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.     

Dermatofibrosarcoma protuberans in two patients with acquired immunodeficiency syndrome

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous tumor of intermediate malignancy. Most commonly, it arises as an asymptomatic, indurated plaque on the trunk within which protuberant nodules develop over time. We describe its occurrence in two patients with human immunodeficiency virus, a previously unreported association. The first patient, a 41-year-old woman, complained of painful lesions around the left shoulder that developed within a scar from previous trauma to the area. The second patient, a 50-year-old man, developed a recurrent DFSP within the scar from a previous surgical procedure. Dermatofibrosarcoma protuberans was confirmed in both cases by the histopathologic and immunohistochemical findings.     

Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant: clinicopathologic analysis of 5 cases

We report a series of five cases of dermatofibrosarcoma protuberans, four of which were fibrosarcomatous and all of which showed histologic and immunohistochemical evidence of focal myoid/myofibroblastic differentiation (accounting for up to 5% of each tumor). These lesions were identified amongst 208 cases of dermatofibrosarcoma protuberans including 24 examples of the fibrosarcomatous variant. Three of the five patients were male and two were females; all were adults (37–72 years). One case arose on the scalp and two cases each on the abdominal wall and upper trunk. All tumors were less than 5cm in diameter and preoperative duration ranged from 2 months to 10 years. In three cases with follow-up there was no recurrence. Histologically, all tumors were typical fibrosarcomatous or ordinary dermatofibrosarcoma protuberans but for the presence of scattered to confluent nodules and bundles of eosinophilic spindle cells associated with well-defined cytoplasmic margins and vesicular nuclei associated with focal stromal hyalinization. While the typical dermatofibrosarcoma protuberans areas were CD34 positive, the myoid areas were negative for this antibody and positive for smooth muscle actin and pan-muscle actin. All tumors were desmin negative. Recognition of myofibroblastic differentiation in fibrosarcomatous dermatofibrosarcoma protuberans is important not only because it gives support to the theory of a fibroblastic/myofibroblastic line of differentiation for this type of tumor, but also because it might be a source of confusion with other myofibroblastic lesions (e.g. myofibromatosis, adult myofibroma), especially when small biopsies are evaluated.     

The fibroblastic nature of dermatofibrosarcoma protuberans: Morphological investigations in vivo and in vitro

Summary Six cases of dermatofibrosarcoma protuberans were studied ultrastructurally. Biopsy material from all six cases as well as cell cultures derived from four cases were examined. In all cases, the tumor cells in vivo and in vitro were related to fibroblasts. The two cases with histiocytoid features and a small spiral pattern exhibited numerous lipid vacuoles but no histiocytic cell markers. In these two cases, cultured cells contained a higher number of intracytoplasmic vacuoles than the control fibroblasts. Two other cases exhibited some basement-membrane-like material surrounding tumor cells. All of the investigated cell strains obtained from the tumors showed synthesis of collagenous proteins similar to that found in fibroblasts. However, the level of total collagen was reduced, and type-III collagen was absent.The morphological variations in these cases of dermatofibrosarcoma protuberans appeared to be related to the histological pattern and may reflect the heterogeneity of normal fibroblasts.     

萎缩型隆突性皮肤纤维肉瘤二例

报道2例萎缩型隆突性皮肤纤维肉瘤.患者分别为16岁和24岁的男性,临床最初均表现为界限清楚的萎缩型红斑,逐渐在红斑基础上发生结节.病程分别为6年和5年.皮损组织病理检查显示表皮萎缩或正常,肿瘤细胞在真皮浅层呈波浪状排列,平行于表皮;肿瘤细胞在真皮下部呈席纹状、车幅状、编织状排列,并可见肿瘤细胞侵犯皮下脂肪,分割脂肪细胞.肿瘤细胞表达波形蛋白、CD34,不表达CD68和S100蛋白.

Abstract：

Two cases of atrophic dermatofibrosarcoma protuberans (DFSP) are reported.The patients were a 16-year-old and a 24-year-old boy with a clinical course of 6 and 5 years respectively.The lesions began as well-marginated atrophic erythema,and subcutaneous nodules appeared gradually on the erythema.Histopathology showed atrophic or normal epidermis,wavy arrangement of tumor (spindle) cells in the superficial dermis which was aligned parallel to the epidermis,storiform arrangement of tumor cells in the lower dennis,and typical lacelike pattern of infiltration of subcutaneous fat tissue with tumor cells.Immunohistochemistry showed that the tumor cells stained positive for vimentin and CD34,but negative for S100 or CD 68.     

Congenital atrophic dermatofibrosarcoma protuberans in a 7-month-old boy treated with Mohs micrographic surgery

Abstract:  Dermatofibrosarcoma protuberans is a rare, malignant, slow-growing, locally invasive tumor of the skin. Although most cases are acquired and diagnosed in adulthood, there have been an increasing number of congenital dermatofibrosarcoma protuberans mimicking benign birthmarks described in the literature. The clinical presentation of this tumor is often one of an indurated exophytic plaque or nodule; however, a rare variant can present as atrophic or sclerotic in nature. We report a case of congenital atrophic dermatofibrosarcoma protuberans of the groin in a 7-month-old boy, successfully treated with Mohs micrographic surgery.     

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.     

The histopathologic spectrum of regression in atypical fibroxanthoma

Background: Atypical fibroxanthoma (AFX) with prominent fibrosis, sclerosis and hyalinization, and near‐total tumor regression is rare. Methods: Eight cases of AFX presenting with fibrosis were reviewed as to their tumor architecture, the degree and pattern of fibrosis and the associated inflammatory cell infiltrate. Results: Seven of eight cases had an exophytic architecture, with ulceration in one case. The degree of fibrosis ranged from 10% to 90%. Early fibrosis (2/8 cases) occurred as thickened sclerotic collagen bundles, either dispersed between the neoplastic cells or as septa imparting a multilobular appearance. Advanced fibrosis (6/8 cases) was associated with lamellar sclerosis, keloidal features, hyalinization and with near‐total tumor replacement. Prominent fibrosis rimming the periphery was present in all tumors. An associated lymphoid cell infiltrate with plasma cells and occasionally eosinophils was observed. Conclusions: Fibrosis with prominent sclerosis and hyalinization replacing the tumor is rare in AFX. Advanced fibrosis, in the absence of a history of prior trauma or surgery, may indicate spontaneous regression. These cases emphasize the importance of recognizing this subset of AFX in order to avoid misinterpretation, particularly in cases with few residual atypical cells. Stefanato CM, Robson A and Calonje JE. The histopathologic spectrum of regression in atypical fibroxanthoma.     

Pseudocystic dermatofibrosarcoma protuberans: report of two cases and demonstration of COL1A1-PDGFB rearrangement

We report two unique cases of dermatofibrosarcoma protuberans (DFSP) that included a pseudocystic component. Molecular analysis of one of the cases showed a characteristic COL1A1-PDGFB rearrangement in both the main tumor and also in the cells lining the pseudocystic portion of the tumor, confirming the diagnosis and indicating that the lining represented a component of the proliferation. It is important to raise awareness of this rare variant within the varied spectrum of DFSP. Shvartsbeyn M, Lazar AJF, Lopez-Terrada D, Meehan SA. Pseudocystic dermatofibrosarcoma protuberans: report of two cases and demonstration of COL1A1-PDGFB rearrangement.     

Congenital dermatofibrosarcoma protuberans: a case report

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence.     

Myxoid dermatofibrosarcoma protuberans: morphological, ultrastructural and immunohistochemical features

Two uncommon cases of dermatofibrosarcoma protuberans with prominent myxoid changes are presented. The tumors appeared as large multinodular cutaneous plaques that arose at the sites of excision of previous tumors some years earlier. In addition to limited Fibrous storiform features, focally observed in deep and peripheral portions of the tumors, a diffuse myxoid pattern could be observed. The latter consisted of homogeneous areas of rare, stellate or spindle-shaped cells, haphazardly scattered in abundant myxoid matrix. Cells of myxoid neoplastic tissue showed mainly a positive immunoreaction for fibrohistocytic markers and the absence either of muscular, neural of human progenitor cell antigens. Mitotic figures were fewer and cell proliferation rates were lower in myxoid as compared to those of typical dermatofibrosarcoma protuberans used as a control. The ultrastructural examination of myxoid areas revealed a prevalent Fibroblast-like cell population showing dilated cytoplasmic vesicles, sometimes containing glycosaminoglycans-like substances. The extent of myxoid changes together with the characteristic morphological, ultrastructural and immunohistochemical features confirm that myxoid dermatofibrosarcoma protuberans is a distinct variant of this fibrohistiocytic tumor to be considered in the differential diagnosis among myxoid tumors of the skin.     

纤维肉瘤型隆突性皮肤纤维肉瘤一例

摘要] 报告1例纤维肉瘤型隆突性皮肤纤维肉瘤(DFSP-FS)。患者女,36岁。背部反复丘疹、结节12年。曾经2次手术切除。组织病理检查示瘤细胞排列呈涡纹状,形态较一致,呈梭形,部分区域内瘤细胞排列成鱼骨状,异型性及分裂相均较前者为高。免疫组化检查示涡纹状结构区域内瘤细胞CD34弥漫强阳性,鱼骨状区域仅个别细胞CD34表达阳性。诊断：纤维肉瘤型隆突性皮肤纤维肉瘤。行广泛切除术,术后9个月,未见复发。     

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.     

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.     

Leiomyomatous nodules and bundles of vascular origin in the fibrosarcomatous variant of dermatofibrosarcoma protuberans

We report 5 cases of the fibrosarcomatous variant of dermatofibrosarcoma protuberans, 4 of which presented a morphologic change of intraneoplastic blood vessels not previously recognized. This change consisted of focal proliferation of smooth muscle cells, resulting in hypertrophy, generally eccentric, of vascular walls with reduction and collapsing of vascular lumina. In 3 cases the proliferation was so intense it formed leiomyomatous nodules and bundles. This proliferation may originate in the smooth muscle cells of the vessel walls either by means of a hyper-plastic mechanism or in the pericytes via a line of differentiation leading to mature smooth muscle cells. In either case, we believe that it concerns a reactive process of the vessel walls very probably induced by adjacent neoplastic cells. The cases recently reported by Calonje and Fletcher as "myoid differentiation" of neoplastic cells in dermatofibrosarcoma protuberans (DFSP) may well be an expression of the same phenomenon, and therefore the presence of leiomyomatous areas in this tumor should not be used to support the theory of a fibroblastic/myofibroblastic line of differentiation for DFSP.     

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.     

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 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.     

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.     

Immunohistochemical studies of the histogenesis of dermatofibrosarcoma protuberans

Paraffin-embedded tissue sections taken from 16 patients with dermatofibrosarcoma protuberans were stained by means of the peroxidase-antiperoxidase technique using antibodies against S 100 protein, NSE, Leu 7, lysozyme, alpha-1 antitrypsin, alpha-1 antichymotrypsin, cytokeratin, desmin, vimentin, and factor VIII. Most of the tumor cells showed positive reactions to vimentin. Only 1-3% of the cells within the tumor area answered to the histiocytic markers lysozyme, alpha-1 antitrypsin, and alpha-1 antichymotrypsin. The remaining antibodies investigated did not react with the tumor cells. Our results support a fibroblastic, and contradict a neural or histiocytic, histogenesis of dermatofibrosarcoma protuberans.