Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report

Suehara, Yoshiyuki; Yazawa, Yasuo; Hitachi, Kouhei

doi:10.1007/s00776-004-0831-2

Cited by 16 publications

(12 citation statements)

References 4 publications

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“…In addition, tumor-related mortality was observed in 5.8% of FS-DFSP cases (3). In contrast, the local recurrence rate of pigmented DFSP (10-13%) is lower compared with conventional DFSP, and distant metastasis is extremely rare (6).…”

Section: Discussionmentioning

confidence: 92%

“…Fibrosarcomatous DFSP (FS-DFSP) is a further variant, characterized by areas histopathologically indistinguishable from fibrosarcoma, and with a clinically increased incidence of metastasis (3). Fibrosarcomatous pigmented DFSP is extremely rare; only five cases have been reported in the English language literature (3)(4)(5)(6)(7). In the present study, we report a further case of this extremely rare lesion and discuss the clinicopathological features.…”

Section: Introductionmentioning

confidence: 99%

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Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature

Ishida

Okabe

2012

Oncology Letters

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Abstract. Dermatofibrosarcoma protuberans (DFSP) is a relatively rare low-grade sarcoma that occasionally exhibits melanin-containing spindle cells within the tumor. Fibrosarcomatous DFSP (FS-DFSP) is a variant that is characterized by areas which are histopathologically indistinguishable from fibrosarcoma. In the present study, we describe a case of fibrosarcomatous pigmented DFSP and review the clinicopathological features of this extremely rare lesion. A 51-year-old male presented with a slow-growing nodular cutaneous mass in his left upper arm. Histopathologically, the resected tumor was comprised of pigmented DFSP in approximately 20% of the tumor, with the remaining area further covered by a fibrosarcomatous component. A review of the clinicopathological features of the five previously reported cases as well as the present case indicated that this lesion mainly affects middle-aged males and occurs mostly in the extremities. Melanin-containing spindle cells are present only in the conventional DFSP component. The prognosis appears to be poor; in the six cases reviewed, four demonstrated multiple metastases and three succumbed to the disease. Our analyses revealed that the presence of a fibrosarcomatous component in pigmented DFSP is associated with aggressive behavior; therefore, careful assessment for the presence of a fibrosarcomatous component is necessary in the diagnosis of this disease.

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Section: Discussionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature

Ishida

Okabe

2012

Oncology Letters

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“…It is seen in young to middle-aged adults in the fourth decade with very occasional cases in the pediatric age group [6,7] . The most common site of the bednar tumor is trunk and proximal extremities [8,9,10] . These tumors are charecterised by the relentless growth over the years.…”

Section: Discussionmentioning

confidence: 99%

Bednar tumor or Pigmented Variant of Dermatofibrosarcoma protuberans: A Rare Case Report

Banerjee¹,

Mridha²,

Shrivastava³

et al. 2017

IOSR JDMS

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“…According to the pertinent literature, the major sites include the lungs, followed by the brain (5)(6)(7)(8)(9)(10)(11)(12)(13)(14). Pancreatic metastasis of DFSP is extremely rare, with only two other reported cases identified in the PubMed database using the search terms "DFSP" and "pancreas" or "distant metastasis" (9, 10).…”

Section: Discussionmentioning

confidence: 99%

“…In general, DFSP has a low malignant potential, however, it has a pronounced tendency for local relapse (1-3, 5, 6). In addition, distant metastases are extremely rare, but there have been occasional reports of distant metastases and a poor prognosis (5)(6)(7)(8)(9)(10)(11).…”

Section: Introductionmentioning

confidence: 99%

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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Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report

Cited by 16 publications

References 4 publications

Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature

Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature

Bednar tumor or Pigmented Variant of Dermatofibrosarcoma protuberans: A Rare Case Report

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

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