Congenital Dermatofibrosarcoma Protuberans

Weinstein, Jill M.; Drolet, Beth A.; Esterly, Nancy B.; Rogers, Maureen; Bauer, Bruce S.; Wagner, Annette; Mancini, Anthony J.

doi:10.1001/archderm.139.2.207

Cited by 94 publications

(83 citation statements)

References 18 publications

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“…1 This neoplasm is most common in adults in their third to fifth decades 14 but has been reported in all age groups, including children, infants, and newborns. 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.…”

Section: Discussionmentioning

confidence: 99%

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

DuBay

Cimmino

Lowe

et al. 2004

Cancer

202

144

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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.

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Section: Discussionmentioning

confidence: 99%

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

DuBay

Cimmino

Lowe

et al. 2004

Cancer

202

144

View full text Add to dashboard Cite

show abstract

“…Dermatofibrosarcoma protuberans is a rare soft-tissue sarcoma of intermediate malignancy, more commonly seen in middle-age adults but also reported in children, even at birth (37,38). It is most commonly found on the trunk or proximal extremities but can occur anywhere (37)(38)(39).…”

Section: Dermatofibrosarcoma Protuberansmentioning

confidence: 99%

“…It is most commonly found on the trunk or proximal extremities but can occur anywhere (37)(38)(39). The tumor originates in the dermis but spreads locally into the deeper tissues, such as the subcutaneous tissues, fascia, muscles, and bones (40,41).…”

Section: Dermatofibrosarcoma Protuberansmentioning

confidence: 99%

“…The tumor originates in the dermis but spreads locally into the deeper tissues, such as the subcutaneous tissues, fascia, muscles, and bones (40,41). It is seen as a slow-growing, indolent subcutaneous plaque or nodule, the overlying skin of which may be discolored reddish blue or purple (37,39). As a result, it can be mistaken clinically for other tumors, such as a vascular malformation or a hemangioma, and thus it is often diagnosed late.…”

Section: Dermatofibrosarcoma Protuberansmentioning

confidence: 99%

“…MR imaging may be performed to monitor tumor recurrence. Metastases are rare, usually found in the lung, and are associated with a much poorer prognosis (37,39).…”

Section: Dermatofibrosarcoma Protuberansmentioning

confidence: 99%

See 2 more Smart Citations

Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation

Laffan¹,

Ngan²,

Navarro³

2009

RadioGraphics

117

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A Clinical, Histologic, and Molecular Study of 9 Cases of Congenital Dermatofibrosarcoma Protuberans

Maire

Fraïtag²,

Galmiche³

et al. 2007

Arch Dermatol

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Background:The diagnosis of dermatofibrosarcoma protuberans (DFSP) in childhood is often difficult because of the deceptive appearance of the lesions. Little is known about congenital DFSP, the frequency of which is probably underestimated because the initial lesion may pass unnoticed.Observations: We studied 9 DFSP congenital cases (8 plaques and 1 nodule) initially suspected to be benign lesions. The first biopsies or excisions were performed after a delay of 5½ months to 15 years. All cases were CD34ϩ. Histologic patterns were similar to the DFSP adult classic pattern in 4 cases. One case was a Bednar tumor. The histologic diagnosis of the 4 remaining cases was difficult. The collagen, type I, ␣ 1-platelet-derived growth factor ␤ fusion gene (COL1A1-PDGFB) was detected by means of reverse transcriptase-polymerase chain reaction or fluorescence in situ hybridization.Conclusions: All cases of congenital DFSP were difficult to identify clinically. The diagnosis was suspected by means of histologic and immunohistochemical evaluation and was confirmed using molecular analyses. This study illustrates the difficulties and pitfalls of the recognition of congenital DFSP and emphasizes the value of immunohistochemical study with anti-CD34 and complementary molecular analysis for all cutaneous spindle cell tumors and plaques in neonates and infants.

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Congenital Dermatofibrosarcoma Protuberans

Cited by 94 publications

References 18 publications

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation

A Clinical, Histologic, and Molecular Study of 9 Cases of Congenital Dermatofibrosarcoma Protuberans

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