Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: our experience, molecular evaluation of selected cases, and short literature review

Lopez, Liurka V.; Yatsenko, Svetlana A.; Burgess, Melissa; Schoedel, Karen E.; Rao, Uma

doi:10.1111/ijd.14462

Cited by 9 publications

(7 citation statements)

References 21 publications

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“…The histopathological differential diagnosis of DFSP includes various spindle cells neoplasms such as dermatofibroma, solitary fibrous tumor, spindle cell lipoma, schwannoma, synovial sarcoma, spindle cell melanoma or sarcomatoid carcinoma. Extensive immunohistochemistry analysis usually suffices for establishing the correct diagnosis, but in rare cases, molecular analysis can be required [1,6,17]. Molecular studies have demonstrated that DFSP displays a characteristic t(17;22)(q22;q13) leading to the formation and overexpression of the fusion gene COL1A1-PDGFB, and subsequently to activation of PDGF receptor [1,16,17].…”

Section: Discussionmentioning

confidence: 99%

“…Extensive immunohistochemistry analysis usually suffices for establishing the correct diagnosis, but in rare cases, molecular analysis can be required [1,6,17]. Molecular studies have demonstrated that DFSP displays a characteristic t(17;22)(q22;q13) leading to the formation and overexpression of the fusion gene COL1A1-PDGFB, and subsequently to activation of PDGF receptor [1,16,17].…”

Section: Discussionmentioning

confidence: 99%

“…Due to the activation of PDGF receptor, oncological therapy with Imatinib mesylate is considered an effective treatment at present. In addition to this, DFSP is sensitive to radiotherapy and patients with large, recurrent, or inoperable tumors can benefit from radiation therapy [17,18].…”

Section: Discussionmentioning

confidence: 99%

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When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

Dumitru

Ciongariu

Aliuș

et al. 2021

Romanian Journal of Orthopaedic Surgery and Traumatology

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Bednar tumor is a rare cutaneous neoplasm, regarded as a variant of dermatofibrosarcoma protuberans with melanotic colonization and it usually affects young and middle-aged adults or children. This lesion is considered a low-grade malignant tumor, which can be associated with multiple local recurrences after surgical excision. Although a rare phenomenon, these lesions may undergo fibrosarcomatous transformation, which implies a poorer prognosis of the disease, as the tumor has a more locally aggressive behavior and patients might also develop distant metastases. We present the case of a 53-year-old female patient, with no significant medical history, who presented with a subcutaneous nodule on her upper back, for which a wide surgical excision was performed. The gross examination of the specimen showed a solitary protuberant grey-white nodule with a bluish shade and flecked with pigment. The microscopic examination revealed a malignant proliferation with a predominantly fascicular growth pattern, composed of spindle cells with highly pleomorphic nuclei and high mitotic rate, as well as the presence of dendritic cells with abundant melanin. Upon immunohistochemical analysis, the proliferation showed negative staining for CD 34 and AE 1/3, whereas the scattered dendritic cells stained positive with S100 protein. Ki 67 was positive in 15% of the tumor cells and the absence of p53 expression was noted. Thus, the diagnosis of Bednar tumor with fibrosarcomatous transformation was established. The aim of this paper was to gain further knowledge about the histopathological and immunohistochemical features, as well as about the treatment of Bednar tumor, especially considering its rarity.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

Dumitru

Ciongariu

Aliuș

et al. 2021

Romanian Journal of Orthopaedic Surgery and Traumatology

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“…2 The present pediatric case (defined as FS-DFSP in a patient under the age of 21) is the twelfth to have been reported in the literature (Table 1). [8][9][10][11][12][13][14][15][16][17] Examining the reported pediatric patients, the median age was 12.5 years [range: [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], and a slight male predominance (male: female ratio 1. MIB-1 proliferation index, and low CD34 expression ( Table 2).…”

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children

Chicaud

Frassati-Biaggi

Kaltenbach

et al. 2020

Pediatric Dermatology

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Dermatofibrosarcoma protuberans (DFSP) is the most common cutaneous sarcoma. It is characterized by a t(17;22)(q22;q13) reciprocal translocation, which results in the COL1A1-PDGFB fusion gene. This slow-growing, locally aggressive tumor occurs mostly in adults (median age: 37 years), occasionally in neonates, and very rarely in later childhood. 1,2 The age-adjusted incidence of DFSP in patients under the age of 20 is 1.0 per million. 3 The tumor's high local recurrence rate contrasts with its low metastatic potential. The "gold standard" treatment for DFSP is wide local excision (WLE) using Mohs micrographic surgery (Mohs). The overall prognosis is excellent, with a 5-year survival rate close to 100%. 4 Ten to twenty percent of adult patients of DFSP undergo de novo or post-recurrence transformation into a high-grade, aggressive fibrosarcoma (FS) with greater metastatic potential. This fibrosarcomatous DFSP (FS-DFSP) shares the same cytogenetic hallmark as its low-grade counterpart. A histopathological assessment is essential for the accurate diagnosis of FS-DFSP, although sampling errors on punch biopsies may be problematic. 5,6 We report on a rare case of de novo FS-DFSP in a young child.

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“…Disease recurrence has a higher degree of local infiltration, tissue destruction, and fibrosarcomatous transformation 4. Fibrosarcomatous DFSP is a rare pathologic variant with a more aggressive infiltrative growth and higher rate of metastasis 5. Morphologically, the transformed variant shows fascicular to herringbone growth with higher degree of pleomorphism and mitotic activity.…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation arising at the site of breast implant

et al. 2020

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Dermatofibrosarcoma protuberans (DFSP) has an incidence rate of 0.8-5 cases per million and accounts for 1% of all soft tissue sarcomas.1,2 It is a slow-growing low-grade malignancy that rarely metastasizes but has a high rate of local infiltration and recurrence.3 Disease recurrence has a higher degree of local infiltration, tissue destruction, and fibrosarcomatous transformation.4 Fibrosarcomatous

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Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: our experience, molecular evaluation of selected cases, and short literature review

Cited by 9 publications

References 21 publications

When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation arising at the site of breast implant

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