the age of 8 years. A biopsy specimen revealed dermatofibrosarcoma protuberans. The tumor was excised widely to include the surrounding burn scar. Herein, we report this third case of dermatofibrosarcoma protuberans arising from a burn scar.     

Pedunculated Nodules as a Variant of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34. Wide excision and skin graft were performed and at the 6-month follow-up, there was no evidence of recurrence or metastasis.     

Dermatofibrosarcoma protuberans

ABSTRACT: Dermatofibrosarcoma protuberans is a rare cutaneous malignancy that is locally invasive, occurs mainly over the trunk and proximal extremities, and has a tendency to recur after wide local excision. The epidemiology, clinical features, diagnosis, histology, and treatment options are discussed.     

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.     

隆突性皮肤纤维肉瘤35例临床分析

目的:探讨隆突性皮肤纤维肉瘤(DFSP)的临床特点、超声表现、手术治疗以及病理诊断和鉴别诊断要点。方法:回顾性分析35例DFSP患者的临床表现、超声特点、组织病理学特点、治疗和预后。结果:35例患者中男性22例,女性13例;平均年龄(35.4±12.4)岁。临床典型表现为皮肤斑块、斑片或结节,未见溃疡。躯干最为多见。超声:肿物位于皮下,肿物边界清楚,边缘尚规则,内呈分叶状,瘤体内有低回声或混合性回声,其内可见稀疏或较丰富的血流信号。组织病理学特征为一致性梭形肿瘤细胞呈席纹状或车辐状排列,浸润性生长,部分区域肿瘤细胞异型性明显,似纤维肉瘤样改变,肿瘤细胞表达Vimentin。本组病例MMS手术者1例术后1年复发,2例术后3年复发。结论:DFSP形态多样,临床容易误诊,B超检查对诊断、判断肿物边界以及手术治疗有一定的指导意义,组织病理及免疫组化检查可进一步确诊。MMS对本病治疗有很大优势。     

Congenital Dermatofibrosarcoma Protuberans: A Case Report and Literature Review

Congenital dermatofibrosarcoma protuberans (DFSP) is an extremely rare skin tumor that is commonly misdiagnosed, or is often diagnosed long after the initial presentation. Although many cases of DFSP are diagnosed in adulthood, there are some differences between adult DFSP and congenital DFSP. We report a case of congenital DFSP that was initially misdiagnosed as a simple vascular lesion. The delay in diagnosis led to the considerable growth of the lesion, such that a huge scar was left after the surgical treatment. The major differences between adult and congenital DFSP are discussed through a literature review. Clinicians should be aware of the characteristics of congenital DFSP, to reduce misdiagnosis and the delay of diagnosis from the initial presentation.