Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Saïag, Philippe; Grob, Jean-Jacques; Lebbe, Célèste; Malvehy, Josep; Marmol, Véronique Del; Pehamberger, Hubert; Peris, Ketty; Stratigos, Alexander J.; Middelton, Mark; Basholt, Lars; Testori, Alessandro; Garbe, Claus

doi:10.1016/j.ejca.2015.06.108

Cited by 131 publications

(170 citation statements)

References 9 publications

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“…The clinical outcomes of imatinib treatment for metastatic or advanced DFSP in Japanese cases that have been reported with English abstracts are summarized in Table. Although PDGFB fusion was not always consistent with a good response to imatinib, the obtained clinical outcomes were promising and similar to the results reported from other countries (12)(13)(14). Several clinical studies and case reports (3,(12)(13)(14)(15)(16)(17)(18) suggested that that a moderate dosage of 400-600 mg/day appeared to be as equally as effective as a higher dosage (800 mg/day) and was better tolerated. Since our case responded to 400 mg of imatinib without any toxicity, the therapy was continued with the same dosage.…”

Section: Discussionsupporting

confidence: 80%

“…The disease occurs more frequently on the trunk than proximal extremities and rarely in the head and neck region (1,2). Cytogenetically, DFSP is characterized by a pathognomonic translocation t(17;22) (q22;q13) with fusion of the collagen type A chain (COL1A1) gene on chromosome 17 with the platelet-derived growth factor beta chain (PDGFB) gene on chromosome 22 (3,4). This results in the constitutive expression of the PDGFB ligand, creating an autocrine stimulatory loop that drives cell proliferation and fibrosis.…”

Section: Introductionmentioning

confidence: 99%

“…Standard treatment for DFSP includes complete resection of a 2-3 cm wide margin of visibly uninvolved tissue, including underlying fascia (1)(2)(3). In general, DFSP has a low malignant potential, however, it has a pronounced tendency for local relapse (1-3, 5, 6).…”

Section: Introductionmentioning

confidence: 99%

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Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases

et al. 2016

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We herein encountered a case of abdominal wall dermatofibrosarcoma protuberans (DFSP) that developed pulmonary and pancreatic metastases 5 years after complete resection. Because specific rearrangements of the platelet-derived growth factor beta (PDGFB) locus by a novel fluorescence in situ hybridization method was detected, the patient was treated with imatinib mesylate at 400 mg/day. A partial response was achieved by imatinib without any specific toxicity. Although metastatic DFSP is an extremely rare disease, an evaluation of PDGFB fusion is essential and imatinib mesylate should be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP.

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Section: Discussionsupporting

confidence: 80%

Section: Introductionmentioning

confidence: 99%

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Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases

et al. 2016

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“…Clinically, this tumor often presents as a protuberant and hard solid lump. The diagnosis depends on the histopathological and immunohistochemical examination (3). Surgical resection is the first choice for treatment and the prognosis is relatively good, with a five-year survival rate of >90% (4).…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans with pit-like lesions: A case report and literature review

et al. 2015

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Abstract. The present study describes a case of pit-like dermatofibrosarcoma protuberans (DFSP) with the clinical manifestations, histopathological features and criteria for diagnosis. The study also reviews the relevant literature in order to raise awareness among dermatologists with regard to the specific behavior of DFSP. A 27-year-old female presented with subcutaneous nodules on the left side of the neck that had been apparent for 5 years and which had gradually begun caving in during the last year. Histopathological examination revealed that the tumor was composed of a large number of spindle cells arranged in a whirlpool-like pattern. Immunohistochemical studies revealed positive staining for cluster of differentiation 34, vimentin and lysozyme, which could be used as diagnostic markers of DFSP. The patient was finally diagnosed with DFSP by pathological and immunohistochemical analyses. The DFSP was treated with an extended resection followed by adjacent skin repair. The patient responded well and no relapse occurred during the 8-month clinical follow-up. Thus, the current study describes a unique pit-like clinical manifestation of DFSP with typical immunohistochemical and pathological features. In addition, histopathological examination revealed a downward depression in the epidermis. Therefore, histopathological examination should be considered as an essential diagnostic tool for DFSP.

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“…Mohs surgery is listed fi rst in the National Comprehensive Cancer Network guidelines (and wide local excision with 2-to 4-cm margins is last in the list) ( 2 ). In 2015, the European consensus-based interdisciplinary guideline for the treatment of DFSP recommended Mohs surgery as the treatment of choice ( 3 ). Th e British Society for Dermatological Surgery position statement on the management of DFSP states that Mohs surgery is the initial treatment of choice for DSFP ( 4 ).…”

mentioning

confidence: 99%

Reader Comments: Operative Management of Dermatofibrosarcoma Protuberans of the Breast

Saporito

Charalampoudis

Kinney

et al. 2017

Baylor University Medical Center Proceedings

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Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

Cited by 131 publications

References 9 publications

Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases

Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases

Dermatofibrosarcoma protuberans with pit-like lesions: A case report and literature review

Reader Comments: Operative Management of Dermatofibrosarcoma Protuberans of the Breast

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