SEARCH · PubMed Health

Results for “Dermatofibrosarcoma”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources

PubMed1985

Dermatofibrosarcoma non-protuberans: description and report of five cases of a morpheaform variant of dermatofibrosarcoma.

Five cases of dermatofibrosarcoma are reported. All showed features typical of dermatofibrosarcoma protuberans except that in four cases, and a portion of the fifth case, no protusion of the tumor was noted clinically despite the rather advanced stage of growth of the tumor. These lesions resembled morphea or a morpheaform basal cell carcinoma clinically but could be recognized as "dermatofibrosarcoma non-protuberans" by physicians who had observed a previous case.

Adolescent↗

PubMed1998

Dermatofibrosarcoma protuberans with fibrosarcomatous areas. Molecular abnormalities of the p53 pathway in fibrosarcomatous transformation of dermatofibrosarcoma protuberans.

Fibrosarcomatous (FS) change in a rare, but well-known phenomenon encountered in dermatofibrosarcoma protuberans (DFSP), and an increased chance in an adverse outcome has been suggested in patients with DFSP having FS areas (DFSP-FS). As altered p53 pathway has been suggested as having a potential role in tumour progression, we analysed the p53 gene and p53 protein together with the p53-related protein mdm2 and p21Waf1 in 5 cases of DFSP-FS and 13 of DFSP to ascertain whether the p53 pathway correlates to the fibrosarcomatous transformation of DFSP. Three of the five DFSP-FSs overexpressed p53 protein immunohistochemically, and one of them had a "missense" mutation of the p53 gene without immunohistochemical overexpression of mdm2 or p21Waf1. The other two DFSP-FSs with p53 overexpression demonstrated increased labelling indices of both mdm2 and p21Waf1. The three DFSP-FS patients with overexpression of p53 protein had frequent local recurrences, ranging from 3 to 5 in number with increasingly short intervals (mean 4.5 years), while one of the other two had no recurrences and the other, only one. None of the 13 DFSPs showed any alterations in the p53 gene or overexpressions of p53, mdm2 and p21Waf1, except for one DFSP having a "silent" mutation of the p53 gene. Three DFSPs had local recurrences once or twice with longer intervals to recurrence (mean 10.3 years). Although the number of cases examined is limited, the results suggest that alterations in the p53 pathway, such as overexpression of p53 protein by a mutated gene and mdm2 overexpression, are involved in fibrosarcomatous transformation in a subset of fibrohistiocytic tumours and possibly correlated with its more locally aggressive behaviour than that without p53 alterations or ordinary DFSP.

Adolescent↗

PubMed1987

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.

Adult↗

PubMed2006

Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is a superficial low-grade sarcoma that rarely evolves into a high-grade fibrosarcoma. Dermatofibrosarcoma protuberans is genetically characterized by the unbalanced chromosomal t(17;22)(q21;q13), usually in the form of a supernumerary ring chromosome. The product of this chromosomal translocation is the chimeric gene COL1A1-PDGFB (collagen type I alpha I-platelet-derived growth factor beta), which is amplified at low levels in the ring chromosome. The aims of this study were to evaluate (1) whether genomic gains of this fusion gene occur during the clonal evolution of dermatofibrosarcoma protuberans into fibrosarcomatous dermatofibrosarcoma protuberans and (2) whether there is a difference between the number of genomic copies of COL1A1-PDGFB between classic dermatofibrosarcoma protuberans and dermatofibrosarcoma protuberans areas associated with fibrosarcomatous dermatofibrosarcoma protuberans. Eleven cases of fibrosarcomatous dermatofibrosarcoma protuberans with both dermatofibrosarcoma protuberans and fibrosarcomatous areas and 10 cases of classic dermatofibrosarcoma protuberans were studied. Genomic copies of COL1A1-PDGFB were evaluated by fluorescence in situ hybridization using a custom designed probe for the PDGFB locus on 4 mum thick paraffin-embedded tissue sections. Genomic gains of the COL1A1-PDGFB gene were observed in six (of 10) fibrosarcomatous dermatofibrosarcoma protuberans in the fibrosarcomatous areas when compared to the dermatofibrosarcoma protuberans areas of the same tumor (2-7 gene copies (median PDGFB copy gain, 2.8) versus 1-3 gene copies (median PDGFB copy gain, 1.7), respectively, P=0.004). Four fibrosarcomatous dermatofibrosarcoma protuberans did not show genomic gains of COL1A1-PDGFB fusion gene between the two areas. Essentially no difference in the copy number of COL1A1-PDGFB fusion gene was observed between dermatofibrosarcoma protuberans areas of classic dermatofibrosarcoma protuberans and dermatofibrosarcoma protuberans areas of fibrosarcomatous dermatofibrosarcoma protuberans (median PDGFB copy gain of 1.8 versus 1.7, respectively, P=0.36). Genomic gains of COL1A1-PDGFB fusion gene is possibly an oncogenic mechanism that is identified in the clonal evolution of a subset of dermatofibrosarcoma protuberans that evolves into fibrosarcomatous dermatofibrosarcoma protuberans. Since this finding was not observed in all cases of fibrosarcomatous dermatofibrosarcoma protuberans, other oncogenic mechanisms may be operating in this form of tumor progression. Copy number of COL1A1-PDGFB fusion gene in the classic dermatofibrosarcoma protuberans areas does not seem to be a major predisposing mechanism for fibrosarcomatous transformation.

Adult↗

PubMed2006

[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.

Adult↗

PubMed2002

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.

Cell Division↗

PubMed1998

Histogenetic relations between giant cell fibroblastoma and dermatofibrosarcoma protuberans. CD34 staining showing the spectrum and a simulator.

The authors describe three lesions that provide further evidence for a close, possibly histogenetic relation between giant cell fibroblastoma and dermatofibrosarcoma protuberans. The first case involves a dermatofibrosarcoma protuberans that contained a single giant cell fibroblastoma-like focus of multi-nucleate giant cells. A second tumor, a giant cell fibroblastoma, recurred 6 years later as a dermatofibrosarcoma protuberans. In the third lesion, there was a juxtaposition and co-mingling of dermatofibrosarcoma protuberans and giant cell fibroblastoma within the same primary lesion. In all cases, both the giant cell fibroblastoma areas and dermatofibrosarcoma protuberans areas stained positively with CD34. A fourth case, a dermatofibrosarcoma protuberans infiltrated skeletal muscle, creating giant cell fibroblastoma-like giant cell mimics--a result of skeletal muscle degeneration or atrophy with nuclear conglomeration. The latter giant cells failed to express CD34 but did show immunoreactivity with desmin. These findings support the concept that giant cell fibroblastoma and dermatofibrosarcoma protuberans probably represent a histologic spectrum of a single CD34 positive (perhaps, dermal dendrocytic) neoplasm, a conclusion supported by a recently cloned t(7;22) breakpoint demonstrated in both neoplasms.

Adolescent↗

PubMed1997

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 x 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.

Aged↗

PubMed1995

Atrophic variants of dermatofibroma and dermatofibrosarcoma protuberans.

Dermal atrophy of more than 50% of the locoregional dermis may be the predominant histopathological feature in dermatofibroma and dermatofibrosarcoma protuberans. This may cause diagnostic difficulties. In the present study 26 cases of atrophic dermatofibroma were compared with three cases of atrophic dermatofibrosarcoma protuberans. Clinically, both conditions mostly occurred on the (upper) trunk of females. While atrophic dermatofibroma usually presented as a reddish, umbilicated lesion (0.5-1cm), often suspected to be a basal cell carcinoma, atrophic dermatofibrosarcoma protuberans showed irregularly arranged tan-brown plaques (3-6 cm). Histologically, atrophic dermatofibroma showed a regular silhouette with a smooth nodular (9/26) or scalloped lower margin with an intervening lace-like pattern of superficial fatty tissue infiltration (17/26) and variable sclerosis: atrophic dermatofibrosarcoma protuberans showed a deep, irregular infiltration of fatty tissue in a lacelike/honeycomb and/ or multilayered pattern, but no sclerosis. Immunohistochemically, atrophic dermatofibroma was mostly negative with QBEnd 10 (CD34;24/26), variably positive for factor XIIIa (20/26) and metallothionein (11/26). Labelling for factor XIIIa and metallothionein was usually seen in 'early' (metabolically active) lesions, while 'late' sclerotic ones were negative. In contrast to atrophic dermatofibroma all three atrophic dermatofibrosarcoma protuberans showed a consistently uniform profile: CD34 positive, factor XIIIa and metallothionein negative. Our study delineates atrophic dermatofibroma and atrophic dermatofibrosarcoma protuberans as distinct entities clearly distinguishable from each other by clinicopathologic criteria.

Adult↗

PubMed2005

Management of scalp dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans is a relatively rare, locally aggressive soft tissue tumor. Less than 5% of these tumors are located on the scalp; however, the recurrence rates for this region are high compared with those of more common locations, such as the trunk and proximal extremities. Standards for the management of dermatofibrosarcoma protuberans in general have been established in the literature, including wide local excision and Mohs micrographic surgery. OBJECTIVE: To describe optimum treatment options for primary and recurrent dermatofibrosarcoma protuberans of the scalp based on cases from Roswell Park Cancer Institute (RPCI) and from the literature. METHODS: Five cases from RPCI and 23 cases from the literature were reviewed. Age, sex, history, tumor size and level of invasion, treatment, reconstruction method, and follow-up were compiled from each case. RESULTS: Twenty-eight cases of scalp dermatofibrosarcoma protuberans were found; 13 were primary, 8 were recurrent, and 3 had persistent positive margins. Fifty-eight percent had classic histologic findings; seven tumors invaded beyond the periosteum. Twenty tumors were treated by surgical excision, whereas eight tumors were treated with Mohs micrographic surgery, requiring between two and four layers. Advanced surgical reconstruction was used in most cases to close the defects. Four patients experienced one tumor recurrence, whereas three other patients had several local recurrences. One patient died of his disease, and one died during surgical treatment of dermatofibrosarcoma protuberans. CONCLUSIONS: Mohs micrographic surgery or a modified version may be the best treatment option for scalp dermatofibrosarcoma protuberans to ensure complete removal of tumor and minimize recurrence. Surgeons should be prepared for advanced reconstruction following tumor removal.

Adolescent↗

PubMed1996

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.

Adult↗

PubMed1998

Atrophic dermatofibrosarcoma protuberans: a case report and review of the literature.

Dermatofibrosarcoma protuberans is not a difficult tumor to recognize because of its characteristic clinical appearance, although some unusual variants have been reported. We describe the atrophic variant of dermatofibrosarcoma protuberans in a 21-year-old female. The lesion was a smooth-surfaced, oval depression on the left subclavicular area, with a violaceous plaque at the center. The suspected clinical diagnosis did not include fibrous tumors, although histological examination showed the typical picture of dermatofibrosarcoma protuberans. Positive CD34 staining was also helpful in the diagnosis. We review 14 cases of the atrophic variant of dermatofibrosarcoma protuberans in the literature. Dermatologists should be aware of this uncommon but characteristic appearance of atrophic dermatofibrosarcoma protuberans.

Adolescent↗

PubMed2004

Nasolacrimal duct obstruction secondary to dermatofibrosarcoma protuberans.

OBJECTIVE: To describe a patient wtih dermatofibrosarcoma protuberans causing a nasolacrimal duct obstruction (NLDO). DESIGN: Case report. PARTICIPANT: A 33-year-old female with an NLDO. RESULTS: We report a patient with NLDO as the presenting sign of a rare tumor of the skin, dermatofibrosarcoma protuberans. The patient presented with epiphora, and after dacryocystorhinostomy was done, histopathology revealed dermatofibrosarcoma protuberans. The clinical course, magnetic resonance imaging, and histopathology are discussed. CONCLUSION: Obstruction of the lacrimal drainage system can be seen with dermatofibrosarcoma protuberans.

Adult↗

PubMed2002

Dermatofibrosarcoma protuberans treated by micrographic surgery.

Dermatofibrosarcoma protuberans is an uncommon cutaneous tumour which rarely metastasises. However, local recurrence following apparently adequate surgical excision is well recognised, presumably as a result of sub-clinical contiguous growth, for which micrographically controlled excision would be a logical treatment. A retrospective study of all patients treated by micrographic surgery, from April 1995-March 2000, at a tertiary skin oncology centre. Twenty-one patients (11 males), age 14 to 71 years with dermatofibrosarcoma protuberans on the trunk (10 patients), groin (four), head and neck (four), and limbs (three) were treated. In 15 patients one micrographic layer cleared the tumour, and four were cleared with two layers. For one patient the second stage was completed by conventional excision guided by positive margins. Another patient with a multiply recurrent perineal dermatofibrosarcoma protuberans, not cleared in one area after two layers, died from a pulmonary embolus before total clearance could be achieved. There was no correlation between tumour size and lateral excision margin. No recurrence was observed during the follow-up, from 21 to 80 months, median 47 months. The study provides further support for micrographic surgery as the treatment of choice for dermatofibrosarcoma protuberans.

Adolescent↗

PubMed1994

Giant-cell fibroblastoma with pigmented dermatofibrosarcoma protuberans component.

A unique case of giant-cell fibroblastoma that contained a pigmented dermatofibrosarcoma component (Bednár tumor) is described. The melanin pigmented dendritic cells found in the dermatofibrosarcoma component were round or spindle-shaped and had bipolar extensions. They were S-100 protein positive. Our case indicates the close histogenetic relationship between giant-cell fibroblastoma and dermatofibrosarcoma protuberans. The occurrence of the pigmented melanin-laden dendritic cells in our tumor further substantiates the theory that Bednár tumor is a pigmented variant of dermatofibrosarcoma protuberans.

Adult↗

PubMed1997

Surgical treatment of dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is a rare dermal tumor that recurs after inadequate primary treatment. In a retrospective study, we analyzed the outcomes of 117 patients (mean age 39 years) treated surgically for a dermatofibrosarcoma protuberans. In most cases (107 patients), surgery was performed according to a protocol of taking wide peripheral resection margins of 5 cm and by resecting a disease-free anatomic zone deep to the lesion. The mean follow-up was 61 months. The results suggest a difference in prognosis between patients treated primarily with wide initial resection and those referred secondarily with recurrent disease following previous treatment by narrow resection margins. There was no recurrent disease in the 66 patients treated primarily by wide peripheral (5 cm) and deep resection of the tumor. Of the 41 patients referred secondarily at the time of recurrence, 2 developed further local disease within a year, despite equally aggressive local treatments. Both patients eventually died of metastatic fibrosarcoma. We emphasize the value of a highly aggressive local resection in the primary treatment of dermatofibrosarcoma protuberans to minimize local recurrence and potential malignant transformation. Modern reconstructive techniques provide satisfactory solutions for defects of almost any size and composition. Our findings suggest that radical primary resection of dermatofibrosarcoma protuberans in conjunction with immediate reconstruction of the primary defect provides the best outcome for the patient.

Adult↗

PubMed2006

Imatinib mesylate in a patient with metastatic disease originating from a dermatofibrosarcoma protuberans of the scalp.

Dermatofibrosarcoma protuberans is a soft-tissue tumor that may recur locally and rarely causes metastases to vital organs. Dermatofibrosarcoma protuberans has specific chromosomal abnormalities involving the platelet-derived growth factor beta-chain locus that may render these tumors responsive to targeted therapy with the tyrosine kinase inhibitor imatinib mesylate. A patient with locally recurrent and metastatic dermatofibrosarcoma protuberans who had already undergone surgery 22 times was initially treated with imatinib mesylate 400 mg/day. The treatment dose was increased after 7 days to 400 mg twice daily. The patient was followed up for response and toxicity by physical examination and imaging studies, comprising computed tomography and fluorodeoxyglucose positron emission tomography. Clinical response could be demonstrated after the first month of treatment, and subsequent computed tomography and positron emission tomography documented a response to imatinib mesylate therapy. Our patient is now in sustained remission with minimal toxicity. We conclude that antitumor activity of metastatic dermatofibrosarcoma protuberans can be obtained with imatinib mesylate treatment with minimal side-effects.

Antineoplastic Agents↗

PubMed2006

Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior.

PURPOSE OF REVIEW: Dermatofibrosarcoma protuberans is a low-grade malignancy of the skin and subcutaneous tissues with low potential for the development of distant metastases. This tumor is characterized by infiltrative growth, and a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the gene platelet-derived growth factor B gene on chromosome 22. This review will examine recent data confirming the central role of surgery in managing this disease and new findings for the application of molecularly targeted therapy in patients with unresectable disease. RECENT FINDINGS: The largest published series of dermatofibrosarcoma protuberans cases has reported that surgery can lead to excellent local control in dermatofibrosarcoma protuberans in over 95% of cases. In patients with metastatic disease, or with locally advanced disease not suitable for surgical excision, inhibition of platelet-derived growth factor receptors with imatinib (Gleevec) can lead to striking clinical results, including complete responses in some patients. SUMMARY: The identification of platelet-derived growth factor B as a near universal translocation partner in chromosomal rearrangements in dermatofibrosarcoma protuberans has led to the successful application of therapy targeted at platelet-derived growth factor receptors. Although wide surgical excision remains the standard of care, patients with locally advanced disease not suitable for wide surgical excision or with metastatic disease can be managed with the platelet-derived growth factor receptors inhibitor imatinib with high probability of response.

Adult↗