Dermatofibrosarcoma protuberans: treatment results of 35 cases

Sun, Li; Wang, Chong Jong; Huang, Chao‐Cheng; Leung, Stephen Wan; Chen, Hui Chun; Fang, Fu Min; Huang, Eagle Yi‐Kung; Lee, Steve P.

doi:10.1016/s0167-8140(00)00228-0

Cited by 110 publications

(84 citation statements)

References 18 publications

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“…Sun et al 12 reported on 35 patients with DFSP who were treated with surgery alone (24 patients) or with surgery and RT (11 patients) at Chang Gung Memorial Hospital (Taipei, Taiwan) between 1987 and 1998. Follow-up ranged from 11 to 131 months in duration (median, 50 months).…”

Section: Outcomementioning

confidence: 99%

“…4 Uncommon variants of DFSP include the Bednar tumor, which is characterized by the presence of melanin-containing dendritic cells, and myxoid DFSP. 11,12 In addition, approximately 10 -15% of all DFSPs contain a fibrosarcomatous component. 3,13,14 Goldblum et al 15 reported that out of a series of 18 DFSP-FSs, 15 were previously untreated lesions, whereas the remaining 3 were recurrent tumors.…”

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confidence: 99%

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Dermatofibrosarcoma protuberans

Mendenhall

Zlotecki

Scarborough

2004

Cancer

198

174

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In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10 -15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is Յ 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection alone; however, postoperative radiotherapy results in local control rates of Ն 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.

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Section: Outcomementioning

confidence: 99%

mentioning

confidence: 99%

Dermatofibrosarcoma protuberans

Mendenhall

Zlotecki

Scarborough

2004

Cancer

198

174

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“…Adjuvant RT can reliably reduce the local recurrence rate and avoid the mutilation and functional deficit caused by repeated surgery. (3) In this case, the tumour had spread to surrounding skin and to deeper soft tissues. Due to the wide distribution of the tumor as well as cosmetic considerations in this case, the local tumor was excised with 2 cm of a grossly clear tumor margin.…”

Section: Fig 1: Gross View Of Umbilical Ulcerative Tumourmentioning

confidence: 84%

Dermato Fibrosarcoma Protuberans in Umbilical Region: A Case Report

S¹,

Babj²

2015

jemds

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Dermatofibrosarcoma protuberans is a rare fibroblastic mesenchymal skin tumor with low-grade malignancy and occasional distant metastasis. The tumour accounts for less than 0.001% of all malignancies. Most cases involve the trunk, extremities, scalp, and neck. In rare cases, the genital urinary system may be affected. Diagnostic tools include computed tomography and magnetic resonance imaging. The standard treatment of wide-margin surgical excision with a clear margin of 3-4 cm may be problematic in large tumors or in some areas of the body, as a radical excision may produce unacceptable cosmetic, structural, or functional results. An un resected or positive margin should be treated with adjuvant radiotherapy to help prevent recurrence. Chemotherapy plays a limited role in treating this disease. This article describes a 55years male with mass in umbilical region who underwent wide local excision and rhombhoid flap transfer to cover the defect. Later patient sent for adjuvant radiotherapy, patient was tumour free at 6 months follow up.

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“…Radiation therapy has become more popular as a form of adjuvant therapy for DFSP especially when the tumor is located in the head and neck region where wide local excision with 3 cm margins may be impossible without significant morbidity. Local control rates for secondary recurrence after surgery vs. surgery with radiation therapy was 28% vs. 80%, respectively [16]. In a retrospective analysis of 53 patients treated with surgery and either preoperative or postoperative radiation therapy, Castle et al found local control of 98% and 93% at 5 and 10 years respectively when using radiation doses of roughly 50 Gy preoperatively or 60 Gy postoperatively [17].…”

Section: Discussionmentioning

confidence: 99%

Multifocal Dermatofibrosarcoma Protuberans

Myssiorek¹,

Berman²,

Sanfilippo³

2016

IJCEO

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IntroductionDermatofibrosarcoma protuberans (DFSP) is a superficial sarcoma stemming from fibroblastic and myofibroblastic differentiation within the dermis and underlying soft tissue. It is a very rare tumor with less than 4.5 cases per million persons per year in the US [1]. Between 16 and 20% of the cases of DFSP may be associated with previous traumatic events ranging from mild ulcerations to bayonet stabbing [2]. Most cases of DFSP (47%) occur in the trunk region, however roughly 10% of cases can be found in the head and neck region and 8% in the shoulder region [3]. Metastasis is a rare occurrence, with a rate of roughly 2% over ten years, and usually occurs in the lung, brain, or bone [4]. DFSP has the propensity to recur locally especially within the head and neck region where wide local excision is difficult to obtain without significant morbidity. Given the high risk of local recurrence adjuvant therapy in the form of radiation has grown in practice.Cases of multicentric DFSP are exceptionally rare, with only a few case reports available in the literature. The following case report describes a patient who had concurrent lesions of DFSP of a multifocal nature, one possibly precipitated by trauma and the other with no apparent causative injury. Case HistoryA 47 year old patient presented with simultaneous lesions of the left mastoid region and right shoulder. These lesions were subcutaneous and not tender. Roughly 20 years prior to presentation, he was struck in the back of the head with a baseball bat and subsequently developed a firm swelling over his left mastoid bone posteriorly. The lesion was removed 18 years after it initially appeared. The pathology of this specific lesion was reportedly benign. Surgical excision and pathological investigation were conducted at an outside hospital and no records were able to be obtained. In 2007, the patient was again struck in the back Abstract Background: Dermatofibrosarcoma Protuberans (DFSP) is an exceptionally rare soft tissue tumor that spreads locally with rare metastasis limited to brain, lung, and liver. Immunocompetent patients presenting with more than one DFSP are rare. Methods and Results: A 47 year old man presented with metachronous lesions of the left mastoid skin and right supraclavicular fossa skin. Biopsies revealed DFSP in both locations. CT scans never showed bony invasion in either location. Multiple excisions and radiation therapy have resulted in no recurrence at the supraclavicular site and recurrence in the mastoid site. Conclusions: DFSP can recur despite wide and deep excision combined with radiation therapy. The treatment algorithm for DFSP in the Head and Neck region is included in our review of the literature. Long term follow up is mandatory in these patients.

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Dermatofibrosarcoma protuberans: treatment results of 35 cases

Cited by 110 publications

References 18 publications

Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans

Dermato Fibrosarcoma Protuberans in Umbilical Region: A Case Report

Multifocal Dermatofibrosarcoma Protuberans

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