色素性隆突性皮肤纤维肉瘤一例

<正>临床资料患者,女,20岁。腰背部4年前无明显诱因出现黄豆大深蓝色斑片,无明显自觉症状,未予重视。2个月前患者发现皮损逐渐扩大,颜色加深,皮损呈深蓝色,无破溃。发病前无外伤史,既往体健,无慢性病史,家族中无遗传病史及类似病史。皮肤科情况:背部可见指甲盖大蓝色斑片,境界清楚,中央萎缩凹陷,表面未见明显丘疹、结节,未见糜烂及渗出,皮损触之较韧,无触痛,皮温正常(图1)。组织病理示:病变主要位于真皮中下层并侵犯皮下脂肪组织,可见梭形瘤细胞围绕     

色素性隆突性皮肤纤维肉瘤一例

患者,女,40岁.背部肿物6年余.组织病理示:真皮及皮下组织巨大肿物,其内大量梭形细胞呈席纹状排列,其间见轻微核异型,并可见大量黑素细胞及黑素颗粒.诊断:色素性隆突性皮肤纤维肉瘤.治疗:手术沿肿物扩大2 cm切除,随访3个月无复发.     

Pigmented dermatofibrosarcoma protuberans (Bednar tumour).

A case of pigmented dermatofibrosarcoma protuberans in a 37-year-old man is reported. The tumour presented as a nodular lesion located in the left scapular area. Surgical resection with wide margins was performed. No recurrences have appeared to date. The presumptive origin of this entity is briefly discussed.     

Pigmented dermatofibrosarcoma protuberans. Report of two cases as a variant of dermatofibrosarcoma protuberans with partial neural differentiation

Two cases of so-called Bednár tumor are reported. The histopathological finding of a typical storiform pattern composed of fusiform tumor cells resembled dermatofibrosarcoma protuberans (DFSP) except for the presence of melanin-containing cells scattered within the lesion. Ultrastructurally, the predominant tumor cells in case 1 possessed extensive cytoplasmic processes, convoluted nuclei, and intercellular junctions, but lacked basal lamina. Melanocytic tumor cells contained melanosomes and were surrounded by basal lamina. Immunohistochemical examinations revealed a positive reaction for S-100 protein and neuron-specific enolase in the pigment-laden cells. Consequently, these two tumors were considered to be a variant of DFSP with partial neural differentiation.     

Atrophic dermatofibrosarcoma protuberans

A 48-year-old woman presented with a 20-year history of an asymptomatic depressed atrophic plaque on the abdomen. Five years earlier a punch biopsy of the same lesion had been carried out and a diagnosis of dermatofibroma was made. She was reassured and discharged. Further consultation was sought due to extension and thickening of the lesion. Re-examination of the initial and new incisional biopsy specimens, along with histochemical staining for CD34, established the diagnosis of atrophic dermatofibrosarcoma protuberans. A wide local excision was carried out. There has been no recurrence at 9 months of follow up.     

Sclerosing dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a low-grade sarcoma of the skin with well characterized clinical and histopathological features. The lesion frequently appears as a plaque on the trunk or on the extremities of middle-aged adults. Microscopically, the tumor is composed of monomorphous spindle cells arranged in storiform pattern and embedded in a sparse to moderately dense fibrous stroma. We report on two unusual cases of dermatofibrosarcoma protuberans in which neoplastic cells were diffusely replaced by sclerotic tissue in more than 50% of the entire tumor. In both cases, no external trauma or radiotherapy were recorded. Since tumor regression has been defined as a loss of tumor mass in the absence of any treatment (or trauma) we believe that sclerosis in DFSP may represent a manifestation of regression and not, as previously suggested, a new variant of the neoplasm.     

先天性隆突性皮肤纤维肉瘤

患儿女,10岁。 主诉：右背部出现多个结节10年。 现病史：患儿于出生时家人发现其右背部皮肤有一米粒大结节,中等硬度,表面皮肤正常。随着年龄增长,结节表面皮肤颜色渐渐变深,面积逐渐扩大并形成凹陷。皮下肿块也逐渐增大并突出皮面。肿块底部逐渐向外扩展,形成新的肿块,结节数目增加到6个。初始结节无任何异常感觉,随着结节逐渐增大、增多,局部有压痛,未曾治疗过。     

Fibrosarcomatous dermatofibrosarcoma protuberans with giant rosettes

A rare case of dermatofibrosarcoma protuberans, which contained low-grade fibrosarcoma with giant rosettes in its recurrence areas is described. The giant rosettes lying in the fibrosarcomatous area produced a pattern similar to that of so-called hyalinizing spindle cell tumor with giant rosettes. The transitions between the tumor components were gradual, and both primary and recurrent lesions showed diffuse immunoreactivity for CD34. The presence of giant rosettes in fibrosarcomatous areas of dermatofibrosarcoma protuberans suggests a possible histogenetic relationship between fibrosarcomatous areas of DFSP and hyalinizing spindle cell tumor with giant rosettes.     

Vascular congenital dermatofibrosarcoma protuberans: a new histological variant of dermatofibrosarcoma protuberans

We describe a case of congenital dermatofibrosarcoma protuberans (DFSP) that masqueraded as a vascular tumor both clinically and histologically. Based on the infiltrative growth pattern, presence of capillary-sized vessels, and spindle cell areas with slit-like vascular spaces and numerous thin-walled vessels at the periphery of the tumor, a kaposiform hemangioendothelioma was initially diagnosed. Strong diffuse CD34 positivity and the extension into the subcutaneous fat with a sieve-like effect prompted the fluorescence in situ hybridization analysis, which demonstrated a reciprocal t(17;22) translocation. According to our knowledge, this is the first report of a vascular histological variant of DFSP. This unique variant represents a potential pitfall for dermatopathologists and underlines the importance of cytogenetic diagnostics in unusual cases of DFSP.     

Childhood onset dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, locally aggressive dermal tumor of disputed histogenesis with a tendency for local recurrence. A 16-year-old female presented with hyperpigmented macules, papules, plaques, erythematous nodules and atrophic, indurated lesions ranging from a few millimeters to a few centimeters. Biopsy revealed thinned out epidermis and a diffuse dermal infiltration by fascicles of spindle cells arranged in storiform pattern. The pseudoseptae formed by the tumor cells interdigitating between the subcutaneous adipocytes resulted in a honeycomb pattern. Histochemical findings are helpful, but the chronicity, size, indolent nature, recurrence after surgery and histopathology are more important to arrive at a diagnosis. Wide and deep surgical excision is the treatment of choice.     

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.     

隆突性皮肤纤维肉瘤1例

报告1例隆突性皮肤纤维肉瘤,患者男性,35岁,头皮起紫红色结节1年半,多次手术均复发,病理检查证实为隆突性皮肤纤维肉瘤。