Atrophic dermatofibrosarcoma protuberans: an uncommon and misleading variant

A 23-year-old man presented with an atrophic lesion on his left anterior shoulder that had been present for at least 10 years. A previous biopsy had suggested a fibrohistiocytic origin; however, clinically it resembled morphoea or atrophoderma, and the lesion was observed. Fifteen months later, the lesion was noted to have grown slightly. A repeat biopsy showed dermatofibrosarcoma protuberans, with a storiform spindle cell proliferation with positive CD34 staining. The lesion was excised with a 3-cm margin. Awareness of this rare presentation may assist in earlier diagnosis.     

Composite tumor consisting of dermatofibrosarcoma protuberans and myxoflbrosarcoma

A unique case of a composite tumor consisting of dermatofibrosarcoma protuberans and low-grade myxofibrosarcoma is described. The myxofibrosarcoma component formed 70% of the lesion volume. Both components protruded above the skin, and equally both infiltrated the subcutaneous tissue in the base of the lesion. The interface between them was mostly sharp but some small areas of gradual transition were also seen. Beside fibrosarcoma arising in the dermatofibrosarcoma protuberans, myxofibrosarcoma seems thus to be another possible manifestation of the change into a more malignant tumor rarely seen in the dermatofibrosarcoma protuberans.     

Childhood-onset multifocal atrophic dermatofibrosarcoma

BACKGROUND: Atrophic dermatofibrosarcoma is a rare clinical variant of dermatofibrosarcoma protuberans (or Darier-Ferrand tumor) preferentially observed in childhood and early adulthood. OBSERVATION: We report a case of multifocal atrophic dermatofibrosarcoma protuberans of childhood onset only diagnosed when the patient was 29 years old. The clinical presentation was an asymptomatic macular brown plaque on the right thigh measuring 10 cm. Initially, because of the large size of the lesion, treatment consisted of limited surgical excision. DISCUSSION: Diagnosis of the atrophic variant of dermatofibrosarcoma in childhood is difficult, and is usually made several years later in early adulthood because of its slow development, lack of symptoms and generally benign appearance. Histological tests and immunohistochemical staining may confirm clinically suspected diagnosis, and in complex cases, cytogenetic studies can help confirm a diagnosis of dermatofibrosarcoma through detection of reciprocal translocation t (17,22), which fuses collagen type Ialpha1 (COLIA1) and platelet-derived growth factor (PDGDFbeta), and which is highly characteristic of dermatofibrosarcoma protuberans. Conventional treatment of dermatofibrosarcoma protuberans consists of extensive surgical excision, but Mohs micrographic surgery is also advocated for removal of certain dermatofibrosarcoma protuberans, while use of tyrosine kinase PDGF receptor inhibitors such as imatinib mesylate (Glivec) is limited to distant metastases.     

Dermatofibrosarcoma protuberans: an early non-protuberant phase of the tumour

A 40-year-old female patient presented with a flat scar like plaque on the upper chest. The patient's perception of subtle change in the lesion was of paramount importance in the decision to biopsy this lesion of innocuous clinical appearance. Histopathological findings were those of dermatofibrosarcoma protuberans. This case illustrates the early clinical features of the tumour and a recommendation for taking a biopsy of any scar-like plaque where there is no clear history of preceding trauma.     

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.     

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。     

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.     

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.     

Pedunculated presentation of dermatofibrosarcoma protuberans

A 26-year-old patient had a dermatofibrosarcoma protuberans that clinically was considered to represent either a skin tag or neurofibroma. Histopathologically, this lesion could easily have been misinterpreted as a neurofibroma, particularly in the context of the clinical findings.     

Mitogenic activity of dermatofibrosarcoma protuberans is mediated via an extracellular signal related kinase dependent pathway

Dermatofibrosarcoma protuberans is a malignant mesenchymal tumor originating in the dermis. Although it is locally aggressive and recurs unless completely excised, it only rarely metastasizes. In this study, we investigated the mechanisms of increased proliferation of dermatofibrosarcoma protuberans cells. The cells showed increased DNA synthesis in serum-free medium, which was demonstrated by the incorporation of [3H]-thymidine. Increased DNA synthesis of dermatofibrosarcoma protuberans cells was abolished by genistein, a tyrosine kinase inhibitor, or by PD98059, a specific extracellular signal related kinase pathway inhibitor, but not by calphostin C, a protein kinase C inhibitor. Immunoblotting analysis of dermatofibrosarcoma protuberans cells using a specific antibody against phosphorylated extracellular signal related kinase (Thr202/Tyr204) showed that extracellular signal related kinase was expressed as constitutively phosphorylated molecules in dermatofibrosarcoma protuberans cells. Immunofluorescence analysis showed that the kinase was constitutively located in the nucleus of the cells. Furthermore, transfection of the dominant negative mutant extracellular signal related kinase into dermatofibrosarcoma protuberans cells abolished the increased mitogenic activity of the cells. These results suggest that an extracellular signal related kinase dependent pathway is implicated in the increased mitogenic activity of dermatofibrosarcoma protuberans cells.     

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。     

Dermatofibrosarcoma protuberans with onset in early childhood: a case report

Dermatofibrosarcoma protuberans is a tumour that grows locally, infiltrating the subcutaneous tissue; it is uncommon in children. We report the case of a 27-year-old man who had had such a lesion from the age of 3 years, and it had enlarged during his lifetime. A wide local excision with margins of 3.0 cm was performed, but the lesion was not entirely removed. This is a new report of infantile dermatofibrosarcoma protuberans, and this case reflects the need for Mohs micrographic surgery.     

A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia

Bednar tumor is a rare pigmented variation of dermatofibrosarcoma protuberans, present in 1 to 5% of all patients with dermatofibrosarcoma protuberans. No significant clinicopathologic differences exist between Bednar tumor and conventional dermatofibrosarcoma protuberans apart from the presence of scattered nonneoplastic pigmented dendritic cells in the former. Although most dermatofibrosarcoma protuberans occur in adults, they may be rarely present at birth. Fanconi anemia is a genetically heterogeneous chromosomal instability syndrome, characterized by multiple congenital anomalies, progressive bone marrow failure, and a predisposition to malignancy. We describe here a patient with Fanconi anemia who had a congenital Bednar tumor. To our knowledge, this is the first such patient described with both dermatofibrosarcoma protuberans and Fanconi anemia.     

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.     

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.     

负压辅助闭合技术在修复隆突性皮肤纤维肉瘤扩大切除创面中的应用

目的 探讨修复隆突性皮肤纤维肉瘤扩大切除创面的新方法。方法 8例隆突性皮肤纤维肉瘤患者,肿瘤直径2.5 ～ 5.5 cm,切除后创面直径为12.5 ～ 17.5 cm。针对扩大切除后的巨大创面,利用筛状皮片及负压辅助闭合技术,修复创面。结果 8例患者筛状皮片均一期存活,无感染、无积液、无坏死。随访3 ～ 40个月,无1例复发,创面外形与功能均较满意,瘢痕平整,增生不明显。结论 负压辅助闭合技术在修复隆突性皮肤纤维肉瘤扩大切除创面中的应用,操作简单,并发症少。     

Atrophic dermatofibrosarcoma protuberans: case report, review, and proposed molecular mechanisms

We report a case of atrophic dermatofibrosarcoma protuberans, an uncommon clinical variant. We discuss molecular and genetic mechanisms of dermatofibrosarcoma protuberans tumorigenesis, review the literature on atrophic dermatofibrosarcoma protuberans, and pose a molecular mechanism for the atrophic variant's phenotype. We also describe using Mohs micrographic excision with CD34 immunostains on the frozen sections to definitively treat the tumor.     

Bednar tumor: a report of two cases

We have recently treated two Japanese with Bednar tumor, pigmented dermatofibrosarcoma protuberans. The tumors were multilobular with brown-pigmentation and an irregular surface. Firm nodules were growing into the deep subcutaneous tissue, but they were movable over the underlying tissue. Histopathological examination and immunohistochemical studies showed the typical features of Bednar tumor and positive staining for CD34, which is known to be expressed in dermatofibrosarcoma protuberans. Bednar tumor is thought to be a variant of dermatofibrosarcoma protuberans and to have intermediate malignancy.     

误诊为脂肪瘤的隆突性皮肤纤维肉瘤一例

患者,男,45岁。右侧胸部肿物6年, 肿块位于皮下,质软,无疼痛不适。6年前B超检查示脂肪瘤可能性大,未予特殊处理。本次就诊行病理检查结果示软组织来源的梭形细胞肿瘤。诊断为隆突性皮肤纤维肉瘤,行肿瘤扩大根治术。     

Tumor in the plantar region: dermatofibrosarcoma protuberans in an infrequent topography

Dermatofibrosarcoma protuberans is a rare mesenchymal tumor; it is locally aggressive and presents high rates of local recurrence. It may present as a nodular or plaque vegetating lesion. It mainly affects the trunk and proximal limbs, being rare in the distal extremities. Biopsy and immunohistochemistry help confirm the diagnosis. The authors report a case of dermatofibrosarcoma protuberans with plantar region involvement, a rare presentation. To the best of the authors’ knowledge, only 11 cases of involvement of the feet were described in the international literature.