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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↗
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↗
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↗
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↗
PubMed1994

Dermatofibrosarcoma protuberans: radiologic appearance.

OBJECTIVE: Dermatofibrosarcoma protuberans is a distinct clinicopathologic entity characterized by fibroblasts with a prominent storiform pattern. It accounts for about 6% of all soft-tissue sarcomas. The lesion is typically superficial, and the diagnosis can be suspected on the basis of the tumor's clinical appearance. Consequently, the imaging appearance of this entity is essentially unreported. Large lesions, however, can infiltrate the deep soft tissue and be confused with higher-grade sarcomas. We report the radiologic appearance in 11 cases of histologically proved dermatofibrosarcoma protuberans. MATERIALS AND METHODS: The radiologic images of 11 patients who had histologically verified dermatofibrosarcoma protuberans were retrospectively studied. The patients included eight males and three females 9-47 years old (mean, 33 years). Each had a soft-tissue mass that had been present for a variable period, from 3 months to 23 years. CT scans were available for review in six cases, MR images in four, radiographs in nine, bone scintigrams in three, and arteriograms in three. RESULTS: All lesions were unmineralized nodular masses involving the skin and subcutaneous adipose tissue. The size, in greatest dimension on imaging studies, was 3-22 cm. CT scans (six cases) showed a well-defined lesion with a distinct lobular or nodular architecture and tissue attenuation approximately equal to or greater than that of skeletal muscle. Moderate enhancement was seen on CT scans obtained after injection of contrast material. Arteriograms (three cases) showed mild to moderate hypervascularity. The MR appearance (four cases) was nonspecific; the lesions had prolonged T1 and T2 relaxation times. In one case, MR images showed multiple nodular lesions. Radiographs (nine cases) showed a soft-tissue mass or soft-tissue swelling without evidence of bone involvement or calcification. Bone scintigrams (three cases) showed increased accumulation of tracer. CONCLUSION: The radiologic appearance of dermatofibrosarcoma protuberans is typically that of an unmineralized, nodular soft-tissue mass involving the skin and subcutaneous adipose tissue. CT scans or MR images are well suited to show this location, the relation of the lesion to underlying structures, and the distinct lobular or nodular architecture. Arteriograms will show mild to moderate hypervascularity and bone scintigrams will show increased accumulation of radiopharmaceutical.

Adipose Tissue↗
PubMed1979

Nature of collagen in dermatofibrosarcoma protuberans.

The nature of collagen from 2 cases of dermatofibrosarcoma protuberans was studied. For this purpose, the tumor tissue was carefully separated from adjacent normal dermis. The collagen types comprised in the tumor were identified by CM-cellulose chromatographic and SDS-gel electrophoretic analysis of the component alpha-chains. Semiquantitative evaluation of the relative type III content was established by separation of the cyanogen bromide peptides on gels of 12% polyacrylamide in SDS. These studies showed that dermatofibrosarcoma protuberans contains alpha 1(I)-, alpha 2-, and alpha 1(III)-chains as well, and corresponding type I- and type III-related CNBr peptides. Comparing the collagen from dermatofibrosarcoma protuberans to that of normal skin, the relatively increased type III content in the case of dermatofibrosarcoma protuberans becomes apparent.

Adult↗
PubMed1975

[The ultrastructure of fibrous and histiocytic skin tumors (dermatofibroma, dermatofibrosarcoma protuberans, fibroxanthoma, and histiocytoma) (author's transl)].

A report on the ultrastructure of the following skin tumors: dermatofibroma (7 cases), histiocytoma (4 cases), dermatofibrosarcoma protuberans (1 case), and fibroxanthoma (1 case). Because of their different ultrastructural characteristics a division into two groups is possible: one group of tumors would include dermatofibroma, dermatofibrosarcoma protuberans, and fibroxanthoma, where the formation of fibers is extensive and fibroblasts are suggested for its histogenesis. These types of tumor differ from histiocytoma, the cellular character of which is determined by a very pronounced phagocytosis and storage of lipid and hemosiderin; a histogenesis from histiocytes is probable. The nuclei of dermatofibroma and histiocytoma show a simple surfacedevelopment, whereas the nuclei of dermatofibrosarcoma protuberans and fibroxanthoma show multisegmented nuclei ("labyrinth nuclei"). In the case of dermatofibrosarcoma protuberans this kind of nuclear segmentation seems to be a morphologic correlate of its semimalignancy. Based on ultrastructural analysis a classification of these fiber-producing tumors into "fibrous histiocytomas" is not justified.

Adolescent↗
PubMed1990

Dermatofibroma extending into the subcutaneous tissue. Differential diagnosis from dermatofibrosarcoma protuberans.

When dermatofibromas are composed predominantly of fibroblasts and extend into the subcutaneous tissue, it may be difficult to distinguish them from dermatofibrosarcoma protuberans. Because the patterns of extension of dermatofibroma have not been well characterized, we studied 185 cases of the fibrous variant of dermatofibroma with extension into the subcutaneous tissue and 40 cases of dermatofibrosarcoma protuberans. Dermatofibromas had two main patterns of extension into subcutaneous tissue. One pattern, seen in 133 of 185 cases (72%), consisted of irregular extension into the subcutaneous tissue in a vertical or radial fashion, predominantly along the septa, which appeared wedge-shaped. The other pattern, seen in 52 of 185 cases (28%), showed a smooth and well-demarcated deep margin that bulged into the subcutaneous tissue. Dermatofibrosarcoma protuberans also had two main patterns of extension into the subcutaneous tissue. In one pattern, seen in 12 of 40 cases (30%), slender spindle-shaped cells extended along septa and between fat cells in a classic honeycomb or lacelike pattern. The other pattern observed in 24 of 40 cases (60%) exhibited a distinct multilayered pattern in which the bundles of slender spindle-shaped cells showed a predominantly parallel orientation to the skin surface. In four cases (10%), a mixture of both patterns was present. We conclude that the patterns of extension of dermatofibroma into the subcutaneous tissue are different from the patterns of dermatofibrosarcoma protuberans.

Diagnosis, Differential↗
PubMed1988

[Pigmented dermatofibrosarcoma protuberans (Bednar's tumor)].

Pigmented dermatofibrosarcoma protuberans, first described by Bednar under the term storiform neurofibroma, is an infrequent neoplasm accounting for 1 to 5% of cases of dermatofibrosarcoma protuberans. Bednar's tumor is composed of spindle shaped cells arranged in a distinctive storiform or carthwheel pattern, and melanin-containing dendritic cells scattered within the tumor. The differential diagnosis with (non-pigmented) dermatofibrosarcoma protuberans is based on the presence of this population of pigmented cells. Two cases of pigmented dermatofibrosarcoma protuberans (Bednar's tumor) are reported here in, and a discussion follows on the clinicopathological features of this neoplasm and the different hypotheses on its histogenesis.

Adult↗
PubMed1994

Dermatofibrosarcoma with lymph node involvement.

Dermatofibrosarcoma protuberans is a slow-growing, locally aggressive, fibrous tumor that, on rare occasions, metastasizes to distant sites or regional lymph nodes. We have found 28 patients with metastasis in the literature; 9 of these patients had lymph node metastasis. In this report we present a case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to inguinal lymph nodes appearing 10 months after wide excision and skin grafting of the primary lesion. The clinical and pathological features of this case are presented. Although, along with our patient, 10 cases of regional lymph node metastasis are not justification for regional lymph node dissections, they do emphasize the need for regional lymph node examinations in the long-term follow-up of dermatofibrosarcoma protuberans cases.

Dermatofibrosarcoma↗
PubMed1995

Flap coverage of a large defect after excision of a massive dermatofibrosarcoma protuberans.

We report the case of a 62-year-old man with a huge (25 X 15 cm) dermatofibrosarcoma protuberans of the left flank and abdomen. This is one of the largest dermatofibrosarcoma protuberans lesions reported in the literature. Wide surgical excision of the tumor with 4-cm tumor-free margins created an extensive wound. Wound coverage was performed by transposing an extended rectus abdominis myocutaneous pedicled flap to the defect. Follow-up at 2 years revealed no evidence of local or regional recurrence. We present a brief review of dermatofibrosarcoma protuberans and a discussion of the reconstruction of such large defects.

Back↗
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↗
PubMed1993

Dermatofibrosarcoma protuberans appearing during pregnancy.

Dermatofibrosarcoma protuberans is an uncommon skin tumor with a high potential for local recurrence if not adequately excised. There are only two reports in the literature describing dermatofibrosarcoma protuberans that enlarged considerably during pregnancy. On the other hand, the new development or enlargement of dermatofibromas in pregnancy is well documented. We present 2 additional patients in whom a dermatofibrosarcoma protuberans appeared and grew rapidly during pregnancy. Immunohistochemical studies were negative for estrogen and progesterone receptors. The possible pathophysiology and surgical management are presented and discussed.

Adult↗