Dermatofibrosarcoma Protuberans of the Head and Neck

Stojadinovic, Alexander; Karpoff, Howard M.; Antonescu, Cristina R.; Shah, Jatin P.; Singh, Bhuvanesh; Spiro, Ronald H.; Dumornay, Wilson; Shaha, Ashok R.

doi:10.1007/s10434-000-0696-3

Cited by 124 publications

(72 citation statements)

References 27 publications

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“…11 The lesions can be multiple and may coalesce to form an indurated plaque (Fig 3), which on clinical examination is often violaceous. 12,13 The growth rate is variable; some lesions remain stable in size for many years, whereas others demonstrate slow progressive growth.…”

Section: Discussionmentioning

confidence: 99%

“…29,30 Treatment options include complete surgical excision, including wide excision and Mohs micrographic surgery, radiation, and imatinib mesylate. 2,11,31 Imatinib, a competitive tyrosine kinase inhibitor, is currently approved by the Food and Drug Administration for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP, and this may in turn facilitate tumor resection and or decrease disfigurement. 32 After local resection, the 5-year survival rate is 93-100%.…”

mentioning

confidence: 99%

“…Local recurrence occurs in 20 -55% of cases. [12][13][14]35 Most local recurrences occur within 3 years 11,12,35 ; only 30% occur after 5 years. 32 The lungs are the most common site of distant metastasis, 12,30,36,37 and metastasis has been reported to occur in 5-6% of cases.…”

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confidence: 99%

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Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

Millare

Guha-Thakurta²,

El‐Naggar

et al. 2013

American Journal of Neuroradiology

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

Millare

Guha-Thakurta²,

El‐Naggar

et al. 2013

American Journal of Neuroradiology

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“…Late recurrences, more than 10 years post-excision, although rare, have been described. 15,18,29,31 Consequently, patients must be examined every three to six months during the first three years after surgery and annually for the rest of their lives.…”

Section: Prognosismentioning

confidence: 99%

Sarcomas cutâneos primários

Fleury

Sanches

2006

An. Bras. Dermatol.

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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-

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“…1 Wide excision with histologically negative margins is the cornerstone of treatment, but relatively high recurrence rates are described in the literature, [1][2][3][4] especially for DFSP lesions located on the head and neck. 5,6 Despite the achievement of "negative" margins, it is believed that the high recurrence rate is due to the fact that large portions of the true margins are not evaluated by standard histologic processing, combined with the difficulty identifying the occult, fingerlike projections that are characteristic of DFSP and are responsible for tumor recurrence. An extensive review of the literature published in 1996 found a mean recurrence rate of 18% (range, 0 -60%) after treatment with wide local excision in 15 studies, with a total recurrence rate of 20% among all reviewed patients (100 recurrences of 489 reviewed patients).…”

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Low recurrence rate after surgery for dermatofibrosarcoma protuberans

DuBay

Cimmino

Lowe

et al. 2004

Cancer

202

141

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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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Dermatofibrosarcoma Protuberans of the Head and Neck

Abstract: Wide margin resection of head and neck DFSP predicts negative histological margins and impacts favorably on local recurrence-free survival. Frozen section analysis does not assess resection margins accurately.

Cited by 124 publications

References 27 publications

Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

Sarcomas cutâneos primários

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

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