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 91 records · Page 5Linked to original sources

PubMed2004

A case of a large dermatofibrosarcoma protuberans successfully treated with radiofrequency ablation and transcatheter arterial embolization.

We present a large dermatofibrosarcoma protuberans on a 52 years-old woman's back that was successfully treated with transcatheter arterial embolization and radiofrequency ablation. The tumor developed on her back at the age of 14. Surgical treatment was not complete, and it relapsed at age 17 and again at age 24. The tumor enlarged to over 20x20x10 cm and flooded intermittently. The tumor cells with atypical nuclei were CD34 positive and proliferated in a storiform pattern. These findings were compatible with dermatofibrosarcoma protuberans. She was treated with blood transfusion for severe anemia. Two large feeding arteries were embolized, and the bottom of the tumor was treated with radiofrequency ablation. The tumor reduced in volume by more than 50%. It was then surgically removed and reconstructed with a free mesh skin graft. The combination pretreatment enabled radical resections of a large DFSP without severe complications.

Biopsy, Needle↗

PubMed2006

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.

Dermatofibrosarcoma↗

PubMed1995

Dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor of the skin, the diagnosis of which can be difficult not only clinically but also histologically. The tumor has a low metastatic potential, but tends to recur locally if removal is incomplete. METHODS: We reviewed the clinical and histologic features of 19 cases of DFSP, seen at the Department of Dermatology (14 cases) and Department of Surgery (5 cases) at the Hull Royal Infirmary between 1975 and 1992. RESULTS: There were only 19 cases of DFSP diagnosed from a population of 500,000 over a period of 17 years. None of the cases were diagnosed clinically prior to histologic diagnosis. The highest incidence was in the 4th decade of age. The most frequent site was on the head and neck. None of the lesions metastasized but there were a total of eight incidents of local recurrence in five patients. Histologically all the cases showed presence of storiform-pattern spindle-shaped cells and new collagen formation. CONCLUSIONS: Dermatofibrosarcoma protuberans is a soft tissue tumor that is often not recognized clinically due to its rarity and variable appearance. Local recurrence is frequent. A wide surgical excision is the treatment of choice.

Adolescent↗

PubMed1998

Sarcomatous dermatofibrosarcoma protuberans metastasized to the lung: preservation of CD34 expression in tumor cells.

A case of metastatic dermatofibrosarcoma protuberans (DFSP) in a 47-year-old woman is presented. Dermatofibrosarcoma protuberans occasionally recurs, but rarely metastasizes. The patient underwent local removal of the nuchal tumor by a general practitioner, followed by a rapid recurrence. She underwent total removal of the tumor and a diagnosis of spindle cell sarcoma was made after an incisional biopsy was performed. This lesion had both a typical DFSP-like area and a fibrosarcoma (FS)-like area. After 7 years, an abnormal lung shadow was observed and a segmental lung resection was performed. Histologically, the lung tumor was similar to the FS-like area in the nuchal tumor. Confirming CD34 expression in the tumor cells, this lung tumor was diagnosed as metastatic DFSP. Usually CD34 expression is unique to DFSP but almost negative in FS-like areas. In the present case, the FS-like area in the nuchal tumor showed decreased CD34 reactivity, as previously reported, but the FS-like area in the metastatic tumor still widely preserved CD34 expression. The presented case suggests that the FS-like area in DFSP is histogenetically different from typical FS or malignant fibrous histiocytoma.

Antigens, CD34↗

PubMed1994

Immunohistochemical margin control applied to Mohs micrographic surgical excision of dermatofibrosarcoma protuberans.

BACKGROUND: Surgical treatment of dermatofibrosarcoma protuberans has a high rate of recurrence presumably secondary to persistent residual tumor. Recently an antigenic marker, CD34, has demonstrated specificity for this tumor. OBJECTIVE: To improve the microscopic detection of dermatofibrosarcoma protuberans tumor elements in Mohs micrographic surgical sections by incorporating immunohistologic staining. METHODS: Standard Mohs micrographic surgical technique was used, coupled with standard immunohistochemical procedures using an antibody to the CD34 antigen. RESULTS: Immunohistochemical staining with anti-CD34 of Mohs micrographic sections clearly delineated the extent of the tumor elements. CONCLUSIONS: We anticipate that the application of this immunohistochemical-modified Mohs surgical technique will further enhance the detection of insidious portions of tumor thereby enhancing removal and reducing recurrence.

Adolescent↗

PubMed1998

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 hyperplastic 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.

Actins↗

PubMed1998

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.

Aged↗

PubMed2002

Reconstruction of a scalp and skull defect with free latissimus dorsi myocutaneous flap following dermatofibrosarcoma protuberans.

A 53-year-old male underwent more than 10 surgical treatments over 14 years, including a simple excision and local flap transfer, after recurrences of dermatofibrosarcoma protuberans of the head. Clinical results indicated that simple excision of dermatofibrosarcoma protuberans should not be performed as initial treatment. Instead, free flap transplantation, which permits a wide excision and complete reconstruction, should be the first treatment option.

Dermatofibrosarcoma↗

PubMed2002

Dermatofibrosarcoma protuberans: MR imaging features.

OBJECTIVE: The objective of our study was to describe the MR imaging features of 10 cases of histologically confirmed dermatofibrosarcoma protuberans. CONCLUSION: MR imaging is useful in identifying the extent and location of dermatofibrosarcoma protuberans. Although most cases of this tumor are superficial and well defined, we have shown three cases in which the tumor was in a deep location and one case in which the tumor was ill defined in appearance. Knowledge of the variable MR imaging appearances of these tumors may aid in the diagnosis of difficult or atypical cases.

Adult↗

PubMedDate not supplied

[Dermatofibrosarcoma protuberans: case report].

OBJECTIVE: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare, slow-growing, fibrohistiocytic sarcoma of intermediate malignancy. The tumor appears most frequently on the trunk and extremities as a slow-growing indurated plaque or nodule. It appears mostly between the second and fifth decades of life. METHODS: This is a case presentation of a 59-year-old woman with a 10 x 12 cm dermal erythematous plaque on the anterior chest wall. RESULTS: The patient has a two year history of painless tumor on the chest. The lesion started as a small erythematous plaque and gradually increased in size. At that time the histologic examination revealed a benign fibrous histiocytoma. Less than two years later the lesion was firm, painless to palpation and movable over deeper structures. The second histologic examination revealed a myxoid liposarcoma. The third incisional biopsy was diagnostic for DFSP showing a dermal tumor consiting of a storiform pattern with cartwheel-like cells arranged around collagenous hubs. The patient underwent a standard surgical treatment. CONCLUSION: We report an interesting case of dermatofibrosarcoma protuberans where limitations of standard vertical histology section resulted in postponed conformation of the diagnosis.

Dermatofibrosarcoma↗

PubMed2000

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.

Acquired Immunodeficiency Syndrome↗

PubMed2001

Supernumerary ring chromosome in a Bednar tumor (pigmented dermatofibrosarcoma protuberans) is composed of interspersed sequences from chromosomes 17 and 22: a fluorescence in situ hybridization and comparative genomic hybridization analysis.

Cytogenetic analysis of Bednar tumor (pigmented dermatofibrosarcoma protuberans) has not been reported previously. Here, we report the identification of a supernumerary ring chromosome in a Bednar tumor by chromosome painting with fluorescence in situ hybridization (FISH) and comparative genomic hybridization (CGH). Chromosome painting with FISH demonstrated that the supernumerary ring chromosome was composed of discontinuous, interwoven sequences from chromosomes 17 and 22. Amplification of chromosomes 17 and 22 sequences was confirmed by CGH. These results indicate that Bednar tumor and dermatofibrosarcoma protuberans are characterized by the same chromosomal features. To our knowledge, this is the first report that the ring chromosome in Bednar tumor is composed of amplified material from chromosomes 17 and 22.

Chromosome Banding↗

PubMed2001

[Contribution of imaging to the diagnosis of Darier-Ferrand's cranio-facial dermatofibrosarcoma. Report of 2 cases].

Dermatofibrosarcoma protubans of Darier-Ferrand is observed in about 0.1% of al malignant skin tumors. Head and face localization in uncommon, estimated to account for about 1 to 10% of all lesions. Relapse is frequent and metastasis in uncommon. Diagnosis is often made late. Surgery is the basic treatment. We report two cases of dermatofibrosarcoma of Darier-Ferrand with head and face localizations. In one case, the tumor extended inside the skull and spread to the superior longitudinal sinus. This is the only such reported case found in the literature. CT scan is more useful for studying the bony tables and the cranial content. MRI provides precision for soft tissue tumors and angio-MRI is particularly useful for analysing with accuracy vascular relations of the tumor.

Adult↗

PubMed2002

[Dermatofibrosarcoma in children. Report of 7 Tunisian cases].

Dermatofibrosarcoma (DFS) protuberans is a rare mesenchyma tumor in childhood characterised by slow growth. We report the result of a retrospective study of 7 childhood (4 males) cases of through a fifteen year period. The mean age was 13 years. 4 Tumors were (DFS) located on the limbs: 2 on the trunk: and on the scalp. Clinical types were multinodular and infiltrated in 3 cases, nodular in two cases and ulcerated with budding also in two cases. The mean delay between diagnostic and the appearance of the tumor was about 24 months for 4 initial tumors. Histological features were typical in all cases. We report 3 recurrences of dermatofibrosarcoma arising few months after surgical excision of an initial tumor, in one case the tumor was diagnosed histocytofibroma. Six patient underwent surgical excision with wide margins, and in one case the margins were considered borderline. No recurrences were reported, the follow-up was between 17 months to 9 years. It's a rare tumor in childhood, 160 cases were reported. The clinical and evolutive mode were entirely comparable to the adult cases. However the initial appearance as benign atrophic or indurated plaque is more frequent in childhood, but this appearance wasn't noted in our series.

Adolescent↗

PubMed2003

[Case report: Dermatofibrosarcoma protuberans metastatic to lung without local recurrence].

Dermatofibrosarcoma protuberans is a rare skin tumor which constitutes 6% of soft tissue sarcomas. Typically, it has a superficial location and a locally aggressive character. Although it has a relatively high recurrence rate, the rate of distant metastasis is about 3%. Furthermore, it rarely metastasizes without local recurrence. Here we present a case with dermatofibrosarcoma protuberans with distant metastasis occurring seven years after the local excision of the primary tumor and without any local recurrence.

Dermatofibrosarcoma↗

PubMed2004

Dermatofibrosarcoma protuberans and breast cancer: genetic link or coincidental association?

Dermatofibrosarcoma protuberans is a low-grade cutaneous neoplasm originating from the dermis with a high propensity for local recurrence. These malignancies are classified as soft tissue sarcoma although regional or distance metastatic spread is rare. Germ-line mutations of p53 have been described with a rare autosomal dominant syndrome in patients with soft tissue sarcoma and breast adenocarcinoma (Li Fraumeni's syndrome). The described case illustrates the rare occurrence of dermatofibrosarcoma protuberans (DFSP) and breast adenocarcinoma with an emphasis on the possibility of a molecular or genetic association.

Adult↗

PubMed2005

[Dermatofibrosarcoma protuberans of Darier and Ferranda study of 28 cases in adult].

Dermatofibrosarcoma protuberans of Darier and Ferrand is a mesenchymatous skin tumor of intermediate malignant potential, characterised by slow growth, local malignancy and a high rate of recurrence. It is a rare tumor, accounting for approximately 0.1% to 0.18% of all malignant skin tumors. Peak incidence of the disease is between 20 to 40 years of age. We report a retrospective study of 28 cases of dermatofibrosarcoma protuberans. We reviewed the clinical features, histopathological and immunohistochemical aspects of this tumor.

Adolescent↗

PubMed2006

[Dermatofibrosarcoma protuberans of the breast].

Dermatofibrosarcoma protuberans is a superficial mesenchymal cutaneous tumor of essentially local malinancy, located more often in the trunk and extremities. This tumor is usually diagnosed after multiple recurrences. We report two cases of rare mammary localisations of dermatofibrosarcoma protuberans in 2 patients aged 39 and 43 years respectively, the two lesions sized 35 x 45 mm were treated by wide excision and reconstruction with a dorsal flap; in the second case.

Adult↗