隆突性皮肤纤维肉瘤16例临床分析

目的 探讨隆突性皮肤纤维肉瘤(DFSP)的临床特点及治疗。方法 收集我院经组织病理检查确诊的DFSP16例，并对其进行分析。结果 本组病例术前诊断为DFSP3例，误诊7例，术后复发6例，复发2次以上2例，转移1例。结论 DFSP是一种好发于真皮的低度恶性软组织肿瘤，局部手术切除后复发率较高，宜采用扩大切除术治疗。     

隆突性皮肤纤维肉瘤1例

隆突性皮肤纤维肉瘤（Dermatofibrosarcoma Protuberans，DFSP）是一种生长缓慢、起源于皮肤并可扩展至皮下组织的局限性低度恶性纤维肉瘤。我所诊断1例隆突性皮肤纤维肉瘤，报道如下。     

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。     

隆突性皮肤纤维肉瘤中PTEN的表达

目的:观察隆突性皮肤性纤维肉瘤中PTEN的表达情况,探讨它的意义.方法:使用免疫组化结合图像分析法检测39例隆突性皮肤纤维肉瘤(DFSP)、30例皮肤纤维瘤(DF)中CD34、PTEN的表达情况.结果:CD34蛋白在DFSP中表达明显高于在DF的表达;而PTEN蛋白在DFSP中的表达低于DF，差异具有统计学意义（P＜0.05) .直线相关分析表明:CD34和PTEN蛋白的表达之间无相关性(P＞0.05).结论:PTEN与隆突性皮肤纤维肉瘤的发生、发展密切相关,除CD34外,可作为隆突性皮肤纤维肉瘤与皮肤纤维瘤鉴别的参考指标.     

隆突性皮肤纤维肉瘤

隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberan．DFSP)是一种少见的中间恶性皮肤肿瘤，约占皮肤恶性肿瘤的0．1％，以其明显的复发倾向受到人们注意。1924年Darier和Ferrand首先将这种肿瘤描述为“进行性和复发性真皮纤维瘤”，次年，Hoffmann将它命名为隆突性皮肤纤维肉瘤。其重要特征是：极易原位复发，罕见转移。     

隆突性皮肤纤维肉瘤误诊2例报告

隆突性皮肤纤维肉瘤是一种生长缓慢 ,低度恶性的软组织肿瘤 ,临床少见 ,容易误诊。现将我科的 2例误诊报告如下。例 1　男 ,48岁。右大腿前上方近腹股沟处有直径 1ｃｍ结节 1年余。表面皮肤呈青兰色 ,略隆起 ,与皮肤粘连 ,质偏硬 ,触痛明显。以皮肤纤维瘤行手术切除 ,组织病理报告为隆突性皮肤纤维肉瘤 ,再次行扩大切除术。随访 4年未复发。例 2　女 ,36岁。右上腹壁见直径 2ｃｍ肿块 3年 ,表面皮肤呈青紫色 ,与皮肤粘连 ,呈分叶状 ,触痛明显。以痛性血管脂肪瘤行手术切除 ,组织病理报告为隆突性皮肤纤维肉瘤 ,再次行扩大切除术。随访 8个月…     

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。     

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。     

白化病伴发隆突性皮肤纤维肉瘤1例

患者男,38岁,白化病患者,伴发左颞部隆突性皮肤纤维肉瘤。曾切除肿瘤2次而复发。组织病理示隆突性皮肤纤维肉瘤病。本文就该病的诊断及治疗进行了讨论。     

隆突性皮肤纤维肉瘤34例分析

隆突性皮肤纤维肉瘤(DFSP)是发生于皮肤的低度恶性肿瘤，较少见，临床表现易与其它疾病相混淆，局部侵袭性强，治疗以手术切除为主，术后易复发。我们收集本院34例DFSP予以总结并进行文献复习。1临床资料1．1一般资料男26例，女8例。年龄10～72岁，平均40．4岁。病程2年以下10例，2～5年11例，6～10年8例，10年以上     

An unusual clinical presentation of myxoid dermatofibrosarcoma protuberans with a prominent vasculature: A potential pitfall in the diagnosis of myxoid soft tissue tumors

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that arises primarily on the trunk and extremities but seldom on the scalp. Several variants of DFSP have been described, including myxoid DFSP. Although typical DFSP may have focally myxoid areas, myxoid DFSP, in which most of the stroma is myxoid, is rare and can pose diagnostic challenges. Here, we report a case of myxoid DFSP with an unusual clinical presentation that could have been mistaken for a lipoma. Additionally, the myxoid DFSP displayed prominent vasculature in a myxoid stroma, which could have been mistaken for a myxoid liposarcoma.     

Congenital dermatofibrosarcoma protuberans: 30 years of follow‐up

Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor that commonly appears in adult patients. Few cases of DFSP in childhood have been reported. We describe a case of congenital DFSP that had been incompletely excised in childhood, and again at 28 years of age. The 33‐year‐old woman presented with a recurrence. When comparing the histologic features of the previous specimens excised in 1970 and 30 years later, the similar typical storiform pattern of fibromatous cells was found. The small number of proliferation‐marker‐positive cells was not altered over the course of 30 years. In line with a few additional reports, we suggest that congenital dermatofibrosarcoma protuberans is a tumor with a low malignant potential.     

A case of childhood dermatofibrosarcoma protuberans without detected cytogenetic abnormality

Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative skin tumour of intermediate malignancy, with a limited potential for metastasis but a high rate of recurrence; specific cytogenetic abnormalities are now known. Childhood DFSP has been considered a rarity in the past, but it is now recognized that many cases of childhood DFSP are diagnosed only in adulthood. Despite advances in the understanding of its pathogenesis as well as the development of valuable immunohistochemical and cytogenetic diagnostic techniques, there often remains a significant delay between the initial presentation and diagnosis of DFSP. We report a case of childhood DFSP in which the diagnosis was reached only after a nodular lesion developed in a plaque that was initially present. Causes for delay between initial presentation and diagnosis in childhood DFSP are discussed. Histology and immunostaining in our patient showed the typical features of DFSP, but the G-banded cytogenetic analysis of short-term tissue culture was negative. However, this technique offers only a detection rate between 50% and 80%. Clinicians should be aware of the limitations of newer diagnostic techniques. Increasing recognition amongst paediatricians and paediatric dermatologists that childhood DFSP is not as rare as once believed will probably lead to the use of newer diagnostic methods at an earlier stage, and so reduce the delay between the onset of symptoms and diagnosis.     

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.     

Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55-year-old woman with a dark-red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast-like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.     

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.     

Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection

Background Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma of the skin characterized by the presence of specific COL1A1–PDGFB fusion protein, which appears as a consequence of the t(17;22) (q22;q13) translocation. Objective The aim of the study was to perform an analysis of patients with advanced DFSP treated with imatinib, with or without surgery, in clinical practice outside trials. Patients and Methods We analysed the data of 15 patients (6 male, 9 female; median age 56 years) with locally advanced/initially inoperable and/or metastatic DFSP treated with imatinib 400–800 mg daily between 12/2004 and 06/2009. All diagnoses were ascertained cytogenetically (fluorescent in situ hybridization). Median follow‐up time was 16 months (range: 4–81). Results Metastases were present in six cases (two lungs, two soft tissue, two lymph nodes). Fibrosarcomatous transformation (FS‐DFSP) was confirmed in seven patients (47%). A 2‐year progression‐free survival (PFS) rate was 60%, and a 2‐year overall survival (OS) rate was 78% (median time for PFS/OS was not reached). The best overall responses were: 10 partial responses (67%, including 5 FS‐DFSP – 1 progressed during the follow‐up), 2 stable diseases (13%) and 3 progressive diseases (20%). Seven patients (47%) underwent resection of residual disease and remained free of disease. Conclusions We have confirmed the profound anti‐tumour effect of imatinib in DFSP harbouring t(17;22) with long‐term responses. Imatinib therapy may in some cases lead to tumour resectability of lesser disfiguration.     

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.     

Expression of nestin in dermatofibrosarcoma protuberans in comparison to dermatofibroma

Making a differential diagnosis to distinguish dermatofibrosarcoma protuberans (DFSP) from dermatofibroma (DF) is occasionally difficult. In those instances, CD34 and factor XIIIa have been used as valuable differential markers. The histogenesis of DFSP, however, remains uncertain and controversial, although it is generally thought to be a neuromesenchymal neoplasm. Nestin is an intermediate filament protein that was first observed in neuroectodermal stem cells. We investigated the expression of nestin in order to distinguish between DFSP and DF in combination with the use of conventional markers, CD34 and factor XIIIa. The nestin expression was investigated in tissue specimens from 16 DFSP cases and 30 DF cases. The expression of other differential markers such as CD34, factor XIIIa, CD163 and CD10 was also observed in these samples. Fifteen (94%) of 16 cases of DFSP showed the expression of nestin, whereas only four (13%) of 30 cases of DF showed the expression of nestin. Most of the DFSP cases showed a diffuse positive reaction in more than half of the tumor cells. In contrast, DF cases with nestin expression showed a partial positive reaction. Nestin is considered to be a useful and additional marker in combination with CD34, factor XIIIa and CD163 in order to distinguish between DFSP and DF. The frequent expression of nestin in DFSP may therefore indicate that DFSP is derived from putative, multipotent neuromesenchymal cells.