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Results for “Dermatofibrosarcoma”

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PubMed1998

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 or 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.

Adult↗

PubMed1998

Composite tumor consisting of dermatofibrosarcoma protuberans and myxofibrosarcoma.

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.

Aged↗

PubMed1997

Atrophic pigmented dermatofibrosarcoma presenting as infraorbital hyperpigmentation.

BACKGROUND: Pigmented dermatofibrosarcoma is a rare tumor of the skin and constitutes 1-5% of all dermatofibrosarcoma. Most cases present as polypoid multinodular growth. Occasional cases may be atrophic. We report an extraordinary case with progressive infraorbital atrophy. OBSERVATION: A 24-year-old woman came to us for a diffuse bluish atrophic lesion over the left infraorbital area. The lesion progressed gradually over 2 years. Histologic examination revealed mature spindle cell proliferation in the lower dermis and hypodermis. Interspersed were some heavily pigmented melanocytes. CONCLUSION: We report an unusual case of progressive bluish discoloration and atrophy of the infraorbital area. This is a rare manifestation of dermatofibrosarcoma.

Adult↗

PubMedDate not supplied

FNAC in the diagnosis of recurrent dermatofibrosarcoma protuberans of the forehead. A case report.

BACKGROUND: Dermatofibrosarcoma protuberans is a rare cutaneous soft tissue tumor of intermediate malignant potential with a characteristic tendency for recurrence. Metastases are unusual. This tumor usually occurs in the trunk and extremities and, infrequently, on the face and scalp. Its cytologic appearance on fine needle aspiration has only been rarely reported. It is characterized by numerous fibroblastlike cells that arrange as single cells or in clusters of spindle cells arrayed in a storiform pattern. CASE: A 42-year-old male presented with a one-year history of an enlarging left forehead mass (lateral brow) that was adjacent to an old surgical scar. Fine needle aspiration revealed a low grade spindle cell neoplasm morphologically identical to a dermatofibrosarcoma protuberans excised 15 years earlier, indicating tumor recurrence. CONCLUSION: Distinguishing dermatofibrosarcoma protuberans from other spindle cell tumors and fibrohistiocytic lesions may pose significant challenges to the pathologist. However, in the appropriate clinical setting and applying strict diagnostic criteria, fine needle aspiration cytology is a reliable tool in establishing the diagnosis of this neoplasm.

Adult↗

PubMed1996

Dermatofibrosarcoma protuberans. CT findings with pathologic correlation in 6 cases.

PURPOSE: To characterize the CT findings of dermatofibrosarcoma protuberans. MATERIAL AND METHODS: CT and pathologic specimens of 6 patients with dermatofibrosarcoma protuberans (5 in the trunk and 1 in the leg) were retrospectively reviewed. RESULTS: CT clearly defined the tumors in the dermis and subcutaneous tissue with a characteristic, if not pathognomonic, protrusion. On postcontrast CT, 3 tumors showed marginal enhancement and central low density, while the others showed homogeneous enhancement. Pathologically, the marginal enhancement on CT corresponded to fibrosarcomatous changes, and the central low density to necrosis. The tumors with homogeneous enhancement had no sarcomatous changes. CONCLUSION: CT allows detection and precise anatomic localization of dermatofibrosarcoma protuberans. Marginal enhancement with central low density on CT may suggest a relatively poor prognosis.

Adult↗

PubMedDate not supplied

Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children's Research Hospital.

The clinical characteristics and outcome of children with dermatofibrosarcoma protuberans (DFSP) treated at a single institution over a 34-year period were reviewed. A retrospective chart review identified five children with dermatofibrosarcoma protuberans. The diagnosis was confirmed in all cases by one of the authors. Age; sex; clinical group; tumor invasiveness and size, nodal status, and metastases (TNM stage); therapy; and outcome were retrieved from the medical records of these patients. The median age at diagnosis was 12.5 years. There were four boys, and all tumors arose in the extremities or trunk. All children underwent at least two surgical procedures to achieve negative or surgically microscopic positive margins. In addition, one patient received adjuvant radiotherapy for microscopically positive margins. All patients survive free of disease 1 month to 16.5 years after diagnosis. Dermatofibrosarcoma protuberans is rare among children and adolescents. Recognition of this pathologic entity is important for its proper treatment. Surgical resection (with 3-cm margins) is currently the standard therapy for DFSP.

Adolescent↗

PubMed1999

[Dermatofibrosarcoma protuberans: authors' experience].

Dermatofibrosarcoma protuberans is an uncommon slow-growing cutaneous neoplasm. Surgical excision is an effective therapeutic approach although a significant number of local recurrences has been documented in the literature. In the present paper the Authors report 3 cases of dermatofibrosarcoma protuberans. All cases have been surgically treated with a wide local excision extended up to 3 cm from the tumor borders. No local recurrence has been reported. The Authors conclude that the surgical technique employed could reduce the local recurrence rate of dermatofibrosarcoma protuberans.

Abdomen↗

PubMed2003

[Good response to treatment with the selective tyrosine-kinase inhibitor imatinib in a patient with metastatic dermatofibrosarcoma protuberans].

A 37-year-old man was diagnosed with metastatic dermatofibrosarcoma. Dermatofibrosarcoma protuberans is a rare skin tumour with a strong tendency for infiltrative growth, resulting in a high rate of local recurrences; metastatic disease develops in approximately 5% of patients, especially in the lungs. On the basis of some recent publications about the use of imatinib in patients with dermatofibrosarcoma, the patient was prescribed this drug. The treatment resulted in a partial response. Imatinib is the first registered selective tyrosine-kinase inhibitor for treatment of chronic myeloid leukaemia. The inhibition of tyrosine kinase interrupts the proliferative signal transduction.

Adult↗

PubMed1996

Dermatofibrosarcoma protuberans treated with Mohs micrographic surgery: cure rates and surgical margins.

BACKGROUND: Dermatofibrosarcoma protuberans is an uncommon malignant tumor of the skin with a frequent tendency to recur after standard surgical excision. This study assesses the degree of subclinical tumor extension and evaluates the cure rate and tissue conservation abilities of Mohs micrographic surgery. METHODS: Twenty-four patients with dermatofibrosarcoma protuberans underwent Mohs micrographic surgery. Surgical margins and clinical outcome were evaluated and compared with the results of standard surgical treatment in the medical literature. RESULTS: Twenty-six Mohs micrographic surgical procedures were performed on 24 patients. Eighty-five percent of the procedures were microscopically cleared with 2.5-cm margins, 69% with 2.0-cm margins, 50% with 1.5-cm margins, and 35% with 1.0-cm margins. Two tumors would have been inadequately excised if standard 3-cm had been used. The assessment of tissue conservation revealed a mean of 43.0 cm(2) of tissue spared in a subset of seven tumors in functionally or cosmetically critical locations. Two tumors were recurrent following MMS and are detailed as case reports. CONCLUSION: The variability of subclinical tumor extension in dermatofibrosarcoma protuberans is confirmed. The ability of Mohs micrographic surgery to minimize surgical margins, preserve cosmetically and functionally vital tissue, and yield high cure rates is confirmed.

Adult↗

PubMed1996

Fibrosarcomatous dermatofibrosarcoma protuberans with myofibroblastic differentiaion: a histologically distinctive variant [corrected].

We report two examples of dermatofibrosarcoma protuberans with fibrosarcomatous change, in which the fibrosarcomatous component of the tumor demonstrated focal myofibroblastic differentiation. This was recognized by light microscopy as plump myoid cells associated with abundant extracellular collagen production, immunohistochemically by positive staining for smooth muscle and muscle specific actin, and ultrastructurally by subplasmalemmal filament aggregates and micropinocytotic vesicles. Myofibroblastic differentiation has not been described before in dermatofibrosarcoma protuberans with fibrosarcomatous change and because of a lack of information concerning the immunohistochemical profile of this variant of DFSP its incidence is unknown. Focal myofibroblastic differentiation, as demonstrated in the fibrosarcomatous regions of these two tumors, might support the proposed dermal fibroblastic origin for dermatofibrosarcoma protuberans with fibrosarcomatous change.

Adult↗

PubMed1998

[Giant cell fibroblastoma associated with Darier-Ferrand dermatofibrosarcoma in an adult].

INTRODUCTION: Giant cell fibroblastoma is a rare mesenchyma tumor of childhood having many similarities with dermatofibrosarcoma protuberans in adults. OBSERVATION: We report the case of a 28-year-old woman presenting a subcutaneous inter-mammary mass associating both tumors. Immunohistochemistry showed an expression of CD 34 only by dermatofibrosarcoma protuberans cells. DISCUSSION: It is important to stress: the rarity of this association, the difficulty to confirm the diagnosis and to establish the links between these two tumors: simple association (as in our case), transformation or recurrence of giant cell fibroblastoma in dermatofibrosarcoma protuberans.

Adult↗

PubMed1997

A Case of Dermatofibrosarcoma Protuberans in the Skin over the Breast.

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing but locally aggressive, fibrous tumor that has a high rate of local recurrence after surgical resection. This tumor occurs most commonly in the trunk and proximal extremities. In this report we present a case of dermatofibrosarcoma protuberans in the skin over the breast which was re-excised after pathological diagnosis, considering cosmetic aspects. Only three other cases of dermatofibrosarcoma protuberans of the anterior chest wall have been encountered in our hospital, and all of these cases were male. The presence of this tumor in the skin over the breast appears to be rare in females. Careful complete resection is recommended for this type of tumor.

Journal Article↗

PubMed1991

Dermatofibrosarcoma protuberans of the vulva.

Dermatofibrosarcoma protuberans of the vulva is an uncommon low-grade sarcoma of dermal origin. Although wide local excision is the treatment of choice, microscopic tumor projections beyond the central tumor nodule explain the tumors' propensity for local recurrence. Frozen sections of margins may be useful to ensure complete resection. The following report contributes two additional patients with this uncommon neoplasm. Notably, one of these two had a fibrosarcomatous area within the dermatofibrosarcoma protuberans. This is the second reported case of a fibrosarcoma arising in a dermatofibrosarcoma protuberans of the vulva.

Adult↗

PubMed1987

Coexistent orbital dermatofibrosarcoma protuberans and bilateral lymphoid hyperplasia.

A 72-year-old man had secondary orbital involvement with dermatofibrosarcoma protuberans 36 years after removal of the initial tumor on his forehead. In addition, multiple bilateral inferior orbital masses were present, which on pathologic examination proved to be reactive lymphoid hyperplasia. Serum immunoelectrophoresis revealed polyclonal elevations of IgG and IgA. Dermatofibrosarcoma protuberans is part of the spectrum of fibrohistiocytic tumors that also includes atypical fibroxanthoma and benign and malignant fibrous histiocytoma. The exact cell of origin of dermatofibrosarcoma protuberans is controversial, though immunohistochemical study of our specimen supports the fibroblast. The coexistence of this rare orbital tumor with noncontiguous reactive lymphoid hyperplasia is unique, and points out the fact that multiple orbital masses may indicate more than one underlying disease process.

Aged↗

PubMed1988

Pigmented dermatofibrosarcoma protuberans (Bednar tumour): melanocytic colonization or neuroectodermal differentiation? A clinicopathological and immunohistochemical study.

Approximately 5% of cases of dermatofibrosarcoma protuberans contain dendritic melanocytes; such lesions are often known as Bednar tumours. These neoplasms have received little attention in the literature but seem to show no great clinicopathological differences from conventional dermatofibrosarcoma protuberans except for the presence of melanocytes. The existence of such tumours, combined with ultrastructural evidence, has led some leading authors to regard them all as being of neuroectodermal origin. Seven examples of the pigmented variant are presented herein, of which six have been studied immunohistochemically and one has been examined ultrastructurally. Except for the presence of melanocytes in each tumour, no evidence of neuroectodermal (in particular perineural fibroblastic) differentiation has been demonstrated. The histogenesis of dermatofibrosarcoma protuberans and its pigmented variant is discussed. The possibility that the pigmentation may simply reflect secondary melanocyte colonization from the epidermis should be considered.

Adolescent↗

PubMedDate not supplied

Dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is an intermediate-grade sarcoma that arises in the dermis of the trunk, proximal extremities, and less commonly, in the head and neck area of adults. Dermatofibrosarcoma protuberans has a deceptively innocuous early clinical appearance and generally exhibits an indolent course for years before entering a rapid growth phase. Its locally invasive behavior results in early recurrence if prompt, wide excision is not performed. Dermatofibrosarcoma protuberans rarely metastasizes through the bloodstream or, less often, to locoregional lymph nodes after multiple local recurrences. Fibrosarcomatous areas within the tumor result in a more aggressive course. Although wide excision is the mainstay of therapy, recent trials using the Mohs' surgical approach suggest that this technique may have a role in the future treatment of this tumor.

Diagnosis, Differential↗

PubMed1976

[Dermatofibrosarcoma protuberans].

Observations were conducted on 26 patients with protruding dermatofibrosarcoma of the skin. Histological and histochemical characteristics, as well as the mitotic regimen of these tumours, were investigated. Protruding dermatofibrosarcoma is a fibroblastic tumour characterized by a slow growth, usually a big size, persistent multiple reoccurrence, late metastases into the lymphatic nodes and lungs. No regularities concerning periods and frequency of developing of reoccurrences could be established. Clinico-anatomical collations failed to show correlation between the mitotic activity of dermatofibrosarcomas and the degree of their malignancy and rate of growth of the tumour.

Adult↗

PubMed1978

Dermatofibrosarcoma protuberans with lymph node and pulmonary metastases.

Dermatofibrosarcoma protuberans is a locally aggressive histiocytic tumor that, on rare occasions, may metastasize to regional lymph nodes or even to distant sites. We report a dermatofibrosarcoma protuberans with lymph node involvement in a 27-year-old woman. This tumor recurred repeatedly after local excisions, and ten years after the first resection, widespread pulmonary metastases developed in the patient and she died four years later. More than 400 patients with dermatofibrosarcoma protuberans have been reported in the literature; including the present case, five of these patients had lymph node metastases, 17 patients had hematogenous spread, and three had both lymphatic and blood-borne metastases.

Adult↗