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1例

报告1例隆突性皮肤纤维肉瘤,患者女,36岁,因右颞部红色结节1年就诊。系统检查未发现异常。颞部结节经组织病理检查,诊断为隆突性皮肤纤维肉瘤,给予手术切除,术后半年随访无原位复发。     

隆突性皮肤纤维肉瘤1例

隆突性皮肤纤维肉瘤（dermalofilmsaroma proluberats）是一种不见的皮肤软组织肿瘤，我科于2003年6月收治1例成年男性腹部隆突性皮肤纤维肉瘤患者，现报告如下。     

隆突性皮肤纤维肉瘤1例

 报告1例隆突性皮肤纤维肉瘤。患者男,71岁。左上胸部淡红色无痛性结节3年余。患者10年前在胸部左上方发现一个“良性肿物”,后在当地医院行手术切除。3年前手术切口右下方出现淡红色结节,逐渐增大,无瘙痒、疼痛等。皮肤专科查体：左上胸部靠近锁骨中段可见淡红色结节,大小约 2 cm×2.5 cm,表面有毛细血管扩张,触之质硬,边界清楚。结节组织病理示：表皮明显萎缩,真皮中下部可见大量梭形肿瘤细胞,肿瘤细胞未见明显异型性,细胞核呈梭形或长柱状,排列成席纹状或漩涡状。免疫组化染色示：真皮梭形细胞Vimentin（+）,CD34（+）。诊断：隆突性皮肤纤维肉瘤。诊断明确后行结节扩大切除+带蒂复合组织瓣及任意皮瓣转移修复术。术后恢复顺利,创面愈合良好。目前在随访中,未发现复发。     

隆突性皮肤纤维肉瘤2例

报告2例隆突性皮肤纤维肉瘤。例1男,37岁,左侧肩部红色斑块20年;例2女,64岁,左侧腹股沟包块7年。皮损组织病理均示真皮全层大量的梭形肿瘤细胞,排列成席纹状或漩涡状,部分肿瘤细胞核大、深染,部分核分裂。均诊断为:隆突性皮肤纤维肉瘤。     

隆突性皮肤纤维肉瘤1例

患儿男,14岁.因右大腿部结节6年,于2008年1月至上海市皮肤病性病医院就诊.患儿6年前右大腿皮肤出现一蚕豆大结节,呈蓝色,无自觉症状.近2年皮损逐渐增至鸽蛋大,其上方又新发一蚕豆大结节.曾在当地医院诊断为皮肤纤维瘤,未予任何治疗.患儿发病前局部无外伤史和昆虫叮咬史.既往体健,家族成员中无类似疾病患者.     

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

ABSTRACT: In view of the important clinicopathologic, evolutional, and ultrastructural characteristics of dermatofibrosarcoma Protuberans, a review was made of 30 patients seen during the last 25 years in Colombia's Instituto Nacional de Cancerologia. The incidence is low, accounting for 0.06% of all malignant tumors and 1.17% of all soft-parts sarcomas. The series comprises 19 women and 11 men, with the highest incidence found between ages of 30 and 50 years, although the study includes two children, ages 8 and 13. The chest was the most common site. Forty percent of the lesions were multinodular, and 23.3% showed ulceration. Histologic appearance is characterized in all cases by fusiform cells arranged like spokes around an empty hub. The fibroblastic origin was ascertained by electron-microscopic studies. The treatment used was broad surgical excision, removing tissue down to the aponeurosis to avoid recurrence.     

Dermatofibrosarcoma Protuberans

An ultrastructural study of five cases of dermatofibrosarcoma protuberans revealed that the basic cell was a spindle shaped fibroblast with a peculiar deeply lobulated nucleus. An origin from perineural and endoneural cells could not be established since basal lamina material was sparsely present in only one case and other differential features were missing. In one case histiocytic cells were found, but also in this case the basic cell was the fibroblast; so evidence of relationship with fibrous histiocytoma was not found.     

A Case of Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor)

The present paper reports a 67-year-old male with pigmented dermatofibrosarcoma protuberans (Bednar tumor) on his right shoulder. The lesion had recurred twice. Clinically, the lesion was a reddish-brown, exophytic, multilobular, firm nodule with an irregular surface, which measured 6.4 times 4.2 cm. Histologically, the proliferated fibroblasts showed a cartwheel pattern and stained positively with CD34, similar to those of a common dermatofibrosarcoma protuberance. However, many melanin-bearing cells that were positive for the S-100 protein were found intermingled among the tumor cells. Based on these findings, we concluded that the clinical manifestations, level of malignancy, and cytological features, as well as the exception of melanin-bearing dendritic cells associated with pigmented dermatofibrosarcoma protuberans, suggest a common dermatofibrosarcoma protuberans. This supports the hypothesis that pigmented dermatofibrosarcoma protuberans is a pigmented variant of dermatofibrosarcoma protuberans. Thus, this type of tumor is not benign, but is an intermediate malignant tumor.     

Congenital Dermatofibrosarcoma Protuberans

Abstract: Congenital dermatofibrosarcoma protuberans occurred in a 16-year-old girl. The lesion was a hard cutaneous plaque on the abdomen. It was present at birth and slowly enlarged during the patient's lifetime. Histologic examination was essential to establish the diagnosis and to differentiate it from other congenital fibrohistiocytic proliferations.     

Congenital Myxoid and Pigmented Dermatofibrosarcoma Protuberans: A Case Report

Dermatofibrosarcoma protuberans (DFSP) is a low‐grade, mesenchymal, spindle cell tumor. In addition to the classical form characterized by a storiform pattern of tumor cells, pigmented (Bednar's tumor) and myxoid variants can be observed. Classical DFSP and Bednar's tumor are easily diagnosed. The myxoid variant represents a diagnostic challenge. Pigmented and myxoid variants are rare and thus far have never been reported in association in congenital DFSP. We came across a unique DFSP that was, at the same time, congenital, pigmented, and myxoid. The tumor was surgically excised with broad free margins and no recurrence. The differential diagnosis with other entities such as giant cell fibroblastoma, CD34‐positive plaque‐like dermal fibroma, superficial plaque‐like CD34 DFSP, and neurocristic hamartoma is discussed. The recognition of this hybrid variant of congenital DFSP is important to avoid under‐ or overtreatment.     

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.     

Dermatofibrosarcoma Protuberans in Childhood

Abstract: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a possibly misleading presentation. It is rare in childhood. We report an 18-month-old child with an acquired atrophic plaque of the left ankle. Based on clinical findings, initial proposed diagnoses included localized scleroderma and localized lipoatrophy. At the age of 5 years the plaque had increased in size and become a purplish color. Several firm nodules were seen at its periphery. A diagnosis of DFSP was proposed and confirmed histologically. A large surgical resection was performed. This case illustrates that DFSP may occur In childhood, during which initial diagnosis is generally more difficult than in adulthood.