Dermatofibrosarcoma protuberans

Gloster, Hugh Morris

doi:10.1016/s0190-9622(96)90597-6

Cited by 349 publications

(340 citation statements)

References 123 publications

Supporting

Mentioning

313

Contrasting

Unclassified

Order By: Relevance

“…15,16 Lesions are located most commonly on the trunk or proximal extremities but may develop anywhere on the skin. 17 Clinically, these neoplasms usually present as a raised, indurated, asymptomatic plaque that may be any combination of blue, red, and brown or flesh colored. 18 DFSP tumors usually are not fixed to deeper structures, unless they are long-standing or recurrent.…”

Section: Discussionmentioning

confidence: 99%

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

DuBay

Cimmino

Lowe

et al. 2004

Cancer

202

141

View full text Add to dashboard Cite

BACKGROUNDDermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years.METHODSThe records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution.RESULTSSixty‐three DFSP lesions were removed from 62 patients. At a median follow‐up of 4.4 years, no local or distant recurrences were detected in any patient. Forty‐three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety‐five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty‐five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%).CONCLUSIONSWide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution. Cancer 2004;100:1008–16. © 2004 American Cancer Society.

show abstract

Section: Discussionmentioning

confidence: 99%

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

DuBay

Cimmino

Lowe

et al. 2004

Cancer

202

141

View full text Add to dashboard Cite

show abstract

“…16 It is more common in the 20 to 50 years-of-age bracket, although there are cases reported from birth up to 80 years of age. Distribution is equal for both genders, with some studies showing a slight male predominance.…”

Section: Epidemiology and Pathogenymentioning

confidence: 99%

Sarcomas cutâneos primários

Fleury

Sanches

2006

An. Bras. Dermatol.

View full text Add to dashboard Cite

Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review. Keywords: Angiosarcoma; Dermatofibrosarcoma; Leiomyosarcoma; Liposarcoma; Sarcoma/diagnosis; Skin neoplasm Resumo: Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sar-

show abstract

“…1 Fibrosarcomatous transformation rarely occurs in dermatofibrosarcoma protuberans and is associated with an increased risk for metastases. 2,3 Current treatment for dermatofibrosarcoma protuberans or fibrosarcomatous dermatofibrosarcoma protuberans consists of wide local excision with negative margins and possible adjuvant radiotherapy in selected cases.…”

mentioning

confidence: 99%

Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans

et al. 2006

View full text Add to dashboard Cite

Dermatofibrosarcoma protuberans is a superficial low-grade sarcoma that rarely evolves into a high-grade fibrosarcoma. Dermatofibrosarcoma protuberans is genetically characterized by the unbalanced chromosomal t(17;22)(q21;q13), usually in the form of a supernumerary ring chromosome. The product of this chromosomal translocation is the chimeric gene COL1A1-PDGFB (collagen type I alpha I-platelet-derived growth factor beta), which is amplified at low levels in the ring chromosome. The aims of this study were to evaluate (1) whether genomic gains of this fusion gene occur during the clonal evolution of dermatofibrosarcoma protuberans into fibrosarcomatous dermatofibrosarcoma protuberans and (2) whether there is a difference between the number of genomic copies of COL1A1-PDGFB between classic dermatofibrosarcoma protuberans and dermatofibrosarcoma protuberans areas associated with fibrosarcomatous dermatofibrosarcoma protuberans. Eleven cases of fibrosarcomatous dermatofibrosarcoma protuberans with both dermatofibrosarcoma protuberans and fibrosarcomatous areas and 10 cases of classic dermatofibrosarcoma protuberans were studied. Genomic copies of COL1A1-PDGFB were evaluated by fluorescence in situ hybridization using a custom designed probe for the PDGFB locus on 4 lm thick paraffin-embedded tissue sections. Genomic gains of the COL1A1-PDGFB gene were observed in six (of 10) fibrosarcomatous dermatofibrosarcoma protuberans in the fibrosarcomatous areas when compared to the dermatofibrosarcoma protuberans areas of the same tumor (2-7 gene copies (median PDGFB copy gain, 2.8) versus 1-3 gene copies (median PDGFB copy gain, 1.7), respectively, P ¼ 0.004). Four fibrosarcomatous dermatofibrosarcoma protuberans did not show genomic gains of COL1A1-PDGFB fusion gene between the two areas. Essentially no difference in the copy number of COL1A1-PDGFB fusion gene was observed between dermatofibrosarcoma protuberans areas of classic dermatofibrosarcoma protuberans and dermatofibrosarcoma protuberans areas of fibrosarcomatous dermatofibrosarcoma protuberans (median PDGFB copy gain of 1.8 versus 1.7, respectively, P ¼ 0.36). Genomic gains of COL1A1-PDGFB fusion gene is possibly an oncogenic mechanism that is identified in the clonal evolution of a subset of dermatofibrosarcoma protuberans that evolves into fibrosarcomatous dermatofibrosarcoma protuberans. Since this finding was not observed in all cases of fibrosarcomatous dermatofibrosarcoma protuberans, other oncogenic mechanisms may be operating in this form of tumor progression. Copy number of COL1A1-PDGFB fusion gene in the classic dermatofibrosarcoma protuberans areas does not seem to be a major predisposing mechanism for fibrosarcomatous transformation.

show abstract

Dermatofibrosarcoma protuberans

Cited by 349 publications

References 123 publications

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

Sarcomas cutâneos primários

Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans

Contact Info

Product

Resources

About