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Results for “Dermatofibrosarcoma”

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PubMed2002

Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans.

PURPOSE: Dermatofibrosarcoma protuberans is caused by activation of the platelet-derived growth factor B (PDGFB) receptor, a transmembrane tyrosine kinase. We investigated the response of dermatofibrosarcoma protuberans to the tyrosine kinase inhibitor imatinib mesylate. PATIENTS AND METHODS: A patient with unresectable, metastatic dermatofibrosarcoma protuberans received imatinib mesylate (400 mg bid). Response to therapy was assessed by [18F]fluorodeoxyglucose (FDG) positron emission tomography, magnetic resonance imaging, and histopathologic and immunohistochemical evaluation. RESULTS: The patient was treated for 4 months with imatinib mesylate. The hypermetabolic uptake of FDG fell to background levels within 2 weeks of treatment, and the tumor volume shrank by over 75% during the 4 months of therapy, allowing for resection of the mass. There was no residual viable tumor in the resected specimen, indicating a complete histologic response to treatment with imatinib mesylate. CONCLUSION: Imatinib mesylate is highly active in dermatofibrosarcoma protuberans. The dramatic response seen in this patient demonstrates that inhibition of PDGFB receptor tyrosine kinase activity can significantly impact viability of at least one type of solid tumor.

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↗

PubMed1999

Dermatofibrosarcoma protuberans. A rare case involving the pediatric foot.

Dermatofibrosarcoma protuberans is a rare, slow-growing, locally destructive, intermediate-grade malignancy that originates in the dermal layer of the skin. Dermatofibrosarcoma protuberans rarely metastasizes and has a marked tendency to recur following local surgical excision. The lesion is found most commonly on the trunk, on the proximal aspect of both the lower and upper extremities, and in the head and neck region. Dermatofibrosarcoma protuberans occurs most commonly in people between the ages of 20 and 50. This article describes the clinical and pathologic features of this rare, malignant neoplasm and describes a rare case of dermatofibrosarcoma protuberans involving the pediatric foot.

Child↗

PubMed1997

[Dermatofibrosarcoma Darier and Ferrand. Immunohistochemistry study of 38 cases. Research on a specific marker].

This is a retrospective pathological and immunohistochemical study of 38 cases of dermatofibrosarcoma protuberans. It is a rare dermal and subcutaneous spindle cell tumor which may be difficult to diagnose. We tested the value of the CD34 antibody and compared this series with 15 fibrous histiocytomas and II neurofibromas. Thirty-one dermatofibrosarcoma protuberans were positive for CD34 and 7 were negative. All the fibrous histiocytomas were negative. Nine neurofibromas were positive with variable expression of CD34 antigen. The CD34 antibody seems to be a sensitive marker to differentiate dermatofibrosarcoma protuberans from fibrous histiocytoma. It is specific for the dermatofibrosarcoma protuberans in the context of differential diagnosis between these two tumors. The role of immunohistochemical staining remains useful within the anatomoclinical context.

Adult↗

PubMed1998

Dermatofibrosarcoma protuberans and its fibrosarcomatous variant with areas of myoid differentiation: a report of three cases.

AIMS: We describe three examples of dermatofibrosarcoma protuberans which demonstrated focal myofibroblastic differentiation, and discuss the nature of myofibroblastic differentiation. METHODS AND RESULTS: We studied three cases of dermatofibrosarcoma protuberans, two of which had fibrosarcomatous portions containing myoid areas, and one which showed myoid areas in ordinary dermatofibrosarcoma protuberans. Myoid areas were recognized histologically as randomly distributed, scattered bundles or small nodules of spindle cells with deeply eosinophilic cytoplasm. Myofibroblastic differentiation was demonstrated immunohistochemically by positive staining for smooth muscle actin and muscle-specific actin, and ultrastructurally by the presence of microfilament bundles with focal dense bodies and pinocytic vesicles. The myoid areas occasionally contained intraneoplastic blood vessels. CONCLUSION: The presence of myoid areas may be related to reactive hyperplasia of stroma, and its relation to the histogenesis of dermatofibrosarcoma protuberans is uncertain.

Actins↗

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↗

PubMed2000

Dermatofibrosarcoma protuberans: treatment results of 35 cases.

BACKGROUND AND PURPOSE: This study evaluates the treatment results of patients with dermatofibrosarcoma protuberans. PATIENTS AND METHODS: Between August 1987 and July 1998, 35 consecutive patients with pathologically proved dermatofibrosarcoma protuberans received surgery with or without radiation therapy. Their treatment results were analyzed retrospectively. RESULTS: The patient ages ranged from 5 to 67 years (median 37 years). There were 24 males and 11 females. The anatomic sites of tumor were: trunk in 21, extremity in eight, and head and neck region in six. The maximal dimension of tumor ranged from 1.5 to 25 cm. Surgery techniques included local excision and wide excision with or without graft or flap. Adjuvant radiation therapy was given to 11 patients, with a dose ranging from 46 to 68 Gy (one pre-operative, ten post-operative). At a median follow-up of 50 months (range 11-131 months), there were 11 patients (nine patients without radiation therapy) who developed local failure. Salvage therapy (excision with or without radiation therapy) was given to all of them, and ten achieved disease control. Some patients had treatment-related moderate cosmetic or functional problems. CONCLUSIONS: Dermatofibrosarcoma protuberans is a malignancy of a high cure rate, and adjuvant radiation therapy can reliably decrease the local recurrence rate and prevent mutilation and functional deficit caused by repeated surgery.

Adolescent↗

PubMed1998

The role of radiation therapy in the management of dermatofibrosarcoma protuberans.

PURPOSE: To evaluate the outcome for dermatofibrosarcoma protuberans treated with conservation surgery and radiation therapy. METHODS AND MATERIALS: A retrospective review was performed of 19 consecutive patients with pathologically confirmed dermatofibrosarcoma protuberans who received radiation as an adjuvant to surgical resection. RESULTS: The patients ages ranged from 19-76 years (median, 40 years); 12 were men. Lesions were located on the trunk in 8, in the head and neck area in 7, and in an extremity in 4. Tumor size ranged from 1.2 to 15 cm (median, 4 cm). Ten patients had at least 1 prior recurrence following earlier resection. Two patients received preoperative radiation to 50 Gy in 5 weeks. Sixteen patients underwent resection followed by radiation (6 of these had positive resection margins). In another patient, the tumor regrew rapidly after resection and definitive radiation was delivered for gross disease. The 6 patients with positive microscopic margins received a median dose of 60 Gy, as did the 10 with negative margins. The 1 patient with gross disease received 65 Gy. At a median follow-up of 6 years, the only patient to develop local recurrence was treated with definitive radiotherapy for gross disease. Actuarial local control was 95% at 10 years. CONCLUSION: Dermatofibrosarcoma protuberans is a radioresponsive tumor and radiation to doses of 50-60 Gy should be considered as an adjuvant to resection if margins are positive. Combined conservation resection and postoperative radiation should also be considered for situations where adequate wide excision alone would result in major cosmetic or functional deficits.

Adult↗

PubMed2003

Atrophic dermatofibrosarcoma protuberans: case report, review, and proposed molecular mechanisms.

We report a case of atrophic dermatofibrosarcoma protuberans, an uncommon clinical variant. We discuss molecular and genetic mechanisms of dermatofibrosarcoma protuberans tumorigenesis, review the literature on atrophic dermatofibrosarcoma protuberans, and pose a molecular mechanism for the atrophic variant's phenotype. We also describe using Mohs micrographic excision with CD34 immunostains on the frozen sections to definitively treat the tumor.

Aged↗

PubMed2005

Gene mutation analysis in five cases of dermatofibrosarcoma protuberans using formalin-fixed, paraffin-embedded tissues.

Fusion of the collagen type I a 1 (COL1A1) gene with the platelet-derived growth factor B-chain (PDGFB) gene has been pointed out in dermatofibrosarcoma protuberans. Various exons of the COL1A1 gene have been shown to be involved in the fusion with exon 2 of the PDGFB gene. We studied the breakpoints of the COL1A1 gene using formalin-fixed, paraffin-embedded tumour specimens from five patients with dermatofibrosarcoma protuberans (three reconfirmations and two new cases). Reverse transcriptase-PCR was performed using paraffin-embedded tissues. Nucleotide sequence analysis was carried out using the PCR products to identify the breakpoints. The COL1A1-PDGFB fusion transcripts were detected from the tumour specimens. Sequence analysis revealed that the ends of exons 18, 29, 38, 42 and 44 in the COL1A1 gene were fused with the start of exon 2 in the PDGFB. This study identified a novel COL1A1 breakpoint, namely, exon 44 of the COL1A1 gene. Detection of the aberrant fusion transcript using formalin-fixed, paraffin-embedded tumour specimens is useful as a diagnostic aid for dermatofibrosarcoma protuberans in cases where fresh or frozen samples of tumour tissue are not available.

Adolescent↗

PubMed2002

COL1A1-PDGFB gene fusion demonstrates a common histogenetic origin for dermatofibrosarcoma protuberans and its granular cell variant.

Granular cell variant of dermatofibrosarcoma protuberans is very rare with only one report of two cases. We report a new case in which we demonstrated the presence of the dermatofibrosarcoma protuberans-specific COL1A1-PDGFB fusion from paraffin-embedded tissue. This case analysis demonstrated the utility of molecular genetics as a powerful tool for the diagnosis of atypical forms of dermatofibrosarcoma protuberans.

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↗

PubMed2003

Dermatofibrosarcoma protuberans of the head and neck: treatment with Mohs surgery using inverted horizontal paraffin sections.

OBJECTIVE: To describe results of treating dermatofibrosarcoma protuberans of the head and neck with a Mohs surgical technique in which tumor margins were evaluated with inverted horizontal paraffin sections to identify the margin of resection necessary to achieve tumor-free borders. STUDY DESIGN: Retrospective case study of nine patients. METHODS: In each case, Mohs surgery using inverted horizontal frozen-section analysis was continued until tumor-free margins were achieved. The tumor-free specimens collected during this procedure were immediately reprocessed by using inverted horizontal paraffin sectioning, and the histological results of the two techniques were compared. To identify the margins of resection needed to achieve tumor-free margins, preoperative lesion size was compared with postoperative defect size. RESULTS: Paraffin section analysis showed persistent tumor in seven of the nine patients in whom the frozen-section technique was used to clear the tumor. Tumor-free margins were achieved in all patients by using one additional excision. The tumor had spread extensively beyond clinical margins and required a mean minimum margin of 3.7 cm around the gross area of tumor (margin range, 2.5-6.0 cm). Patients treated by this technique showed no local, regional, or distant recurrence at follow-up performed at a mean time of 43 months postoperatively (range, 19-74 mo). CONCLUSIONS: Dermatofibrosarcoma protuberans of the head and neck is a tumor that shows extensive infiltration beyond gross margins. Wide local excision with 2- to 3-cm margins results in an unacceptably high recurrence rate; larger excisional margins are necessary to remove all disease. Accurate margin identification achieved by using Mohs surgery with rush paraffin section analysis results in complete tumor removal and excellent control rates. This method should be preferred for treatment of dermatofibrosarcoma protuberans of the head and neck.

Adult↗

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.

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