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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↗

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↗

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↗

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↗

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↗

PubMed1997

Effect of resection margins on dermatofibrosarcoma protuberans of the head and neck.

OBJECTIVE: To determine clinical and surgical factors that affect the recurrence of dermatofibrosarcoma protuberans of the head and neck. DESIGN: Retrospective case series of 32 patients who were seen between 1960 and 1995 with a diagnosis of dermatofibrosarcoma protuberans of the head and neck. Data extracted from patients records included sex, age at diagnosis, tumor site, presence or absence of tumor-free margins, initial and salvage treatment, time to recurrence, and status after last treatment. SETTING: Academic tertiary care referral center. INTERVENTION: Surgical excision and radiation therapy. RESULTS: Univariate Kaplan-Meier analysis for recurrence showed the difference in survival to be statistically significant (P < .05) between patients who had wide (> or = 2 cm) surgical margins vs those who had close (< 2 cm) margins. Univariate Kaplan-Meier analysis for recurrence with or without radiation as the grouping variable showed the difference to be statistically significant (P < .01). Similar analyses with sex, age, tumor site, and size of tumor as the grouping variables were not statistically significant. Multivariate Cox regression analysis was used with sex, age, and close or wide margins as variables. Close margins (< 2 cm) had a statistically significant positive correlation with recurrence (P < .05), while sex and age were not associated with recurrence. CONCLUSION: Surgical margins of at least 2 cm should be used in all cases of dermatofibrosarcoma protuberans of the head and neck to prevent recurrence.

Adolescent↗

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↗

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↗

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↗

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↗